During the early phase of the coronavirus disease 2019(COVID-19)pandemic caused by severe acute respiratory syndrome coronavirus 2(SARS-CoV-2),diagnosis was difficult due to the diversity in symptoms and imaging findi...During the early phase of the coronavirus disease 2019(COVID-19)pandemic caused by severe acute respiratory syndrome coronavirus 2(SARS-CoV-2),diagnosis was difficult due to the diversity in symptoms and imaging findings and the variability of disease presentation.Pulmonary manifestations are reportedly the main clinical presentations of COVID-19 patients.Scientists are working hard on a myriad of clinical,epidemiological,and biological aspects to better understand SARS-CoV-2 infection,aiming to mitigate the ongoing disaster.Many reports have documented the involvement of various body systems and organs apart from the respiratory tract including the gastrointestinal,liver,immune system,renal,and neurological systems.Such involvement will result in diverse presentations related to effects on these systems.Other presentations such as coagulation defects and cutaneous manifestation may also occur.Patients with specific comorbidities including obesity,diabetes,and hypertension have increased morbidity and mortality risks with COVID-19.展开更多
Potential causes of abnormal liver function tests include viral hepatitis,alcohol intake,nonalcoholic fatty liver disease,autoimmune liver diseases,hereditary diseases,hepatobiliary malignancies or infection,gallstone...Potential causes of abnormal liver function tests include viral hepatitis,alcohol intake,nonalcoholic fatty liver disease,autoimmune liver diseases,hereditary diseases,hepatobiliary malignancies or infection,gallstones and drug-induced liver injury.Moreover,the liver may be involved in systemic diseases that mainly affect other organs.Therefore,in patients without etiology of liver injury by screening serology and diagnostic imaging,but who have systemic diseases,the abnormal liver function test results might be caused by the systemic disease.In most of these patients,the systemic disease should be treated primarily.However,some patients with systemic disease and severe liver injury or fulminant hepatic failure require intensive treatments of the liver.展开更多
AIM： To investigate the prevalence of pseudoexfoliation syndrome （PEX） and its associations with ocular and systemic diseases in a population sample aged over 40y. METHODS： A total of 2356 subjects were randomly c...AIM： To investigate the prevalence of pseudoexfoliation syndrome （PEX） and its associations with ocular and systemic diseases in a population sample aged over 40y. METHODS： A total of 2356 subjects were randomly chosen for the sample population based on the database of the Turkish Statistical Agency in Eskisehir. Of the invited 2356 subjects, 2017 subjects participated, out of which 2009 were eligible for the study. Systemic diseases, drug use, smoking and body mass index were assessed using questionnaires. Measurements of central corneal thickness （CCT）, anterior chamber depth （ACD） and intraocular pressure （IOP） were performed during June to December, 2015. After pupillary dilation, the anterior segment and lenses were evaluated using a slit lamp.RESULTS： Prevalence of PEX in this sample was 5.0% （n=100）. Of patients with PEX, 26% also had glaucoma. Incidence of cataracts, and using drugs for hypertension, cardiac and psychiatric conditions was higher in patients with PEX compared to normal cases （P〈0.05）. Hearing loss was more common in PEX cases （34.0% vs 5.4%; P〈0.001）. The mean CCT, ACD and IOP were not significantly different between PEX and non-PEX cases.CONCLUSION： This study is the first population-based, randomized trial in Turkey. Prevalence of PEX in patients over 40 years old was found to be 5.0%. Besides glaucoma and cataract, hypertension, hearing loss, using drugs for cardiac and psychiatric diseases were associated with PEX.展开更多
<strong>Background: </strong>Dental students play a role in both oral and general health education in their communities, so it is important that they have good knowledge to enable them offer good counsel. ...<strong>Background: </strong>Dental students play a role in both oral and general health education in their communities, so it is important that they have good knowledge to enable them offer good counsel. Knowing the relationship between periodontal disease and systemic conditions will put them in better stead when giving oral health education. The aim of this study was to assess the level of knowledge among dental students of the relationship between periodontal diseases and systemic conditions, and to determine whether there was a correlation between this knowledge and their oral hygiene practices. <strong>Methods:</strong> This was a cross-sectional study among dental students of the University of Port Harcourt, Rivers State, Nigeria. Consenting participants were given a self-administered questionnaire eliciting socio-demographic information, knowledge about the relationship between periodontal diseases and systemic diseases, as well as oral hygiene practices. Each correct response on knowledge was scored as 1, while incorrect responses were scored 0, with a maximum score of 7. Participants’ knowledge was graded as poor (0 - 2), fair (3 - 5) and good (6 - 7). Data was analyzed using IBM SPSS version 21. p ≤ 0.05 was considered statistically significant. Spearman’s ranked correlation was used to assess the correlation between knowledge of the relationship between periodontal diseases and systemic diseases and oral hygiene practices. <strong>Results:</strong> There were 109 dental students (58 males, 51 females) in this study, with a mean age of 24.1 ± 3.6 years. Preclinical students were 43 (39.4%) while clinical students were 66 (60.6%). All (100%) participants knew that oral health was important to systemic health, with 89.9% knowing that periodontal disease was specifically linked to systemic disease. Only 50.5% of participants knew that there was a link between periodontal disease and diabetes mellitus. Sixty-four (58.7%) of the participants regularly cleaned in between their teeth. The most commonly used interdental cleaning aid was toothpick (45.0%). Clinical students had significantly better knowledge on the relationship between periodontal disease and systemic disease compared to pre-clinical students (p = 0.001). There was positive correlation between knowledge about periodontal disease and its link with systemic disease and better oral hygiene practices, but this was not statistically significant. <strong>Conclusion: </strong>The majority of dental students had a fair knowledge about the relationship between periodontal diseases and systemic diseases.展开更多
Introduction: Systemic autoimmune diseases have been poorly studied in sub-Saharan Africa and their frequency is not well known. This study provided an overview of the main reasons for the use of care and their evolut...Introduction: Systemic autoimmune diseases have been poorly studied in sub-Saharan Africa and their frequency is not well known. This study provided an overview of the main reasons for the use of care and their evolution in the main hospitals in Benin. Methods: This was a multi-centric descriptive cross-sectional study conducted in the internal medicine, rheumatology, dermatology and nephrology departments of nine (09) public and private hospital centers in Benin over a 57-month period, from January 1st, 2017 to September 30th, 2021. It involved patients followed for a systemic autoimmune disease. The data was collected with a digital survey sheet and then processed and analyzed with the R software (version 3.4). Results: Two hundred and three (203) patients were recorded, which represents a hospital frequency of 0.13%. The mean age was 44 years and the sex ratio (M/F) was 0.29. Connective tissue disease accounted for 95.07% of systemic autoimmune diseases which were dominated by rheumatoid arthritis (40.9%) and systemic lupus (37.4%). Ten cases of vasculitis have been reported and dominated by Behçet’s disease (40%). The main reasons for seeking care were asthenia, weight loss and fever. Arthralgia and skin lesions are the main guiding signs. Six deaths (3.1%) were recorded among connective tissue disease and 1 death (10%) among vasculitis. Conclusion: In spite of being rare, systemic autoimmune diseases are a reality in Benin. A general population study would provide a better understanding of clinical characteristics and identify prognostic factors.展开更多
