BACKGROUND Granulomatosis with polyangiitis(GPA)is one of the most prevalent forms of the antineutrophil cytoplasmic antibody(ANCA)-associated vasculitis.GPA is characterized histologically by necrotizing granulomatou...BACKGROUND Granulomatosis with polyangiitis(GPA)is one of the most prevalent forms of the antineutrophil cytoplasmic antibody(ANCA)-associated vasculitis.GPA is characterized histologically by necrotizing granulomatous inflammation in addition to vasculitis.The diagnosis of GPA depends on clinical presentation,serological evidence of a positive ANCA,and/or histological evidence of necrotizing vasculitis or granulomatous destructive parenchymal inflammation.Cytoplasmic ANCA(c-ANCA)is positive in 65%-75% of GPA patients,accompanied by proteinase 3(PR3),the main target antigen of c-ANCA,another 5% of GPA patients had negative ANCA.CASE SUMMARY The patient,a 52-year-old male,presented with unexplained nasal congestion,tinnitus,and hearing loss.After a duration of 4 months experiencing these symptoms,the patient subsequently developed fever and headache.The imaging examination revealed the presence of bilateral auricular mastoiditis and partial paranasal sinusitis,and the ANCA results were negative.The anti-infective therapy proved to be ineffective,but the patient's symptoms and fever were quickly relieved after 1 wk of treatment with methylprednisolone 40 mg once a day.However,after continuous use of methylprednisolone tablets for 3 months,the patient experienced a recurrence of fever accompanied by right-sided migraine,positive c-ANCA and PR3,and increased total protein in cerebrospinal fluid.The and cyclophosphamide 0.8 g monthly,the patient experienced alleviation of fever and headache.Additionally,the ANCA levels became negative and there has been no recurrence.CONCLUSION For GPA patients with negative ANCA,there is a potential for early missed diagnosis.The integration of histopathological results and multidisciplinary communication plays a crucial role in facilitating ANCA-negative GPA.展开更多
AIM： Anti-Saccharomyces anti-nuclear associated cerevisiae antibodies （ASCA）, anti-neutrophil antibodies （NANA） and antibodies to exocrine pancreas （PAB）, are serological tools for discriminating Crohn＇s disea...AIM： Anti-Saccharomyces anti-nuclear associated cerevisiae antibodies （ASCA）, anti-neutrophil antibodies （NANA） and antibodies to exocrine pancreas （PAB）, are serological tools for discriminating Crohn＇s disease （CrD） and ulcerative colitis （UC）. Like CrD, coeliac disease （COD） is an inflammatory bowel disease （IBD） associated with （auto） antibodies. Performing a multicenter study we primarily aimed to determine the performance of ASCA, NANA and PAB tests for IBD diagnosis in children and adults, and secondarily to evaluate the prevalence of these markers in CoD. METHODS： Sera of 109 patients with CrD, 78 with UC, 45 with CoD and 50 healthy blood donors were retrospectively included. ASCA, NANA and PAB were detected by indirect immunofluorescence （IIF）. RESULTS： ASCA＋/NANA- profile displayed a positive predictive value of 94.2% for CrD. Detection of ASCA was correlated with a more severe clinical profile of CrD and treatment of the disease did not influence their serum levels. ASCA positivity was found in 37.9% of active CoD.PAB were found in 36.7% CrD and 13.3% CoD patients and were not correlated with clinical features of CrD, except with an early onset of the disease. Fifteen CrD patients were ASCA negative and PAB positive. CONCLUSION： ASCA and PAB detected by IIF are specific markers for CrD although their presence does not rule out a possible active CoD. The combination of ASCA, NANA and PAB tests improves the sensitivity of immunological markers for CrD. Repeating ASCA, NANA, and PAB testing during the course of CrD has no clinical value.展开更多
AIM： To assess the prevalence and clinical value of p-ANCA in a sample of Mexican ulcerative colitis （UC） patients. METHODS： In a prospective, IRB-approved protocol, p-ANCA was determined in 80 patients with UC （...AIM： To assess the prevalence and clinical value of p-ANCA in a sample of Mexican ulcerative colitis （UC） patients. METHODS： In a prospective, IRB-approved protocol, p-ANCA was determined in 80 patients with UC （mean age, 32 ± 12.9 years）. The severity and extension of disease were determined by clinical methods, searching a statistical association with p-ANCA status. RESULTS： p-ANCA were detected in 41 （51%） patients. Severity of disease was the only clinical variable statistically associated with their presence （P 〈 0.0001; OR = 9; CI 95% = 3.2-24.7）. CONCLUSION： The prevalence of p-ANCA was similar to that reported in other countries. Their presence was associated to UC severity, but offered no more information than the obtained by clinical methods.展开更多
The differential diagnosis of Behcet's disease(BD) from inflammatory bowel disease(IBD) is sometimes difficult and challenging.Hereby,we suggested the utility of anti-saccharomyces cerevisiae antibody(ASCA) and an...The differential diagnosis of Behcet's disease(BD) from inflammatory bowel disease(IBD) is sometimes difficult and challenging.Hereby,we suggested the utility of anti-saccharomyces cerevisiae antibody(ASCA) and anti-neutrophilic cytoplasmic antibody(p-ANCA) in the differential diagnosis of BD from IBD.展开更多
