The clinical spectrum of immunoglobulin A vasculitis nephritis(IgAVN)ranges from the relatively common transitory microscopic hematuria and/or low-grade proteinuria to nephritic or nephrotic syndrome,rapidly progressi...The clinical spectrum of immunoglobulin A vasculitis nephritis(IgAVN)ranges from the relatively common transitory microscopic hematuria and/or low-grade proteinuria to nephritic or nephrotic syndrome,rapidly progressive glomerulonephritis,or even renal failure.Clinical and experimental studies have shown a multifactor pathogenesis:Infection triggers,impaired glycosylation of IgA1,complement activation,Toll-like-receptor activation and B cell proliferation.This knowledge can identify IgAVN patients at a greater risk for adverse outcome and increase the evidence for treatment recommendations.展开更多
Antineutrophil cytoplasmic autoantibody(ANCA)-associated vasculitis(AAV)is a rare and severe systemic autoimmune disease characterized by pauci-immune necrotizing inflammation of small blood vessels.AAV involves multi...Antineutrophil cytoplasmic autoantibody(ANCA)-associated vasculitis(AAV)is a rare and severe systemic autoimmune disease characterized by pauci-immune necrotizing inflammation of small blood vessels.AAV involves multiple organ systems throughout the body.Our knowledge of the pathogenesis of AAV has increased considerably in recent years,involving cellular,molecular and genetic factors.Because of the controlled environment with no other confounding factors,animal models are beneficial for studying the mechanistic details of disease development and for providing novel therapeutic targets with fewer toxic side effects.However,the complexity and heterogeneity of AAV make it very difficult to establish a single animal model that can fully represent the entire clinical spectrum found in patients.The aim of this review is to overview the current status of animal models for AAV,outline the pros and cons of methods,and propose potential directions for future research.展开更多
BACKGROUND Patients with proteinase 3-antineutrophil cytoplasmic antibody associated vasculitis(AAV)experience different manifestations at the initial onset and relapse.However,such cases of different initial and rela...BACKGROUND Patients with proteinase 3-antineutrophil cytoplasmic antibody associated vasculitis(AAV)experience different manifestations at the initial onset and relapse.However,such cases of different initial and relapse manifestations have not been reported in myeloperoxidase(MPO)-AAV patients.CASE SUMMARY A 52-year-old woman was admitted to our hospital because of headache.Laboratory findings indicated nephrotic range proteinuria and microscopic hematuria,serum creatinine of 243μmol/L,anti-MPO antibody titer of>400 RU/mL,and positive perinuclearantineutrophil cytoplasmic antibody.Renal biopsy showed pauci-immune crescentic glomerulonephritis.The cerebrospinal fluid examination and brain magnetic resonance imaging did not show any abnormality.Therefore,MPO-AAV was diagnosed.Corticosteroids,plasmapheresis,and cyclophosphamide as induction therapy and mycophenolate mofetil(MMF)as maintenance therapy were administered.The patient’s headache disappeared;serum creatinine returned to normal;complete remission of microscopic hematuria and proteinuria was observed.Anti-MPO antibody titer reached normal limits after immunosuppressive treatment.Twenty-five months after stopping the immunosuppressive treatment,the patient relapsed with arthralgia,without neurological or renal involvement.The patient’s arthralgia improved after treatment with prednisone and MMF.CONCLUSION We have reported a rare case of MPO-AAV who initially presented with headache and kidney involvement.However,relapse presented with only arthralgia,which was completely different from the initial manifestations.This case suggests that AAV relapse should be highly suspected in MPO-AAV patients after remission,when clinical manifestations at relapse are different from those at onset.Prednisone and MMF may provide a good choice for refractory arthralgia during relapse in MPO-AAV patients.展开更多