Objective and Method:Symptoms manifesting in other parts of the body signaling the existence of spinal dysfunction(Ogundana Syndrome)in patients(N=35,age range 20-56yrs;mean age 36yrs)diagnosed to have systemic diseas...Objective and Method:Symptoms manifesting in other parts of the body signaling the existence of spinal dysfunction(Ogundana Syndrome)in patients(N=35,age range 20-56yrs;mean age 36yrs)diagnosed to have systemic diseases but treated with manual therapy were identified after a 30 year case note review.Results:Incipient parkinsonism(general edema and expressionless mask face),stroke(weakness of one side of the body),tinnitus(headache with noise in one or both ears),breast cancer(breast pain)and dyspnea(difficulty in breathing)were some physician diagnoses and clinical symptoms presented.One percent rate occurrence was noted affecting significantly more females(74%)than males(26%,P<0.001).This involved people with family history of back pain(66%)engaged in low income occupations(farming 40%,trading 25%,artisans 15% and civil servants 20%).Symptoms got better after vertical oscillatory pressure therapy to appropriate spinal regions.Duration of treatment was influenced by duration of spinal dysfunction prior to therapy and age of younger patients.Conclusion:One percent of patients with spinal dysfunction misdiagnosed to have systemic diseases due to misleading symptoms are finally referred for physiotherapy of manifesting symptoms.Manual therapy to the appropriate vertebro-geographic region(s)relieves the symptom(s)without prejudice to existing diagnosis.展开更多
BACKGROUND Immunoglobulin G4-related prostate disease(IgG4-RPD)characterized by a high count of IgG4-positive plasma cells has distinctive serological and radiological findings.Here we report a case of a patient who w...BACKGROUND Immunoglobulin G4-related prostate disease(IgG4-RPD)characterized by a high count of IgG4-positive plasma cells has distinctive serological and radiological findings.Here we report a case of a patient who was successfully treated for IgG4-RPD,which manifested as frequent micturition,dysuric,and systemic lymphadenopathy.CASE SUMMARY The patient was a 33-year-old man who was referred to our hospital because of urinary tract symptoms that had persisted for 4 years.A physical examination revealed systemic lymphadenopathy and blood tests showed hyperglobulinemia with an IgG level of 18.90 g/L and an IgG4 level of 18.40 g/L.Computed tomography(CT)revealed bilateral lacrimal gland,right parotid gland and prostatic enlargement.Based on these findings,IgG4-RD was suspected,and further pathological examination and follow-up results showed expected results.Finally,the patient was diagnosed with IgG4-RPD based on clinical symptoms,pathological examination,therapeutic effects,and follow-up results.He received 50 mg oral prednisolone(the dose was gradually reduced and a low dose was used for long-term maintenance)in combination with cyclophosphamide 1.0 g via an intravenous drip for 6 mo.One year after the treatment was initiated,he was free of urinary or other complaints and his serum IgG4 level normalized.CONCLUSION In IgG4-RPD with severe urinary tract symptoms,radiological findings should be carefully examined.IgG4-RPD prognosis is good because the disease responds well to glucocorticoids.Furthermore,it is urgent for clinicians and pathologists to improve their understanding of IgG4-RPD.展开更多
Systemic rheumatic diseases(SRDs)are chronic,inflammatory,autoimmune disorders with the presence of autoantibodies that may affect any organ or system.Liver dysfunction in SRDs can be associated with prescribed drugs,...Systemic rheumatic diseases(SRDs)are chronic,inflammatory,autoimmune disorders with the presence of autoantibodies that may affect any organ or system.Liver dysfunction in SRDs can be associated with prescribed drugs,viral hepatitis,alternative hepatic comorbidities and coexisting autoimmune liver diseases(AILDs),requiring an exclusion of secondary conditions before considering liver involvement.The patterns of overlap diseases depend predominantly on genetic determinants with common susceptible loci widely distributing in both disorders.In AILDs,it is important to identify the overlapping SRDs at an early stage since such a coexistence may influence the disease course and prognosis.Commonly co-occurring SRDs in AILDs are Sjögren syndrome(SS),rheumatoid arthritis(RA)or systemic lupus erythematosus(SLE)in autoimmune hepatitis(AIH),and SS,RA or systemic sclerosis in primary biliary cholangitis.Owing to different disease complications and therapies,it is imperative to differentiate between SLE liver involvement and SLE-AIH overlap disease.Therapeutic options can be personalized to control coexisting conditions of liver autoimmunity and rheumatic manifestations in AILD-SRD overlap diseases.The collaboration between hepatologists and rheumatologists can lead to significant advances in managing such a complex scenario.In this review,we provide a comprehensive overview on coexisting AILDs in different SRDs and the therapeutic approach in managing these overlap diseases.展开更多
Objective To evaluate the feasibility, efficacy, and safety of high dose immunosuppressive therapy （HDIT） and autolognus hemopoietic stem cell transplantation （HSCT） with CD^34＋ cell selection in patients with se...Objective To evaluate the feasibility, efficacy, and safety of high dose immunosuppressive therapy （HDIT） and autolognus hemopoietic stem cell transplantation （HSCT） with CD^34＋ cell selection in patients with severe, refractory autoim mune diseases. Methods Twenty-six patients with persistent systemic lupus erythematosus （SLE）, rheumatoid arthritis （RA）, primary Sjogren＇s syndrome （pSS）, or systemic sclerosis （SSc） who had been treated unsuccessfully with conventional treatment were enrolled in the trial in Peking Union Medical College Hospital from September 1999 to June 2004. The patients received HDIT with 200 mg/kg cyclophosphamide followed by an infusion of autologous stem cells that were CD34 selected. Disease acti- vity, adverse effect, hemopoietic and immune reconstitution, and time to recurrence of disease were monitored. Overall treatment related mortality was 7.7% （2/26） with 1 patient died of cytomegalovirus infection and an other of severe pneumonia. Relapse occurred in 3 SLE patients （17.6%） in 37, 26, and 19 months posttransplantation respectively, and 1 RA patient in 15 months posttransplantation. SLE Disease Activity Index （SLEDAI） scores of SLE survivors decreased significantly （P 〈 0.01）. RA patients recorded a drop of Disease Activity Score 28 （DAS 28）. The pSS patient remained symptoms free up to now, more than 50 months aider the transplantation. Conclusion HSCT can be performed relative safely in patients with severe autoimmune disease. Short-term effect of HSCT is promising. However treatment related mortality and relapse were observed in a subset of patients.展开更多
AIM: To investigate the association between autoimmune pancreatitis (AIP) and systemic autoimmune diseases (SAIDs) by measurement of serum immunoglobulin G4 (IgG4). METHODS: The serum level of IgG4 was measured in 61 ...AIM: To investigate the association between autoimmune pancreatitis (AIP) and systemic autoimmune diseases (SAIDs) by measurement of serum immunoglobulin G4 (IgG4). METHODS: The serum level of IgG4 was measured in 61 patients with SAIDs of different types who had not yet participated in glucocorticosteroid treatment. Patients with an elevated IgG4 level were examined by abdominal ultrasonography (US) and, in some cases, by computer tomography (CT). RESULTS: Elevated serum IgG4 levels (919 ± 996 mg/L) were detected in 17 (28%) of the 61 SAID patients. 10 patients had Sj gren's syndrome (SS) (IgG4: 590 ± 232 mg/L), 2 of them in association with Hashimoto's thyroiditis, and 7 patients (IgG4: 1388 ± 985.5 mg/L) had systemic lupus erythematosus (SLE). The IgG4 level in the SLE patients and that in patients with SS were not significantly different from that in AIP patients (783 ± 522 mg/L). Abdominal US and CT did not reveal any characteristic features of AIP among the SAID patients with an elevated IgG4 level. CONCLUSION: The serum IgG4 level may be elevated in SAIDs without the presence of AIP. The determination of serum IgG4 does not seem to be suitable for the differentiation between IgG4-related diseases and SAIDs.展开更多