Objectives: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is an autoimmune disease usually seen in middle-aged and older adults but which is rare in children and adolescents. We sought to determine...Objectives: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is an autoimmune disease usually seen in middle-aged and older adults but which is rare in children and adolescents. We sought to determine if there has been a change in the incidence of this disorder. Methods: Single-center, retrospective review. Results: Over the last 2 years, we have encountered a striking increase in the frequency of this disease in pediatric patients. All eight patients seen during this period had renal involvement and 5 patients rapidly progressed to end stage kidney disease. The prognosis was worse in younger patients, those with microscopic polyangiitis, and those with chronic kidney damage in the diagnostic renal biopsy. Conclusions: We report these observations to highlight this change in the epidemiology of ANCA-associated vasculitis and to promote earlier recognition and treatment of this severe form of glomerulonephritis.展开更多
Objective To investigate the characteristics and outcome of glomerulonephritis in patients with both antineutrophil cytoplasmic antibody and anti-glomerular basement membrane antibody.Methods The sera of 23 antiGBM gl...Objective To investigate the characteristics and outcome of glomerulonephritis in patients with both antineutrophil cytoplasmic antibody and anti-glomerular basement membrane antibody.Methods The sera of 23 antiGBM glomerulonephritis patients were collected and were tested for ANCA respectively.Characteristics and outcome of patients with coexisting anti-GBM antibody展开更多
AIM:To study serological antibodies in Caucasians and Asians,in health and inflammatory bowel disease(IBD),in Australia and Hong Kong(HK).METHODS:Anti-glycan antibodies[anti-chitobioside(ACCA),anti-laminaribioside(ALC...AIM:To study serological antibodies in Caucasians and Asians,in health and inflammatory bowel disease(IBD),in Australia and Hong Kong(HK).METHODS:Anti-glycan antibodies[anti-chitobioside(ACCA),anti-laminaribioside(ALCA)],and anti-mannobioside(AMCA),anti-Saccharomyces cervisiae(gASCA);and atypical perinuclear anti-neutrophil cytoplasmic antibody(pANCA)were tested in IBD patients,their unaffected relatives,and healthy controls in Australia and HK(China).Antibody status(positive or negative)and titre was compared between subjects of different geography,ethnicity and disease state.RESULTS:Ninety subjects were evaluated:21 Crohn’s disease(CD),32 ulcerative colitis(UC),29 healthy controls,and 8 IBD patient relatives.Forty eight subjects were Australian(29 Caucasian and 19 ethnic Han Chinese)and 42 were from HK(all Han Chinese).Caucasian CD patients had a significantly higher antibody prevalence of gASCA(67%vs 3%,P<0.001),ALCA(44%vs 6%,P=0.005),and AMCA(67%vs 15%,P=0.002),whereas HK CD patients had a higher prevalence of only AMCA(58%vs 25%,P=0.035),when compared with UC and healthy subjects in both countries.Caucasian CD had significantly higher gASCA prevalence(67%vs 0%,P<0.001)and titre(median59 vs 9,P=0.002)than HK CD patients.Prevalence and titres of ALCA,ACCA and AMCA did not differ between CD in the two countries.Presence of at least one antibody was higher in Caucasian than HK CD patients(100%vs 58%,P=0.045).pANCA did not differ between countries or ethnicity.CONCLUSION:Serologic CD responses differ between HK Asian and Australian Caucasian patients.Different genetic,environmental or disease pathogenic factors may account for these differences.展开更多
AIM:To define the parameters that positively predict diagnosis of Crohn's disease (CD) and differentiate it from gastrointestinal tuberculosis (GITB). METHODS:This prospective study over 3 years was carried out in...AIM:To define the parameters that positively predict diagnosis of Crohn's disease (CD) and differentiate it from gastrointestinal tuberculosis (GITB). METHODS:This prospective study over 3 years was carried out in the consecutive Indian patients with definite diagnosis of CD and equal numbers of patients with definite diagnosis of GITB. Demographic, clinical, laboratory, morphological and histological features were noted in all the patients. Serological tests such as p-ANCA, c-ANCA, IgA ASCA and IgG ASCA, were performed. Endoscopic biopsy and/or surgical tissue specimens were subjected to smear and culture for acid-fast bacilli (AFB) and tissue polymerase chain reaction for tuberculosis (TB PCR). Diagnosis of CD and GITB was based on the standard criteria. Data were analyzed using univariate Chi-square test and multiple logistic regression (MLR). RESULTS:The study is comprised of 26 patients with CD (age 36.6 ± 8.6 year, male:female, 16:10) and 26 patients with GITB (age 37.2 ± 9.6 year, male:female, 15:11). The following clinical variables between the two groups (CD vs TB) were significant in univariate analysis:duration of symptoms (58.1 ± 9.8 vs 7.2 ± 3.4 mo), diarrhoea (69.2% vs 34.6%), bleeding per rectum (30.7% vs 3.8%), fever (23.1% vs 69.2%), ascites (7.7% vs 34.6%) and extra-intestinal manifestations