Objective: According to clinical phenotypic classification, there is a significant overlap of clinical features between different anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV), and disease cl...Objective: According to clinical phenotypic classification, there is a significant overlap of clinical features between different anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV), and disease classification based on ANCA subtype helps to differentiate clinical phenotypes. We investigated the clinical features and outcomes of patients based on ANCA serotype classification. Methods: Clinical and laboratory data were collected retrospectively. We compared clinical manifestations and organ involvement based on serotype. The risk factors for death and renal survival were investigated with univariate and multivariate Cox regression models. Results: Patients with MPO-ANCA were predominant, whose median age and lung involvement at diagnosis was higher than that of the PR3-ANCA patients. Compared to the AAV patients without renal involvement, those with renal involvement have older, anemia, low complement C3, and hypoproteinemia, more likely to have cardiovascular and abdominal involvement, and have less lung involvement. Multivariate Cox analysis revealed that age ≥ 65 years (HR = 2.611, p p = 0.019), BVAS ≥ 15 (HR = 1.943, p = 0.001), low C3 (HR = 1.696, p = 0.008), and hypoproteinemia (HR = 1.438, p = 0.044) were associated with mortality. SCR ≥ 500 μmol/L (HR = 13.583, p p = 0.020), low C3 (HR = 1.506, p = 0.049) were independent detrimental factors for renal survival, and immunosuppressive treatment was a protective factor for renal survival (HR = 0.523, p = 0.003). Conclusions: Clinical manifestations varied by AAV categories. Age, SCR, BVAS, low C3 and hypoproteinemia at diagnosis were independent predictors of mortality. BVAS, low C3, SCR at diagnosis and immunosuppressive treatment were independently related to renal survival in ANCA positive patients.展开更多
BACKGROUND Single-organ vasculitis(SOV)is characterized by inflammation of a blood vessel,affecting one organ,such as the skin,genitourinary system,or the aorta without systemic features.Gastrointestinal SOV is rare,w...BACKGROUND Single-organ vasculitis(SOV)is characterized by inflammation of a blood vessel,affecting one organ,such as the skin,genitourinary system,or the aorta without systemic features.Gastrointestinal SOV is rare,with hepatic artery involvement reported only in two prior published cases.Herein,we presented a case of isolated hepatic artery vasculitis presenting after Pfizer-BioNTech mRNA corona virus disease 2019(COVID-19)vaccination.CASE SUMMARY A 50-year-old woman with hypertension presented to our Emergency Department with recurrent diffuse abdominal pain that localized to the epigastrium and emesis without diarrhea that began eight days after the second dose of the PfizerBioNTech COVID-19 vaccine.Blood work revealed an elevated C-reactive protein(CRP)of 19 mg/L(normal<4.8 mg/L),alkaline phosphatase 150 U/L(normal 25-105 U/L),gamma-glutamyl transferase(GGT)45 U/L(normal<43 U/L)and elevated immunoglobulins(Ig)G 18.4 g/L(normal 7-16 g/L)and IgA 4.4 g/L(normal 0.7-4 g/L).An abdominal computed tomography revealed findings in keeping with hepatic artery vasculitis.A detailed review of her history and examination did not reveal infectious or systemic autoimmune causes of her presentation.An extensive autoimmune panel was unremarkable.COVID-19polymerase chain reaction nasopharyngeal swab,human immunodeficiency virus,viral hepatitis and Heliobacter pylori serology were negative.At six months,the patient’s symptoms,and blood work spontaneously normalized.CONCLUSION High clinical suspicion of SOV is required for diagnosis in patients with acute abdominal pain and dyspepsia.展开更多
Vasculitis is an inflammation of vessel walls,followed by alteration of the blood flow and damage to the dependent organ.Vasculitis can cause local or diffuse pathologic changes in the gastrointestinal (GI) tract.The ...Vasculitis is an inflammation of vessel walls,followed by alteration of the blood flow and damage to the dependent organ.Vasculitis can cause local or diffuse pathologic changes in the gastrointestinal (GI) tract.The variety of GI lesions includes ulcer,submucosal edema,hemorrhage,paralytic ileus,mesenteric ischemia,bowel obstruction,and life-threatening perforation.The endoscopic and radiographic features of GI involvement in vasculitisare reviewed with the emphasis on small-vessel vasculitis by presenting our typicalcases,including Churg-Strauss syndrome,HenochSch nlein purpura,systemic lupus erythematosus,and Beh et's disease.Important endoscopic features are ischemic enterocolitis and ulcer.Characteristic computed tomographic findings include bowel wall thickening with the target sign and engorgement of mesenteric vessels with comb sign.Knowledge of endoscopic and radiographic GI manifestations can help make an early diagnosis and establish treatment strategy.展开更多