<strong>Introduction:</strong> Systemic Autoimmune Diseases (SAID) long considered very rare in Africa are increasingly the subject of publications. The objective of this work is to identify the difficulti...<strong>Introduction:</strong> Systemic Autoimmune Diseases (SAID) long considered very rare in Africa are increasingly the subject of publications. The objective of this work is to identify the difficulties in the management of these pathologies in an internal medicine department in northern Senegal by analyzing the epidemiological, clinical-biological, therapeutic and evolutionary aspects of SAID. <strong>Methods:</strong> This was a descriptive cross-sectional study carried out in the internal medicine department of the Saint-Louis University Hospital Center. Included were all the files of patients followed in outpatient and/or hospitalization for autoimmune diseases according to the criteria of the American College of Rheumatology, during the period from January 2017 to December 2020. The data were analyzed using SPSS software version 21.0. As the study was descriptive, no statistical test was performed. <strong>Results: </strong>Out of 3800 patients, 70 presented SAID, <i>i.e. </i> a hospital prevalence of 1.8%. Polyarthritis was the first reason for consultation in 97% followed by skin manifestations in 8%. The patients had positive anti-nuclear autoantibodies in 88% of cases. Rheumatoid arthritis was the predominant condition (71%) followed by systemic lupus erythematosus (SLE) (15%) and undifferentiated autoimmune diseases in 10%. Eleven percent (11%) of patients had an associated autoimmune disease. Corticosteroids were used in the treatment of these conditions in 97% of cases and methotrexate was the most prescribed immunosuppressant (54%). Thirty-two percent (32%) of patients are lost to follow-up. <strong>Conclusion:</strong> SAID are diverse and under diagnosed;they are characterized by diagnostic delay above all linked to access to specialists and sometimes to the high cost of paraclinical examinations, in particular immunology. Treatment remains based primarily on corticosteroid therapy and conventional immunosuppressants in the face of the unavailability of biotherapies.展开更多
BACKGROUND The IFIH1 gene codes the MDA5 protein and the DDX58 gene codes the RIG-I receptor.Both proteins are parts of the interferon(IFN)I signaling pathway and are responsible for antiviral defense and innate immun...BACKGROUND The IFIH1 gene codes the MDA5 protein and the DDX58 gene codes the RIG-I receptor.Both proteins are parts of the interferon(IFN)I signaling pathway and are responsible for antiviral defense and innate immune response.IFIH1 and DDX58 polymorphisms are associated with a spectrum of autoimmune diseases.Rare gain-of-function IFIH1 mutations have been found in Singleton-Merten and Aicardi-Goutières syndrome,while DDX58 mutation can cause atypical Singleton-Merten syndrome.AIM To characterize children with pediatric rheumatic diseases(PRD)carrying DDX58 or IFIH1 variants.METHODS Clinical exome sequencing was performed on 92 children with different PRD.IFIH1 and DDX58 variants have been detected in 14 children.IFN-I score has been analyzed and the clinical characteristics of patients have been studied.RESULTS A total of seven patients with systemic lupus erythematosus(SLE)(n=2),myelodysplastic syndrome with SLE features at the onset of the disease(n=1),mixed connective tissue disease(MCTD)(n=1),undifferentiated systemic autoinflammatory disease(uSAID)(n=3)have 5 different variants of the DDX58 gene.A common non-pathogenic variant p.D580E has been found in five children.A rare variant of uncertain significance(VUS)p.N354S was found in one patient with uSAID,a rare likely non-pathogenic variant p.E37K in one patient with uSAID,and a rare likely pathogenic variant p.Cys864fs in a patient with SLE.Elevated IFN-I score was detected in 6 of 7 patients with DDX58 variants.Seven patients had six different IFIH1 variants.They were presented with uSAID(n=2),juvenile dermatomyositis(JDM)(n=1),SLElike disease(n=1),Periodic fever with aphthous stomatitis,pharyngitis,and adenitis syndrome(n=1),and systemic onset juvenile idiopathic arthritis(n=1).Three patients have VUS p.E627X,one patient has benign variant p.I923V.Rare VUS p.R595H was detected in the JDM patient.Another rare VUS p.L679Ifs*2 and previously not reported variant p.V599Ffs*5 were detected in the patient with uSAID.One patient with uSAID has rare VUS p.T520A.All patients had elevated IFN-I scores.CONCLUSION Rare compound-heterozygous IFIH1 variant(p.L679Ifs*2 and p.V599Ffs*5),heterozygous IFIH1 variant(p.T520A)and heterozygous DDX58 variant(p.Cys864fs)are probably disease causative for uSAID and SLE.The majority of patients with different DDX58 and IFI1 variants had hyperactivation of the IFN I signaling pathway.展开更多
Introduction: Autoimmune diseases are characterized by a very large clinical polymorphism that can lead to a diagnostic wandering. So, we aimed to determine their epidemiological profile outside the context of Dakar (...Introduction: Autoimmune diseases are characterized by a very large clinical polymorphism that can lead to a diagnostic wandering. So, we aimed to determine their epidemiological profile outside the context of Dakar (capital of Senegal) where the technical plateau is more elevated. Methodology: We conducted a retrospective descriptive and observational study from January 1, 2007 to December 31, 2017. All patients admitted or followed in outpatient in the Internal Medicine departments of the Saint Jean de Dieu and Regional Hospitals of Thiès as well as in the dermatology department of the CHRT (Regional Hospital Center of Thiès), and who met the MAI criteria (autoimmune diseases) have been included. The data were collected on a standardised sheet and analysed by EPI INFO version 7.2. Results: A total of 121 patients were included out of 25951 records i.e a prevalence of 0.46% in internal medicine departments. In dermatology, out of 31973 patients, 95 had MAIS (systemic autoimmune diseases): 0.29% as hospital prevalence. The average age was 40.7 years in internal medicine departments compared to 37.66 years 14.8 years in the dermatology department. Patients aged 30 to 59 years represented 57.89% of the study population. The sex ratio (H/F) was 0.3 in the internal medicine departments compared to 0.17 in the dermatology department. Circumstances of discovery were incidental in 16.52% and clinical in 3.30%. Biermer disease accounted for 29.75% of organ-specific MAI. Concerning systemic presentations, rheumatoid arthritis (RA) was present in 23.14%. Lupus was more representative in dermatology (65.2%) as well as systemic scleroderma (21%), dermatomyositis (6.3%). Cytopenia was found in 105 patients, showing in detail anemia (42.9%);leukopenia (14.8%);thrombocytopenia (2.4%). Autoantibodies were tested in 58 patients (47.9%). Skin histology was contributory in all cases of systemic scleroderma and in 5 cases of lupus. The main therapy prescribed was corticosteroid therapy alone or in combination with an immunosuppressant. Conclusion: In addition of infectious diseases, Subsaharan Africa is under the era of changing face of its epidemiology, and cardiovascular diseases shows signs of emergence, like auto-immune presentations. However, the difficult apprehension of these so subtle last diseases suggests that they are few reported. Technical tools in regions should be enhanced associated to a non-binding capacity building system targeting such diseases with an emphasis on good record keeping.展开更多
The glymphatic system is a relatively recently identified fluid exchange and transpo rt system in the brain.Accumulating evidence indicates thatglymphatic function is impaired not only in central nervous system disord...The glymphatic system is a relatively recently identified fluid exchange and transpo rt system in the brain.Accumulating evidence indicates thatglymphatic function is impaired not only in central nervous system disorders but also in systemic diseases.Systemic diseases can trigger the inflammatory responses in the central nervous system,occasionally leading to sustained inflammation and functional disturbance of the central nervous system.This review summarizes the current knowledge on the association between glymphatic dysfunction and central nervous system inflammation.In addition,we discuss the hypothesis that disease conditions initially associated with peripheral inflammation ove rwhelm the performance of the glymphatic system,thereby triggering central nervous system dysfun ction,chronic neuroinflammation,and neurodegeneration.Future research investigating the role of the glymphatic system in neuroinflammation may offer innovative therapeutic approaches for central nervous system disorders.展开更多