of inflammatory bowel disease (61.5% vs 23.1%). Of these, all except ascites and extra-colonic manifestations were found statistically significant by MLR. Accuracy of predicting CD was 84.62% based on the fever, bleeding P/R, diarrhoea and duration of symptoms while it was 63.4% when histology was reported as inflammatory bowel disease and 42.3% when there was recurrence of disease after surgery. Accuracy of predicting GITB was 73.1% when there was co-existing pulmonary lesions and/or abdominal lymphadenopathy;75% when tuberculosis was reported in histology;63.4% when granuloma was found in histology;82.6% when TB PCR was positive;and 61.5% when smear and/ or culture was positive for AFB. Serological test was not useful in differentiation of CD from GITB. Positivity rates for CD and GITB were:p-ANCA-3.8% and 3.8%, c-ANCA-3.8% and 0%, IgA ASCA-38.4% and 23.1%, and IgG ASCA-38.4% and 42.3%, respectively. CONCLUSION:Simple clinical parameters like fever, bleeding P/R, diarrhoea and duration of symptoms have the highest accuracy in differentiating CD from GITB.展开更多
Crohn's disease(CD) is a chronic idiopathic inflammatory disease of gastrointestinal tract characterizedby segmental and transmural involvement of gastrointestinal tract. Ileocolonic and colonic/anorectal is a mos...Crohn's disease(CD) is a chronic idiopathic inflammatory disease of gastrointestinal tract characterizedby segmental and transmural involvement of gastrointestinal tract. Ileocolonic and colonic/anorectal is a most common and account for 40% of cases and involvement of small intestine is about 30%. Isolated involvement of stomach is an extremely unusual presentation of the disease accounting for less than 0.07% of all gastrointestinal CD. To date there are only a few documented case reports of adults with isolated gastric CD and no reports in the pediatric population. The diagnosis is difficult to establish in such cases with atypical presentation. In the absence of any other source of disease and in the presence of nonspecific upper gastrointestinal endoscopy and histological findings, serological testing can play a vital role in the diagnosis of atypical CD. Recent studies have suggested that perinuclear anti-neutrophil cytoplasmic antibody and anti-Saccharomycescervisia antibody may be used as additional diagnostic tools. The effectiveness of infliximab in isolated gastric CD is limited to only a few case reports of adult patients and the long-term outcome is unknown.展开更多
The search for biologic markers that can assess the natural history and perhaps predict the course of individual's disease including response to treatments over time has become an important focus of inflammatory b...The search for biologic markers that can assess the natural history and perhaps predict the course of individual's disease including response to treatments over time has become an important focus of inflammatory bowel disease research.The knowledge of an individual's prognosis can help physicians and patients make important management decisions and aid communication on risk and benefits of disease and treatment.展开更多
Here, we present the case of a 74-year-old Japanese man with segmental intestinal necrosis, which developed after treatment with pulsed methylprednisolone for mononeuritis multiplex. The patient was weakly positive fo...Here, we present the case of a 74-year-old Japanese man with segmental intestinal necrosis, which developed after treatment with pulsed methylprednisolone for mononeuritis multiplex. The patient was weakly positive for myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA). Computed tomography and surgical findings were compatible with nonocclusive mesenteric ischemia (NOMI). He underwent small intestinal resection by emergency surgery and an intestinal fistula was made. Pathologically, necrotizing vasculitis with fibrinoid necrosis was present in medium to small-sized arteries, which was equivalent to Arkin's classification Ⅱ-Ⅳ. Most of the arteries had fibrous intimal thickening, which was considered to obstruct the arteries and thus cause segmental intestinal necrosis. A diagnosis of polyarteritis nodosa (PAN) was made, and intravenous cyclophosphamide pulse therapy was added to the therapeutic regimen. This patient was successfully treated with these multidisciplinary therapies and his stoma was finally closed. This is a very rare and indicative case of PAN weakly positive for MPO-ANCA and clinically mimicking NOMI, which occurred even after treatment with pulsed methylprednisolone.展开更多
Relapsing polychondritis(RP) is a rare autoimmune disease with chronic inflammatory/destructive lesions of the cartilaginous tissues. In one third of the cases it is associated with other autoimmune disorders, mostly ...Relapsing polychondritis(RP) is a rare autoimmune disease with chronic inflammatory/destructive lesions of the cartilaginous tissues. In one third of the cases it is associated with other autoimmune disorders, mostly with anti-neutrophil cytoplasmic antibody(ANCA) associated vasculitis(AAV). We report three cases of RP with p-ANCA positive AAV. In the first patient RP developed 1.5 years after the onset of AAV. In the others the signs of RP were present before the onset of severe crescent glomerulonephritis. Patients responded well on steroid and cyclophosphamide. In dialysis dependent cases plasmapheresis was also used successfully. During the 2 and 1.5 years of follow up, they were symptom-free, and had stable glomerular filtration rate. The first patient died after four years of follow-up due to the complications of sudden unset pancytopenia,which raises the possibility of associated hemophagocytic syndrome. In the setting of RP or AAV physicians should always be aware of the possibility of sudden or insidious appearance of the other disease.展开更多
Crohn's disease(CD) is a chronic idiopathic inflammatory disease of gastrointestinal tract characterized by segmental and transmural involvement of gastrointestinal tract. Ileocolonic and colonic/anorectal is a mo...Crohn's disease(CD) is a chronic idiopathic inflammatory disease of gastrointestinal tract characterized by segmental and transmural involvement of gastrointestinal tract. Ileocolonic and colonic/anorectal is a most common and account for 40% of cases and involvement of small intestine in about 30%. The stomach is rarely the sole or predominant site of CD. To date there are only a few documented case reports of adults with isolated gastric CD and no reports in the pediatric population. Isolated stomach involvement is very unusual presentation accounting for less than 0.07% of all gastrointestinal CD. The diagnosis is difficult to establish in cases of atypical presentation as in isolated gastroduodenal disease. In the absence of any other source of disease and in the presence of nonspecific upper GI endoscopy and histological findings, serological testing can play a vital role in the diagnosis of atypical CD. Recent stud-ies have suggested that perinuclear anti-neutrophil cytoplasmic antibody and anti-Saccharomycescervisia antibody may be used as additional diagnostic tools. The effectiveness of infliximab in isolated gastric CD is limited to only a few case reports of adult patients and the long-term outcome is unknown.展开更多
Rationale:Coexistence of anti-glomerular basement membrane(anti-GBM)disease with anti-neutrophil cytoplasmic antibody(ANCA)in a case of glomerulonephritis is often identified as a"double-positive"disease.Int...Rationale:Coexistence of anti-glomerular basement membrane(anti-GBM)disease with anti-neutrophil cytoplasmic antibody(ANCA)in a case of glomerulonephritis is often identified as a"double-positive"disease.Interestingly,the majority of"double positive"ANCA is myeloperoxidase(MPO)-ANCA and some of the MPO-ANCA positive cases showed intrarenal arteritis,suggesting an ANCA-associated kidney lesion.Proteinase 3-ANCA positive diseases are also rarely reported.Patients positive for all three antibodies,i.e.,triple-positive patients,are extremely rare.Patient's Concern:A 53 year-old female presented with anasarca and oliguria of 2 months'duration.Diagnosis:Pauci-immune type renal limited crescentic glomerulonephritis positive for MPO-ANCA,proteinase 3-ANCA,and anti-GBM antibody(triple-positive).Interventions:Intravenous high dose cyclophosphamide,oral azathioprine,intravenous methylprednisolone,and plasma exchange as per British Health Professionals in Rheumatology Guidelines.Outcomes:After one-month follow-up,anasarca and proteinuria were lessened,serum creatinine was normalized,titers of MPO-ANCA levels were decreased,and anti-GBM antibody levels were normalized.Lessons:Triple-positive renal limited vasculitis is rare and response to combined immunosuppressive therapy and plasma exchange can contribute to successful remission.展开更多
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are characterized by destruction of small vessels, granulomatous inflammation of the respiratory tract and necrotizing glomerulonephritis. This revie...Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are characterized by destruction of small vessels, granulomatous inflammation of the respiratory tract and necrotizing glomerulonephritis. This review describes the clinical diagnosis and therapy as well as the patho-physiology of ANCA-associated vasculitides with a specific focus on the interplay of ANCAs with activated neutrophils and the deleterious pathophysiological consequences of neutrophil-endothelium interaction.展开更多
Diagnosis of anti-neutrophil cytoplasmic antibodies(ANCA)-associated vasculitis is usually not difficult in patient with systemic disease, including lung and kidneys involvement, and laboratory signs of inflammation. ...Diagnosis of anti-neutrophil cytoplasmic antibodies(ANCA)-associated vasculitis is usually not difficult in patient with systemic disease, including lung and kidneys involvement, and laboratory signs of inflammation. The presence of ANCA and the results of histological investigation confirm diagnosis of ANCAassociated vasculitis. Cyclophosphamide/azathioprine in combination with high dose steroids are used to induce and maintain remission of systemic vasculitis. The clinical trials also showed efficacy of rituximab that induces depletion of B-cells. Our understanding and management of ANCA-associated vasculitis improved significantly over the last decades but there is still a lot of debate over its classification, diagnostic criteria, assessment of activity and optimum treatment.展开更多