Saliva is secreted from the salivary glands and has multiple functions, including mouth cleaning and protection, antibacterial effects and digestion. With the rapid advancement in salivaomics, saliva is well recognize...Saliva is secreted from the salivary glands and has multiple functions, including mouth cleaning and protection, antibacterial effects and digestion. With the rapid advancement in salivaomics, saliva is well recognized as a pool of biological markers. Saliva, as a non-invasive and safe source, could be a substitute for blood in the diagnosis and prognosis of diseases. This review summarizes the latest advancements in saliva-related studies and addresses the potential value of saliva in the early diagnosis of oral diseases, such as dental caries and periodontal disease, as well as cancer, diabetes and other systemic disorders. Saliva biomarkers range from changes in the biochemical indices of DNA, RNA and proteins to the diversification of microbiota structures. This study integrates data reported in the recent literature and discusses the clinical significance and prospects for the application of saliva in the early diagnosis of diseases, translational medicine and precision medicine.展开更多
The object of this study is to assess the Ivlg （intravenous immunoglobulin） use in inflammatory systemic and immune-mediated illnesses, in patients older than 18 years in a tertiary hospital. The assessment also int...The object of this study is to assess the Ivlg （intravenous immunoglobulin） use in inflammatory systemic and immune-mediated illnesses, in patients older than 18 years in a tertiary hospital. The assessment also intends to ensure if the clinical indications matched with the evidence-based clinical guidelines recommendations of use. Analytical, observational, transversal and retrospective study carried out during 2012. Patients with inflammatory systemic and immuno-mediated illnesses, older than 18 years old, were included. The data collected were： age, sex, number of administrations, dosage, frequency, commercial brand and the indication for what the Ivlg treatment has been prescribed. As a reference guide the British Health Department Clinical Guidelines for Immunoglobulin Use （2nd edition, 2008, and 2nd edition update 2011） and its Spanish adaption were used. The lvlg treatment was justified by a grade of recommendation A, B or C in 41% of the indications. Thus in 59% （grey indications or unclear diagnosis） the IvIg use would be questionable because of its weak evidence. It was found one indication for what the prescription of IvIg was clearly not recommended. The inflammatory systemic and immune-mediated diseases include many pathologies for what the IvIg use has not been properly studied. There is a need of consensus guidelines for IvIg use to guide doctors and pharmacists in their clinical practice. Moreover, it is important to prioritize which indications and circumstances are of first importance to have their supply guaranteed.展开更多
Systemic lupus erythematosus is an autoimmune disease with an etiopathogenesis that is still unclear. With a higher incidence in women of childbearing age. Pregnancy in patients suffering from this pathology is a cons...Systemic lupus erythematosus is an autoimmune disease with an etiopathogenesis that is still unclear. With a higher incidence in women of childbearing age. Pregnancy in patients suffering from this pathology is a constant challenge for multidisciplinary teams. The changes of pregnancy, especially to the immune system and the kidney, have repercussions on the renal compromise secondary to lupus. Lupus nephropathy is more active during pregnancy and leads to adverse outcomes for the mother-fetus binomial. The early identification of pregnant women with a higher risk of complications, access to health resources and the participation of a multidisciplinary team is the strategy that increases maternal-fetal survival. A report case of a 25-year-old black female with SLE and a 10-week pregnancy who was admitted to the emergency department with criteria for dialysis. The literature focused on lupus nephritis and dialysis in pregnancy was reviewed. Articles were reviewed in databases such as PubMed, Cochrane, among others, focused on the topic of pregnancy with SLE and pregnancy with dialysis. A total of 15 review articles, 2 meta-analyses, 3 observational studies and 6 cohort studies (3 prospective and 3 retrospective) were filtered.展开更多
Objective:To evaluate the effects of rituximab versus mycophenolate mofetil or cyclophosphamide as control in lupus nephritis by meta-analysis.Methods:A systematic search was carried out up to January 2022,obtaining 7...Objective:To evaluate the effects of rituximab versus mycophenolate mofetil or cyclophosphamide as control in lupus nephritis by meta-analysis.Methods:A systematic search was carried out up to January 2022,obtaining 7 studies involving 645 participants with lupus nephritis at the commencement of the investigation;198 of them were treated with rituximab,while 447 were treated with mycophenolate mofetil or cyclophosphamide.We determined the odds ratio(OR)and mean difference(MD)with 95%confidence index(CI)to compare rituximab’s efficacy to that of mycophenolate mofetil or cyclophosphamide as control in lupus nephritis using random-or fixed-effects model by dichotomous or continuous techniques.Results:The rituximab group showed significantly higher complete renal remission rate(OR=2.52;95%CI 1.30-4.91,P=0.006)and total renal remission rates(OR=2.22;95%CI 1.36-3.63,P=0.001)than the control group.However,there was no significant difference in terms of end Systemic Lupus Erythematosus Disease Activity Index(SLEDAI)score(MD-1.16;95%CI-2.88-0.57,P=0.19),proteinuria(MD-0.31;95%CI-0.70-0.09,P=0.013),and serum creatinine(MD 0.01;95%CI-0.04-0.07,P=0.64)between the rituximab group and the control.Conclusion:Rituximab exhibited significantly greater complete renal remission rate and total renal remission rates,with no significant difference in terms of shorter-end SLEDAI,proteinuria,and serum creatinine,compared with the control in individuals with lupus nephritis.展开更多
BACKGROUND: Autoimmune pancreatitis (AIP) is a rare form of chronic pancreatitis with a discrete pathophysiology occasional diagnostic radiological findings, and characteristic histological features. Its etiology and ...BACKGROUND: Autoimmune pancreatitis (AIP) is a rare form of chronic pancreatitis with a discrete pathophysiology occasional diagnostic radiological findings, and characteristic histological features. Its etiology and pathogenesis are still under investigation, especially during the last decade Another aspect of interest is the attempt to establish specific criteria for the differential diagnosis between autoimmune pancreatitis and pancreatic cancer, entities that are frequently indistinguishable. DATA SOURCES: An extensive search of the PubMed database was performed with emphasis on articles about the differential diagnosis between autoimmune pancreatitis and pancreatic cancer up to the present. RESULTS: The most interesting outcome of recent research is the theory that autoimmune pancreatitis and its various extra-pancreatic manifestations represent a systemic fibro inflammatory process called IgG4-related systemic disease The diagnostic criteria proposed by the Japanese Pancreatic Society, the more expanded HISORt criteria, the new definitions of histological types, and the new guidelines of the International Association of Pancreatology help to establish the diagnosis of the disease types. CONCLUSION: The valuable help of the proposed criteria for the differential diagnosis between autoimmune pancreatitis and pancreatic cancer may lead to avoidance of pointless surgical treatments and increased patient morbidity.展开更多