The primary systemic vasculitides(PSV) are a group of rare inflammatory disorders affecting blood vessels of varying size and multiple organs. Urological manifestations of PSV are uncommon. Testicular vasculitis is th...The primary systemic vasculitides(PSV) are a group of rare inflammatory disorders affecting blood vessels of varying size and multiple organs. Urological manifestations of PSV are uncommon. Testicular vasculitis is the most commonly reported finding and is associated with Polyarteritis Nodosa(PAN), Henoch-Sch?nleinPurpura(HSP), anti-neutrophil cytoplasm antibody associated Vasculitides(AAV), Giant Cell Arteritis(GCA) and Kawasaki disease. Prostatic vasculitis has been reported in association with GCA and AAV. Ureteric involvement has been noted in PAN, HSP and AAV. Other urogenital manifestations of PSV include genital ulceration and bladder dysfunction in Beh?ets Disease and haematuria which is commonly seen in many of the PSV. Finally, therapies used to treat the PSV, especially cyclophosphamide, are associated with urological sideeffects including haemorrhagic cystitis and urothelial malignancy. The aim of this review is to examine how the urological system is involved in the PSV. Each PSV is examined in turn, with a brief clinical description of the disease followed by a description of the urological manifestations and management. Identification of urological manifestations of PSV is important as in many cases symptoms may improve with immunosuppressive therapy, avoiding the need for invasive surgery. Additionally, patients who present with isolated urogenital PSV are at higher risk of developing subsequent systemic vasculitis and will need to be followed up closely.展开更多
基金Supported by The Research Project of Zhejiang Chinese Medical University,No.2023JKZKTS33.
文摘BACKGROUND Granulomatosis with polyangiitis(GPA)is one of the most prevalent forms of the antineutrophil cytoplasmic antibody(ANCA)-associated vasculitis.GPA is characterized histologically by necrotizing granulomatous inflammation in addition to vasculitis.The diagnosis of GPA depends on clinical presentation,serological evidence of a positive ANCA,and/or histological evidence of necrotizing vasculitis or granulomatous destructive parenchymal inflammation.Cytoplasmic ANCA(c-ANCA)is positive in 65%-75% of GPA patients,accompanied by proteinase 3(PR3),the main target antigen of c-ANCA,another 5% of GPA patients had negative ANCA.CASE SUMMARY The patient,a 52-year-old male,presented with unexplained nasal congestion,tinnitus,and hearing loss.After a duration of 4 months experiencing these symptoms,the patient subsequently developed fever and headache.The imaging examination revealed the presence of bilateral auricular mastoiditis and partial paranasal sinusitis,and the ANCA results were negative.The anti-infective therapy proved to be ineffective,but the patient's symptoms and fever were quickly relieved after 1 wk of treatment with methylprednisolone 40 mg once a day.However,after continuous use of methylprednisolone tablets for 3 months,the patient experienced a recurrence of fever accompanied by right-sided migraine,positive c-ANCA and PR3,and increased total protein in cerebrospinal fluid.The and cyclophosphamide 0.8 g monthly,the patient experienced alleviation of fever and headache.Additionally,the ANCA levels became negative and there has been no recurrence.CONCLUSION For GPA patients with negative ANCA,there is a potential for early missed diagnosis.The integration of histopathological results and multidisciplinary communication plays a crucial role in facilitating ANCA-negative GPA.
文摘AIM： Anti-Saccharomyces anti-nuclear associated cerevisiae antibodies （ASCA）, anti-neutrophil antibodies （NANA） and antibodies to exocrine pancreas （PAB）, are serological tools for discriminating Crohn＇s disease （CrD） and ulcerative colitis （UC）. Like CrD, coeliac disease （COD） is an inflammatory bowel disease （IBD） associated with （auto） antibodies. Performing a multicenter study we primarily aimed to determine the performance of ASCA, NANA and PAB tests for IBD diagnosis in children and adults, and secondarily to evaluate the prevalence of these markers in CoD. METHODS： Sera of 109 patients with CrD, 78 with UC, 45 with CoD and 50 healthy blood donors were retrospectively included. ASCA, NANA and PAB were detected by indirect immunofluorescence （IIF）. RESULTS： ASCA＋/NANA- profile displayed a positive predictive value of 94.2% for CrD. Detection of ASCA was correlated with a more severe clinical profile of CrD and treatment of the disease did not influence their serum levels. ASCA positivity was found in 37.9% of active CoD.PAB were found in 36.7% CrD and 13.3% CoD patients and were not correlated with clinical features of CrD, except with an early onset of the disease. Fifteen CrD patients were ASCA negative and PAB positive. CONCLUSION： ASCA and PAB detected by IIF are specific markers for CrD although their presence does not rule out a possible active CoD. The combination of ASCA, NANA and PAB tests improves the sensitivity of immunological markers for CrD. Repeating ASCA, NANA, and PAB testing during the course of CrD has no clinical value.
基金Supported by the "Consejo Nacional de Ciencia y Tecnologia (CONACYT)", Mexico
文摘AIM： To assess the prevalence and clinical value of p-ANCA in a sample of Mexican ulcerative colitis （UC） patients. METHODS： In a prospective, IRB-approved protocol, p-ANCA was determined in 80 patients with UC （mean age, 32 ± 12.9 years）. The severity and extension of disease were determined by clinical methods, searching a statistical association with p-ANCA status. RESULTS： p-ANCA were detected in 41 （51%） patients. Severity of disease was the only clinical variable statistically associated with their presence （P 〈 0.0001; OR = 9; CI 95% = 3.2-24.7）. CONCLUSION： The prevalence of p-ANCA was similar to that reported in other countries. Their presence was associated to UC severity, but offered no more information than the obtained by clinical methods.
文摘The differential diagnosis of Behcet's disease(BD) from inflammatory bowel disease(IBD) is sometimes difficult and challenging.Hereby,we suggested the utility of anti-saccharomyces cerevisiae antibody(ASCA) and anti-neutrophilic cytoplasmic antibody(p-ANCA) in the differential diagnosis of BD from IBD.
文摘Objectives: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is an autoimmune disease usually seen in middle-aged and older adults but which is rare in children and adolescents. We sought to determine if there has been a change in the incidence of this disorder. Methods: Single-center, retrospective review. Results: Over the last 2 years, we have encountered a striking increase in the frequency of this disease in pediatric patients. All eight patients seen during this period had renal involvement and 5 patients rapidly progressed to end stage kidney disease. The prognosis was worse in younger patients, those with microscopic polyangiitis, and those with chronic kidney damage in the diagnostic renal biopsy. Conclusions: We report these observations to highlight this change in the epidemiology of ANCA-associated vasculitis and to promote earlier recognition and treatment of this severe form of glomerulonephritis.
文摘Objective To investigate the characteristics and outcome of glomerulonephritis in patients with both antineutrophil cytoplasmic antibody and anti-glomerular basement membrane antibody.Methods The sera of 23 antiGBM glomerulonephritis patients were collected and were tested for ANCA respectively.Characteristics and outcome of patients with coexisting anti-GBM antibody
文摘AIM:To study serological antibodies in Caucasians and Asians,in health and inflammatory bowel disease(IBD),in Australia and Hong Kong(HK).METHODS:Anti-glycan antibodies[anti-chitobioside(ACCA),anti-laminaribioside(ALCA)],and anti-mannobioside(AMCA),anti-Saccharomyces cervisiae(gASCA);and atypical perinuclear anti-neutrophil cytoplasmic antibody(pANCA)were tested in IBD patients,their unaffected relatives,and healthy controls in Australia and HK(China).Antibody status(positive or negative)and titre was compared between subjects of different geography,ethnicity and disease state.RESULTS:Ninety subjects were evaluated:21 Crohn’s disease(CD),32 ulcerative colitis(UC),29 healthy controls,and 8 IBD patient relatives.Forty eight subjects were Australian(29 Caucasian and 19 ethnic Han Chinese)and 42 were from HK(all Han Chinese).Caucasian CD patients had a significantly higher antibody prevalence of gASCA(67%vs 3%,P<0.001),ALCA(44%vs 6%,P=0.005),and AMCA(67%vs 15%,P=0.002),whereas HK CD patients had a higher prevalence of only AMCA(58%vs 25%,P=0.035),when compared with UC and healthy subjects in both countries.Caucasian CD had significantly higher gASCA prevalence(67%vs 0%,P<0.001)and titre(median59 vs 9,P=0.002)than HK CD patients.Prevalence and titres of ALCA,ACCA and AMCA did not differ between CD in the two countries.Presence of at least one antibody was higher in Caucasian than HK CD patients(100%vs 58%,P=0.045).pANCA did not differ between countries or ethnicity.CONCLUSION:Serologic CD responses differ between HK Asian and Australian Caucasian patients.Different genetic,environmental or disease pathogenic factors may account for these differences.