Summary:Studies have shown that periodontal pathogens can enter the bloodstream,causing a series of reactions that can lead to a variety of systemic diseases.Epidemiological investigations also found a tight correlati...Summary:Studies have shown that periodontal pathogens can enter the bloodstream,causing a series of reactions that can lead to a variety of systemic diseases.Epidemiological investigations also found a tight correlation between periodontitis(PD)and osteoporosis.This study aimed to further explore the effect of periodontal pathogens on bone volume fraction like bone tissue and mass,and explain the relationship between PD and osteoporosis.Sprague Dawley rats(female,16 weeks old)were divided into the wild-type(WT)control group(m=9)and PD group(n=9).After eight weeks,periodontal tissues and ligatures,the fourth lumbar vertebra,the femur,the tibia,and blood were extracted and analyzed by micro-computed tomography(micro-CT),hematoxylin and eosin(H&E)staining,tartrate-resistant acid phosphatase(TRAP)staining,polymerase chain reaction(PCR),and enzyme-linked immunoassay(ELISA),respectively.We found that the bone mass of the lumbar vertebra,femur,and tibia was decreased in the PD group.The number of osteoclasts was higher in bone tissue in the PD group than in the WT group(P<0.05).The levels of inflammatory mediators and type I collagen C-terminal peptide(CTX-1)were higher in the PD group than in the WT group(P<0.05),although no significant difference in bone glutamic acid protein(BGP)levels was observed(P>0.05).In addition,we detected several periodontal pathogens,such as Porphyromonas gingivalis,Actinobacillus actinomycetemcomitans,and Fusobacterium nucleatum,in blood samples from rats in the PD group.These findings suggest that periodontal pathogens can enter the blood circulation from periodontal tissue,promote a systemic inflammation response,and subsequently reduce systemic bone density.展开更多
文摘During the early phase of the coronavirus disease 2019(COVID-19)pandemic caused by severe acute respiratory syndrome coronavirus 2(SARS-CoV-2),diagnosis was difficult due to the diversity in symptoms and imaging findings and the variability of disease presentation.Pulmonary manifestations are reportedly the main clinical presentations of COVID-19 patients.Scientists are working hard on a myriad of clinical,epidemiological,and biological aspects to better understand SARS-CoV-2 infection,aiming to mitigate the ongoing disaster.Many reports have documented the involvement of various body systems and organs apart from the respiratory tract including the gastrointestinal,liver,immune system,renal,and neurological systems.Such involvement will result in diverse presentations related to effects on these systems.Other presentations such as coagulation defects and cutaneous manifestation may also occur.Patients with specific comorbidities including obesity,diabetes,and hypertension have increased morbidity and mortality risks with COVID-19.
文摘Potential causes of abnormal liver function tests include viral hepatitis,alcohol intake,nonalcoholic fatty liver disease,autoimmune liver diseases,hereditary diseases,hepatobiliary malignancies or infection,gallstones and drug-induced liver injury.Moreover,the liver may be involved in systemic diseases that mainly affect other organs.Therefore,in patients without etiology of liver injury by screening serology and diagnostic imaging,but who have systemic diseases,the abnormal liver function test results might be caused by the systemic disease.In most of these patients,the systemic disease should be treated primarily.However,some patients with systemic disease and severe liver injury or fulminant hepatic failure require intensive treatments of the liver.
基金Spported by the Eskisehir Osmangazi University Scientific Research Project Commission(No.1311042)
文摘AIM： To investigate the prevalence of pseudoexfoliation syndrome （PEX） and its associations with ocular and systemic diseases in a population sample aged over 40y. METHODS： A total of 2356 subjects were randomly chosen for the sample population based on the database of the Turkish Statistical Agency in Eskisehir. Of the invited 2356 subjects, 2017 subjects participated, out of which 2009 were eligible for the study. Systemic diseases, drug use, smoking and body mass index were assessed using questionnaires. Measurements of central corneal thickness （CCT）, anterior chamber depth （ACD） and intraocular pressure （IOP） were performed during June to December, 2015. After pupillary dilation, the anterior segment and lenses were evaluated using a slit lamp.RESULTS： Prevalence of PEX in this sample was 5.0% （n=100）. Of patients with PEX, 26% also had glaucoma. Incidence of cataracts, and using drugs for hypertension, cardiac and psychiatric conditions was higher in patients with PEX compared to normal cases （P〈0.05）. Hearing loss was more common in PEX cases （34.0% vs 5.4%; P〈0.001）. The mean CCT, ACD and IOP were not significantly different between PEX and non-PEX cases.CONCLUSION： This study is the first population-based, randomized trial in Turkey. Prevalence of PEX in patients over 40 years old was found to be 5.0%. Besides glaucoma and cataract, hypertension, hearing loss, using drugs for cardiac and psychiatric diseases were associated with PEX.
文摘<strong>Background: </strong>Dental students play a role in both oral and general health education in their communities, so it is important that they have good knowledge to enable them offer good counsel. Knowing the relationship between periodontal disease and systemic conditions will put them in better stead when giving oral health education. The aim of this study was to assess the level of knowledge among dental students of the relationship between periodontal diseases and systemic conditions, and to determine whether there was a correlation between this knowledge and their oral hygiene practices. <strong>Methods:</strong> This was a cross-sectional study among dental students of the University of Port Harcourt, Rivers State, Nigeria. Consenting participants were given a self-administered questionnaire eliciting socio-demographic information, knowledge about the relationship between periodontal diseases and systemic diseases, as well as oral hygiene practices. Each correct response on knowledge was scored as 1, while incorrect responses were scored 0, with a maximum score of 7. Participants’ knowledge was graded as poor (0 - 2), fair (3 - 5) and good (6 - 7). Data was analyzed using IBM SPSS version 21. p ≤ 0.05 was considered statistically significant. Spearman’s ranked correlation was used to assess the correlation between knowledge of the relationship between periodontal diseases and systemic diseases and oral hygiene practices. <strong>Results:</strong> There were 109 dental students (58 males, 51 females) in this study, with a mean age of 24.1 ± 3.6 years. Preclinical students were 43 (39.4%) while clinical students were 66 (60.6%). All (100%) participants knew that oral health was important to systemic health, with 89.9% knowing that periodontal disease was specifically linked to systemic disease. Only 50.5% of participants knew that there was a link between periodontal disease and diabetes mellitus. Sixty-four (58.7%) of the participants regularly cleaned in between their teeth. The most commonly used interdental cleaning aid was toothpick (45.0%). Clinical students had significantly better knowledge on the relationship between periodontal disease and systemic disease compared to pre-clinical students (p = 0.001). There was positive correlation between knowledge about periodontal disease and its link with systemic disease and better oral hygiene practices, but this was not statistically significant. <strong>Conclusion: </strong>The majority of dental students had a fair knowledge about the relationship between periodontal diseases and systemic diseases.
文摘Introduction: Systemic autoimmune diseases have been poorly studied in sub-Saharan Africa and their frequency is not well known. This study provided an overview of the main reasons for the use of care and their evolution in the main hospitals in Benin. Methods: This was a multi-centric descriptive cross-sectional study conducted in the internal medicine, rheumatology, dermatology and nephrology departments of nine (09) public and private hospital centers in Benin over a 57-month period, from January 1st, 2017 to September 30th, 2021. It involved patients followed for a systemic autoimmune disease. The data was collected with a digital survey sheet and then processed and analyzed with the R software (version 3.4). Results: Two hundred and three (203) patients were recorded, which represents a hospital frequency of 0.13%. The mean age was 44 years and the sex ratio (M/F) was 0.29. Connective tissue disease accounted for 95.07% of systemic autoimmune diseases which were dominated by rheumatoid arthritis (40.9%) and systemic lupus (37.4%). Ten cases of vasculitis have been reported and dominated by Behçet’s disease (40%). The main reasons for seeking care were asthenia, weight loss and fever. Arthralgia and skin lesions are the main guiding signs. Six deaths (3.1%) were recorded among connective tissue disease and 1 death (10%) among vasculitis. Conclusion: In spite of being rare, systemic autoimmune diseases are a reality in Benin. A general population study would provide a better understanding of clinical characteristics and identify prognostic factors.