文摘AIM:To define the parameters that positively predict diagnosis of Crohn's disease (CD) and differentiate it from gastrointestinal tuberculosis (GITB). METHODS:This prospective study over 3 years was carried out in the consecutive Indian patients with definite diagnosis of CD and equal numbers of patients with definite diagnosis of GITB. Demographic, clinical, laboratory, morphological and histological features were noted in all the patients. Serological tests such as p-ANCA, c-ANCA, IgA ASCA and IgG ASCA, were performed. Endoscopic biopsy and/or surgical tissue specimens were subjected to smear and culture for acid-fast bacilli (AFB) and tissue polymerase chain reaction for tuberculosis (TB PCR). Diagnosis of CD and GITB was based on the standard criteria. Data were analyzed using univariate Chi-square test and multiple logistic regression (MLR). RESULTS:The study is comprised of 26 patients with CD (age 36.6 ± 8.6 year, male:female, 16:10) and 26 patients with GITB (age 37.2 ± 9.6 year, male:female, 15:11). The following clinical variables between the two groups (CD vs TB) were significant in univariate analysis:duration of symptoms (58.1 ± 9.8 vs 7.2 ± 3.4 mo), diarrhoea (69.2% vs 34.6%), bleeding per rectum (30.7% vs 3.8%), fever (23.1% vs 69.2%), ascites (7.7% vs 34.6%) and extra-intestinal manifestations of inflammatory bowel disease (61.5% vs 23.1%). Of these, all except ascites and extra-colonic manifestations were found statistically significant by MLR. Accuracy of predicting CD was 84.62% based on the fever, bleeding P/R, diarrhoea and duration of symptoms while it was 63.4% when histology was reported as inflammatory bowel disease and 42.3% when there was recurrence of disease after surgery. Accuracy of predicting GITB was 73.1% when there was co-existing pulmonary lesions and/or abdominal lymphadenopathy;75% when tuberculosis was reported in histology;63.4% when granuloma was found in histology;82.6% when TB PCR was positive;and 61.5% when smear and/ or culture was positive for AFB. Serological test was not useful in differentiation of CD from GITB. Positivity rates for CD and GITB were:p-ANCA-3.8% and 3.8%, c-ANCA-3.8% and 0%, IgA ASCA-38.4% and 23.1%, and IgG ASCA-38.4% and 42.3%, respectively. CONCLUSION:Simple clinical parameters like fever, bleeding P/R, diarrhoea and duration of symptoms have the highest accuracy in differentiating CD from GITB.
文摘Crohn's disease(CD) is a chronic idiopathic inflammatory disease of gastrointestinal tract characterizedby segmental and transmural involvement of gastrointestinal tract. Ileocolonic and colonic/anorectal is a most common and account for 40% of cases and involvement of small intestine is about 30%. Isolated involvement of stomach is an extremely unusual presentation of the disease accounting for less than 0.07% of all gastrointestinal CD. To date there are only a few documented case reports of adults with isolated gastric CD and no reports in the pediatric population. The diagnosis is difficult to establish in such cases with atypical presentation. In the absence of any other source of disease and in the presence of nonspecific upper gastrointestinal endoscopy and histological findings, serological testing can play a vital role in the diagnosis of atypical CD. Recent studies have suggested that perinuclear anti-neutrophil cytoplasmic antibody and anti-Saccharomycescervisia antibody may be used as additional diagnostic tools. The effectiveness of infliximab in isolated gastric CD is limited to only a few case reports of adult patients and the long-term outcome is unknown.
文摘The search for biologic markers that can assess the natural history and perhaps predict the course of individual's disease including response to treatments over time has become an important focus of inflammatory bowel disease research.The knowledge of an individual's prognosis can help physicians and patients make important management decisions and aid communication on risk and benefits of disease and treatment.
文摘Here, we present the case of a 74-year-old Japanese man with segmental intestinal necrosis, which developed after treatment with pulsed methylprednisolone for mononeuritis multiplex. The patient was weakly positive for myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA). Computed tomography and surgical findings were compatible with nonocclusive mesenteric ischemia (NOMI). He underwent small intestinal resection by emergency surgery and an intestinal fistula was made. Pathologically, necrotizing vasculitis with fibrinoid necrosis was present in medium to small-sized arteries, which was equivalent to Arkin's classification Ⅱ-Ⅳ. Most of the arteries had fibrous intimal thickening, which was considered to obstruct the arteries and thus cause segmental intestinal necrosis. A diagnosis of polyarteritis nodosa (PAN) was made, and intravenous cyclophosphamide pulse therapy was added to the therapeutic regimen. This patient was successfully treated with these multidisciplinary therapies and his stoma was finally closed. This is a very rare and indicative case of PAN weakly positive for MPO-ANCA and clinically mimicking NOMI, which occurred even after treatment with pulsed methylprednisolone.
基金Supported by Department of Nephrology,University of Debrecen,Clinical Centre,Debrecen,HungaryThe project implemented through the New Hungary Development Planco-financed by the European Union and the European Social Fund,No.TáMOP4.2.2.A-11/1/KONV-2012-0045
文摘Relapsing polychondritis(RP) is a rare autoimmune disease with chronic inflammatory/destructive lesions of the cartilaginous tissues. In one third of the cases it is associated with other autoimmune disorders, mostly with anti-neutrophil cytoplasmic antibody(ANCA) associated vasculitis(AAV). We report three cases of RP with p-ANCA positive AAV. In the first patient RP developed 1.5 years after the onset of AAV. In the others the signs of RP were present before the onset of severe crescent glomerulonephritis. Patients responded well on steroid and cyclophosphamide. In dialysis dependent cases plasmapheresis was also used successfully. During the 2 and 1.5 years of follow up, they were symptom-free, and had stable glomerular filtration rate. The first patient died after four years of follow-up due to the complications of sudden unset pancytopenia,which raises the possibility of associated hemophagocytic syndrome. In the setting of RP or AAV physicians should always be aware of the possibility of sudden or insidious appearance of the other disease.
文摘Crohn's disease(CD) is a chronic idiopathic inflammatory disease of gastrointestinal tract characterized by segmental and transmural involvement of gastrointestinal tract. Ileocolonic and colonic/anorectal is a most common and account for 40% of cases and involvement of small intestine in about 30%. The stomach is rarely the sole or predominant site of CD. To date there are only a few documented case reports of adults with isolated gastric CD and no reports in the pediatric population. Isolated stomach involvement is very unusual presentation accounting for less than 0.07% of all gastrointestinal CD. The diagnosis is difficult to establish in cases of atypical presentation as in isolated gastroduodenal disease. In the absence of any other source of disease and in the presence of nonspecific upper GI endoscopy and histological findings, serological testing can play a vital role in the diagnosis of atypical CD. Recent stud-ies have suggested that perinuclear anti-neutrophil cytoplasmic antibody and anti-Saccharomycescervisia antibody may be used as additional diagnostic tools. The effectiveness of infliximab in isolated gastric CD is limited to only a few case reports of adult patients and the long-term outcome is unknown.
文摘Rationale:Coexistence of anti-glomerular basement membrane(anti-GBM)disease with anti-neutrophil cytoplasmic antibody(ANCA)in a case of glomerulonephritis is often identified as a"double-positive"disease.Interestingly,the majority of"double positive"ANCA is myeloperoxidase(MPO)-ANCA and some of the MPO-ANCA positive cases showed intrarenal arteritis,suggesting an ANCA-associated kidney lesion.Proteinase 3-ANCA positive diseases are also rarely reported.Patients positive for all three antibodies,i.e.,triple-positive patients,are extremely rare.Patient's Concern:A 53 year-old female presented with anasarca and oliguria of 2 months'duration.Diagnosis:Pauci-immune type renal limited crescentic glomerulonephritis positive for MPO-ANCA,proteinase 3-ANCA,and anti-GBM antibody(triple-positive).Interventions:Intravenous high dose cyclophosphamide,oral azathioprine,intravenous methylprednisolone,and plasma exchange as per British Health Professionals in Rheumatology Guidelines.Outcomes:After one-month follow-up,anasarca and proteinuria were lessened,serum creatinine was normalized,titers of MPO-ANCA levels were decreased,and anti-GBM antibody levels were normalized.Lessons:Triple-positive renal limited vasculitis is rare and response to combined immunosuppressive therapy and plasma exchange can contribute to successful remission.
文摘Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are characterized by destruction of small vessels, granulomatous inflammation of the respiratory tract and necrotizing glomerulonephritis. This review describes the clinical diagnosis and therapy as well as the patho-physiology of ANCA-associated vasculitides with a specific focus on the interplay of ANCAs with activated neutrophils and the deleterious pathophysiological consequences of neutrophil-endothelium interaction.
文摘Diagnosis of anti-neutrophil cytoplasmic antibodies(ANCA)-associated vasculitis is usually not difficult in patient with systemic disease, including lung and kidneys involvement, and laboratory signs of inflammation. The presence of ANCA and the results of histological investigation confirm diagnosis of ANCAassociated vasculitis. Cyclophosphamide/azathioprine in combination with high dose steroids are used to induce and maintain remission of systemic vasculitis. The clinical trials also showed efficacy of rituximab that induces depletion of B-cells. Our understanding and management of ANCA-associated vasculitis improved significantly over the last decades but there is still a lot of debate over its classification, diagnostic criteria, assessment of activity and optimum treatment.
文摘The primary systemic vasculitides(PSV) are a group of rare inflammatory disorders affecting blood vessels of varying size and multiple organs. Urological manifestations of PSV are uncommon. Testicular vasculitis is the most commonly reported finding and is associated with Polyarteritis Nodosa(PAN), Henoch-Sch?nleinPurpura(HSP), anti-neutrophil cytoplasm antibody associated Vasculitides(AAV), Giant Cell Arteritis(GCA) and Kawasaki disease. Prostatic vasculitis has been reported in association with GCA and AAV. Ureteric involvement has been noted in PAN, HSP and AAV. Other urogenital manifestations of PSV include genital ulceration and bladder dysfunction in Beh?ets Disease and haematuria which is commonly seen in many of the PSV. Finally, therapies used to treat the PSV, especially cyclophosphamide, are associated with urological sideeffects including haemorrhagic cystitis and urothelial malignancy. The aim of this review is to examine how the urological system is involved in the PSV. Each PSV is examined in turn, with a brief clinical description of the disease followed by a description of the urological manifestations and management. Identification of urological manifestations of PSV is important as in many cases symptoms may improve with immunosuppressive therapy, avoiding the need for invasive surgery. Additionally, patients who present with isolated urogenital PSV are at higher risk of developing subsequent systemic vasculitis and will need to be followed up closely.