文摘Objective and Method:Symptoms manifesting in other parts of the body signaling the existence of spinal dysfunction(Ogundana Syndrome)in patients(N=35,age range 20-56yrs;mean age 36yrs)diagnosed to have systemic diseases but treated with manual therapy were identified after a 30 year case note review.Results:Incipient parkinsonism(general edema and expressionless mask face),stroke(weakness of one side of the body),tinnitus(headache with noise in one or both ears),breast cancer(breast pain)and dyspnea(difficulty in breathing)were some physician diagnoses and clinical symptoms presented.One percent rate occurrence was noted affecting significantly more females(74%)than males(26%,P<0.001).This involved people with family history of back pain(66%)engaged in low income occupations(farming 40%,trading 25%,artisans 15% and civil servants 20%).Symptoms got better after vertical oscillatory pressure therapy to appropriate spinal regions.Duration of treatment was influenced by duration of spinal dysfunction prior to therapy and age of younger patients.Conclusion:One percent of patients with spinal dysfunction misdiagnosed to have systemic diseases due to misleading symptoms are finally referred for physiotherapy of manifesting symptoms.Manual therapy to the appropriate vertebro-geographic region(s)relieves the symptom(s)without prejudice to existing diagnosis.
基金Supported by the Guangdong Provincial Bureau of Traditional Chinese Medicine,No.20222065Science and Technology Planning Project of Guangdong Province of China,No.A2021100National Natural Science Foundation of China,No.82104989。
文摘BACKGROUND Immunoglobulin G4-related prostate disease(IgG4-RPD)characterized by a high count of IgG4-positive plasma cells has distinctive serological and radiological findings.Here we report a case of a patient who was successfully treated for IgG4-RPD,which manifested as frequent micturition,dysuric,and systemic lymphadenopathy.CASE SUMMARY The patient was a 33-year-old man who was referred to our hospital because of urinary tract symptoms that had persisted for 4 years.A physical examination revealed systemic lymphadenopathy and blood tests showed hyperglobulinemia with an IgG level of 18.90 g/L and an IgG4 level of 18.40 g/L.Computed tomography(CT)revealed bilateral lacrimal gland,right parotid gland and prostatic enlargement.Based on these findings,IgG4-RD was suspected,and further pathological examination and follow-up results showed expected results.Finally,the patient was diagnosed with IgG4-RPD based on clinical symptoms,pathological examination,therapeutic effects,and follow-up results.He received 50 mg oral prednisolone(the dose was gradually reduced and a low dose was used for long-term maintenance)in combination with cyclophosphamide 1.0 g via an intravenous drip for 6 mo.One year after the treatment was initiated,he was free of urinary or other complaints and his serum IgG4 level normalized.CONCLUSION In IgG4-RPD with severe urinary tract symptoms,radiological findings should be carefully examined.IgG4-RPD prognosis is good because the disease responds well to glucocorticoids.Furthermore,it is urgent for clinicians and pathologists to improve their understanding of IgG4-RPD.
文摘Systemic rheumatic diseases(SRDs)are chronic,inflammatory,autoimmune disorders with the presence of autoantibodies that may affect any organ or system.Liver dysfunction in SRDs can be associated with prescribed drugs,viral hepatitis,alternative hepatic comorbidities and coexisting autoimmune liver diseases(AILDs),requiring an exclusion of secondary conditions before considering liver involvement.The patterns of overlap diseases depend predominantly on genetic determinants with common susceptible loci widely distributing in both disorders.In AILDs,it is important to identify the overlapping SRDs at an early stage since such a coexistence may influence the disease course and prognosis.Commonly co-occurring SRDs in AILDs are Sjögren syndrome(SS),rheumatoid arthritis(RA)or systemic lupus erythematosus(SLE)in autoimmune hepatitis(AIH),and SS,RA or systemic sclerosis in primary biliary cholangitis.Owing to different disease complications and therapies,it is imperative to differentiate between SLE liver involvement and SLE-AIH overlap disease.Therapeutic options can be personalized to control coexisting conditions of liver autoimmunity and rheumatic manifestations in AILD-SRD overlap diseases.The collaboration between hepatologists and rheumatologists can lead to significant advances in managing such a complex scenario.In this review,we provide a comprehensive overview on coexisting AILDs in different SRDs and the therapeutic approach in managing these overlap diseases.
基金Supported by Ministry of Health for ClinicalIntensiveSubjects(2001-2003 ).
文摘Objective To evaluate the feasibility, efficacy, and safety of high dose immunosuppressive therapy （HDIT） and autolognus hemopoietic stem cell transplantation （HSCT） with CD^34＋ cell selection in patients with severe, refractory autoim mune diseases. Methods Twenty-six patients with persistent systemic lupus erythematosus （SLE）, rheumatoid arthritis （RA）, primary Sjogren＇s syndrome （pSS）, or systemic sclerosis （SSc） who had been treated unsuccessfully with conventional treatment were enrolled in the trial in Peking Union Medical College Hospital from September 1999 to June 2004. The patients received HDIT with 200 mg/kg cyclophosphamide followed by an infusion of autologous stem cells that were CD34 selected. Disease acti- vity, adverse effect, hemopoietic and immune reconstitution, and time to recurrence of disease were monitored. Overall treatment related mortality was 7.7% （2/26） with 1 patient died of cytomegalovirus infection and an other of severe pneumonia. Relapse occurred in 3 SLE patients （17.6%） in 37, 26, and 19 months posttransplantation respectively, and 1 RA patient in 15 months posttransplantation. SLE Disease Activity Index （SLEDAI） scores of SLE survivors decreased significantly （P 〈 0.01）. RA patients recorded a drop of Disease Activity Score 28 （DAS 28）. The pSS patient remained symptoms free up to now, more than 50 months aider the transplantation. Conclusion HSCT can be performed relative safely in patients with severe autoimmune disease. Short-term effect of HSCT is promising. However treatment related mortality and relapse were observed in a subset of patients.
基金Supported by Grants TáMOP-4.2.1./B-09/1/KONV and 4.2.2-08/1-2008-0002 (partly)
文摘AIM: To investigate the association between autoimmune pancreatitis (AIP) and systemic autoimmune diseases (SAIDs) by measurement of serum immunoglobulin G4 (IgG4). METHODS: The serum level of IgG4 was measured in 61 patients with SAIDs of different types who had not yet participated in glucocorticosteroid treatment. Patients with an elevated IgG4 level were examined by abdominal ultrasonography (US) and, in some cases, by computer tomography (CT). RESULTS: Elevated serum IgG4 levels (919 ± 996 mg/L) were detected in 17 (28%) of the 61 SAID patients. 10 patients had Sj gren's syndrome (SS) (IgG4: 590 ± 232 mg/L), 2 of them in association with Hashimoto's thyroiditis, and 7 patients (IgG4: 1388 ± 985.5 mg/L) had systemic lupus erythematosus (SLE). The IgG4 level in the SLE patients and that in patients with SS were not significantly different from that in AIP patients (783 ± 522 mg/L). Abdominal US and CT did not reveal any characteristic features of AIP among the SAID patients with an elevated IgG4 level. CONCLUSION: The serum IgG4 level may be elevated in SAIDs without the presence of AIP. The determination of serum IgG4 does not seem to be suitable for the differentiation between IgG4-related diseases and SAIDs.
文摘<strong>Introduction:</strong> Systemic Autoimmune Diseases (SAID) long considered very rare in Africa are increasingly the subject of publications. The objective of this work is to identify the difficulties in the management of these pathologies in an internal medicine department in northern Senegal by analyzing the epidemiological, clinical-biological, therapeutic and evolutionary aspects of SAID. <strong>Methods:</strong> This was a descriptive cross-sectional study carried out in the internal medicine department of the Saint-Louis University Hospital Center. Included were all the files of patients followed in outpatient and/or hospitalization for autoimmune diseases according to the criteria of the American College of Rheumatology, during the period from January 2017 to December 2020. The data were analyzed using SPSS software version 21.0. As the study was descriptive, no statistical test was performed. <strong>Results: </strong>Out of 3800 patients, 70 presented SAID, <i>i.e. </i> a hospital prevalence of 1.8%. Polyarthritis was the first reason for consultation in 97% followed by skin manifestations in 8%. The patients had positive anti-nuclear autoantibodies in 88% of cases. Rheumatoid arthritis was the predominant condition (71%) followed by systemic lupus erythematosus (SLE) (15%) and undifferentiated autoimmune diseases in 10%. Eleven percent (11%) of patients had an associated autoimmune disease. Corticosteroids were used in the treatment of these conditions in 97% of cases and methotrexate was the most prescribed immunosuppressant (54%). Thirty-two percent (32%) of patients are lost to follow-up. <strong>Conclusion:</strong> SAID are diverse and under diagnosed;they are characterized by diagnostic delay above all linked to access to specialists and sometimes to the high cost of paraclinical examinations, in particular immunology. Treatment remains based primarily on corticosteroid therapy and conventional immunosuppressants in the face of the unavailability of biotherapies.
文摘BACKGROUND The IFIH1 gene codes the MDA5 protein and the DDX58 gene codes the RIG-I receptor.Both proteins are parts of the interferon(IFN)I signaling pathway and are responsible for antiviral defense and innate immune response.IFIH1 and DDX58 polymorphisms are associated with a spectrum of autoimmune diseases.Rare gain-of-function IFIH1 mutations have been found in Singleton-Merten and Aicardi-Goutières syndrome,while DDX58 mutation can cause atypical Singleton-Merten syndrome.AIM To characterize children with pediatric rheumatic diseases(PRD)carrying DDX58 or IFIH1 variants.METHODS Clinical exome sequencing was performed on 92 children with different PRD.IFIH1 and DDX58 variants have been detected in 14 children.IFN-I score has been analyzed and the clinical characteristics of patients have been studied.RESULTS A total of seven patients with systemic lupus erythematosus(SLE)(n=2),myelodysplastic syndrome with SLE features at the onset of the disease(n=1),mixed connective tissue disease(MCTD)(n=1),undifferentiated systemic autoinflammatory disease(uSAID)(n=3)have 5 different variants of the DDX58 gene.A common non-pathogenic variant p.D580E has been found in five children.A rare variant of uncertain significance(VUS)p.N354S was found in one patient with uSAID,a rare likely non-pathogenic variant p.E37K in one patient with uSAID,and a rare likely pathogenic variant p.Cys864fs in a patient with SLE.Elevated IFN-I score was detected in 6 of 7 patients with DDX58 variants.Seven patients had six different IFIH1 variants.They were presented with uSAID(n=2),juvenile dermatomyositis(JDM)(n=1),SLElike disease(n=1),Periodic fever with aphthous stomatitis,pharyngitis,and adenitis syndrome(n=1),and systemic onset juvenile idiopathic arthritis(n=1).Three patients have VUS p.E627X,one patient has benign variant p.I923V.Rare VUS p.R595H was detected in the JDM patient.Another rare VUS p.L679Ifs*2 and previously not reported variant p.V599Ffs*5 were detected in the patient with uSAID.One patient with uSAID has rare VUS p.T520A.All patients had elevated IFN-I scores.CONCLUSION Rare compound-heterozygous IFIH1 variant(p.L679Ifs*2 and p.V599Ffs*5),heterozygous IFIH1 variant(p.T520A)and heterozygous DDX58 variant(p.Cys864fs)are probably disease causative for uSAID and SLE.The majority of patients with different DDX58 and IFI1 variants had hyperactivation of the IFN I signaling pathway.
文摘Introduction: Autoimmune diseases are characterized by a very large clinical polymorphism that can lead to a diagnostic wandering. So, we aimed to determine their epidemiological profile outside the context of Dakar (capital of Senegal) where the technical plateau is more elevated. Methodology: We conducted a retrospective descriptive and observational study from January 1, 2007 to December 31, 2017. All patients admitted or followed in outpatient in the Internal Medicine departments of the Saint Jean de Dieu and Regional Hospitals of Thiès as well as in the dermatology department of the CHRT (Regional Hospital Center of Thiès), and who met the MAI criteria (autoimmune diseases) have been included. The data were collected on a standardised sheet and analysed by EPI INFO version 7.2. Results: A total of 121 patients were included out of 25951 records i.e a prevalence of 0.46% in internal medicine departments. In dermatology, out of 31973 patients, 95 had MAIS (systemic autoimmune diseases): 0.29% as hospital prevalence. The average age was 40.7 years in internal medicine departments compared to 37.66 years 14.8 years in the dermatology department. Patients aged 30 to 59 years represented 57.89% of the study population. The sex ratio (H/F) was 0.3 in the internal medicine departments compared to 0.17 in the dermatology department. Circumstances of discovery were incidental in 16.52% and clinical in 3.30%. Biermer disease accounted for 29.75% of organ-specific MAI. Concerning systemic presentations, rheumatoid arthritis (RA) was present in 23.14%. Lupus was more representative in dermatology (65.2%) as well as systemic scleroderma (21%), dermatomyositis (6.3%). Cytopenia was found in 105 patients, showing in detail anemia (42.9%);leukopenia (14.8%);thrombocytopenia (2.4%). Autoantibodies were tested in 58 patients (47.9%). Skin histology was contributory in all cases of systemic scleroderma and in 5 cases of lupus. The main therapy prescribed was corticosteroid therapy alone or in combination with an immunosuppressant. Conclusion: In addition of infectious diseases, Subsaharan Africa is under the era of changing face of its epidemiology, and cardiovascular diseases shows signs of emergence, like auto-immune presentations. However, the difficult apprehension of these so subtle last diseases suggests that they are few reported. Technical tools in regions should be enhanced associated to a non-binding capacity building system targeting such diseases with an emphasis on good record keeping.
基金supported by the National Natural Science Foundation of China,Nos.82071249 and 81771207 (both to CH)。
文摘The glymphatic system is a relatively recently identified fluid exchange and transpo rt system in the brain.Accumulating evidence indicates thatglymphatic function is impaired not only in central nervous system disorders but also in systemic diseases.Systemic diseases can trigger the inflammatory responses in the central nervous system,occasionally leading to sustained inflammation and functional disturbance of the central nervous system.This review summarizes the current knowledge on the association between glymphatic dysfunction and central nervous system inflammation.In addition,we discuss the hypothesis that disease conditions initially associated with peripheral inflammation ove rwhelm the performance of the glymphatic system,thereby triggering central nervous system dysfun ction,chronic neuroinflammation,and neurodegeneration.Future research investigating the role of the glymphatic system in neuroinflammation may offer innovative therapeutic approaches for central nervous system disorders.
基金supported by the National Natural Science Foundation of China (81430011 to Xue-Dong Zhou and 81371135 to Ji-Yao Li)Clinical Advanced Technique Award of West China Hospital of Stomatology (LCXJS2015 to Xin Xu)the Brilliant Young Investigator Award,Sichuan University (2015SCU04A16 to Xin Xu)
文摘Saliva is secreted from the salivary glands and has multiple functions, including mouth cleaning and protection, antibacterial effects and digestion. With the rapid advancement in salivaomics, saliva is well recognized as a pool of biological markers. Saliva, as a non-invasive and safe source, could be a substitute for blood in the diagnosis and prognosis of diseases. This review summarizes the latest advancements in saliva-related studies and addresses the potential value of saliva in the early diagnosis of oral diseases, such as dental caries and periodontal disease, as well as cancer, diabetes and other systemic disorders. Saliva biomarkers range from changes in the biochemical indices of DNA, RNA and proteins to the diversification of microbiota structures. This study integrates data reported in the recent literature and discusses the clinical significance and prospects for the application of saliva in the early diagnosis of diseases, translational medicine and precision medicine.
文摘The object of this study is to assess the Ivlg （intravenous immunoglobulin） use in inflammatory systemic and immune-mediated illnesses, in patients older than 18 years in a tertiary hospital. The assessment also intends to ensure if the clinical indications matched with the evidence-based clinical guidelines recommendations of use. Analytical, observational, transversal and retrospective study carried out during 2012. Patients with inflammatory systemic and immuno-mediated illnesses, older than 18 years old, were included. The data collected were： age, sex, number of administrations, dosage, frequency, commercial brand and the indication for what the Ivlg treatment has been prescribed. As a reference guide the British Health Department Clinical Guidelines for Immunoglobulin Use （2nd edition, 2008, and 2nd edition update 2011） and its Spanish adaption were used. The lvlg treatment was justified by a grade of recommendation A, B or C in 41% of the indications. Thus in 59% （grey indications or unclear diagnosis） the IvIg use would be questionable because of its weak evidence. It was found one indication for what the prescription of IvIg was clearly not recommended. The inflammatory systemic and immune-mediated diseases include many pathologies for what the IvIg use has not been properly studied. There is a need of consensus guidelines for IvIg use to guide doctors and pharmacists in their clinical practice. Moreover, it is important to prioritize which indications and circumstances are of first importance to have their supply guaranteed.
文摘Systemic lupus erythematosus is an autoimmune disease with an etiopathogenesis that is still unclear. With a higher incidence in women of childbearing age. Pregnancy in patients suffering from this pathology is a constant challenge for multidisciplinary teams. The changes of pregnancy, especially to the immune system and the kidney, have repercussions on the renal compromise secondary to lupus. Lupus nephropathy is more active during pregnancy and leads to adverse outcomes for the mother-fetus binomial. The early identification of pregnant women with a higher risk of complications, access to health resources and the participation of a multidisciplinary team is the strategy that increases maternal-fetal survival. A report case of a 25-year-old black female with SLE and a 10-week pregnancy who was admitted to the emergency department with criteria for dialysis. The literature focused on lupus nephritis and dialysis in pregnancy was reviewed. Articles were reviewed in databases such as PubMed, Cochrane, among others, focused on the topic of pregnancy with SLE and pregnancy with dialysis. A total of 15 review articles, 2 meta-analyses, 3 observational studies and 6 cohort studies (3 prospective and 3 retrospective) were filtered.
文摘Objective:To evaluate the effects of rituximab versus mycophenolate mofetil or cyclophosphamide as control in lupus nephritis by meta-analysis.Methods:A systematic search was carried out up to January 2022,obtaining 7 studies involving 645 participants with lupus nephritis at the commencement of the investigation;198 of them were treated with rituximab,while 447 were treated with mycophenolate mofetil or cyclophosphamide.We determined the odds ratio(OR)and mean difference(MD)with 95%confidence index(CI)to compare rituximab’s efficacy to that of mycophenolate mofetil or cyclophosphamide as control in lupus nephritis using random-or fixed-effects model by dichotomous or continuous techniques.Results:The rituximab group showed significantly higher complete renal remission rate(OR=2.52;95%CI 1.30-4.91,P=0.006)and total renal remission rates(OR=2.22;95%CI 1.36-3.63,P=0.001)than the control group.However,there was no significant difference in terms of end Systemic Lupus Erythematosus Disease Activity Index(SLEDAI)score(MD-1.16;95%CI-2.88-0.57,P=0.19),proteinuria(MD-0.31;95%CI-0.70-0.09,P=0.013),and serum creatinine(MD 0.01;95%CI-0.04-0.07,P=0.64)between the rituximab group and the control.Conclusion:Rituximab exhibited significantly greater complete renal remission rate and total renal remission rates,with no significant difference in terms of shorter-end SLEDAI,proteinuria,and serum creatinine,compared with the control in individuals with lupus nephritis.
文摘BACKGROUND: Autoimmune pancreatitis (AIP) is a rare form of chronic pancreatitis with a discrete pathophysiology occasional diagnostic radiological findings, and characteristic histological features. Its etiology and pathogenesis are still under investigation, especially during the last decade Another aspect of interest is the attempt to establish specific criteria for the differential diagnosis between autoimmune pancreatitis and pancreatic cancer, entities that are frequently indistinguishable. DATA SOURCES: An extensive search of the PubMed database was performed with emphasis on articles about the differential diagnosis between autoimmune pancreatitis and pancreatic cancer up to the present. RESULTS: The most interesting outcome of recent research is the theory that autoimmune pancreatitis and its various extra-pancreatic manifestations represent a systemic fibro inflammatory process called IgG4-related systemic disease The diagnostic criteria proposed by the Japanese Pancreatic Society, the more expanded HISORt criteria, the new definitions of histological types, and the new guidelines of the International Association of Pancreatology help to establish the diagnosis of the disease types. CONCLUSION: The valuable help of the proposed criteria for the differential diagnosis between autoimmune pancreatitis and pancreatic cancer may lead to avoidance of pointless surgical treatments and increased patient morbidity.
基金This study was supported by the Natural Science Foundation of Hubei Province(No.2017CFB251).
文摘Summary:Studies have shown that periodontal pathogens can enter the bloodstream,causing a series of reactions that can lead to a variety of systemic diseases.Epidemiological investigations also found a tight correlation between periodontitis(PD)and osteoporosis.This study aimed to further explore the effect of periodontal pathogens on bone volume fraction like bone tissue and mass,and explain the relationship between PD and osteoporosis.Sprague Dawley rats(female,16 weeks old)were divided into the wild-type(WT)control group(m=9)and PD group(n=9).After eight weeks,periodontal tissues and ligatures,the fourth lumbar vertebra,the femur,the tibia,and blood were extracted and analyzed by micro-computed tomography(micro-CT),hematoxylin and eosin(H&E)staining,tartrate-resistant acid phosphatase(TRAP)staining,polymerase chain reaction(PCR),and enzyme-linked immunoassay(ELISA),respectively.We found that the bone mass of the lumbar vertebra,femur,and tibia was decreased in the PD group.The number of osteoclasts was higher in bone tissue in the PD group than in the WT group(P<0.05).The levels of inflammatory mediators and type I collagen C-terminal peptide(CTX-1)were higher in the PD group than in the WT group(P<0.05),although no significant difference in bone glutamic acid protein(BGP)levels was observed(P>0.05).In addition,we detected several periodontal pathogens,such as Porphyromonas gingivalis,Actinobacillus actinomycetemcomitans,and Fusobacterium nucleatum,in blood samples from rats in the PD group.These findings suggest that periodontal pathogens can enter the blood circulation from periodontal tissue,promote a systemic inflammation response,and subsequently reduce systemic bone density.