Henoch-Sch?nlein purpura(HSP) is generally a selflimited vasculitis disease and has a good prognosis. We report a 4-year-old Thai boy who presented with palpable purpura, abdominal colicky pain, seizure, and eventuall...Henoch-Sch?nlein purpura(HSP) is generally a selflimited vasculitis disease and has a good prognosis. We report a 4-year-old Thai boy who presented with palpable purpura, abdominal colicky pain, seizure, and eventually developed intestinal ischemia and perforation despite adequate treatment, including corticosteroid and intravenous immunoglobulin therapy. Imaging modalities, including ultrasonography and contrastenhanced computed tomography, could not detect intestinal ischemia prior to perforation. In this patient, we also postulated that vasculitis-induced mucosal ischemia was a cause of the ulcer, leading to intestinal perforation, and high-dose corticosteroid could have been a contributing factor since the histopathology revealed depletion of lymphoid follicles. Intestinal perforation in HSP is rare, but life-threatening. Close monitoring and thorough clinical evaluation are essential to detect bowel ischemia before perforation, particularly in HSP patients who have hematochezia, persistent localized abdominal tenderness and guarding. In highly suspicious cases, exploratory laparotomy may be needed for the definite diagnosis and prevention of further complications.展开更多
Human hepatitis viruses(HHVs)include hepatitis A virus,hepatitis B virus(HBV),hepatitis C virus(HCV),hepatitis delta virus,and hepatitis E virus and can cause liver inflammation in their common human host.Usually,HHV ...Human hepatitis viruses(HHVs)include hepatitis A virus,hepatitis B virus(HBV),hepatitis C virus(HCV),hepatitis delta virus,and hepatitis E virus and can cause liver inflammation in their common human host.Usually,HHV is rapidly cleared by the immune system,following acute HHV invasion.The morbidities associated with hepatitis A virus and hepatitis E virus infection occur shortly after their intrusion,in the acute stage.Nevertheless,the viral infectious process can persist for a long period of time,especially in HBV and HCV infection,leading to chronic hepatitis and further progressing to hepatic cirrhosis and liver cancer.HHV infection brings about complications in other organs,and both acute and chronic hepatitis have been associated with clinical presentations outside the liver.Vascular involvement with cutaneous and systemic vasculitis is a well-known extrahepatic presentation;moreover,there is growing evidence for a possible causal relationship between viral pathogens and vasculitis.Except for hepatitis delta virus,other HHVs have participated in the etiopathogenesis of cutaneous and systemic vasculitis via different mechanisms,including direct viral invasion of vascular endothelial cells,immune complex-mediated vessel wall damage,and autoimmune responses with stimulation of autoreactive B-cells and impaired regulatory T-cells.Cryoglobulinemic vasculitis and polyarteritis nodosa are recognized for their association with chronic HHV infection.Although therapeutic guidelines for HHV-associated vasculitis have not yet been established,antiviral therapy should be initiated in HBV and HCV-related systemic vasculitis in addition to the use of corticosteroids.Plasma exchange and/or combined cyclophosphamide and corticosteroid therapy can be considered in patients with severe life-threatening vasculitis manifestations.展开更多
Objectives:Malignant hypertension(MHT)is defi ned as severe hypertension accompanied by ischemic failure of one or more organs.The aims of this study were to evaluate the current clinical and etiologic profi les of MH...Objectives:Malignant hypertension(MHT)is defi ned as severe hypertension accompanied by ischemic failure of one or more organs.The aims of this study were to evaluate the current clinical and etiologic profi les of MHT.Methods:As a retrospective study,we selected all patients admitted to our center from January 2013 to December 2016.Seventy patients with MHT were included.Results:The average age of the patients was 40 years,and more than half of the patients were male(78.57%).There were 24 patients with essential hypertension,accounting for 34.29%of the patients,and 46 with secondary hypertension,accounting for 65.71%of the patients.For secondary MHT,systemic vasculitis(25.57%)was the most common cause,followed by severe obstructive sleep apnea syndrome(15.71%),primary renal parenchymal hypertension(11.43%),primary aldosteronism(7.14%),and Cushing syndrome(1.43%)and nutcracker phenomenon(1.43%).Twenty patients with systemic vasculitis were characterized by severe hypertension accompanied by damage to two or more target organs of differing severity.The levels of white blood cells,hypersensitive C-reactive protein,serum creatinine,and 24-hour urinary protein were above their normal range.Conclusion:Systemic vasculitis may be one of the main causes of MHT,and has been underestimated in the past.In future clinical work,clinicians need to pay more attention to patients with systemic vasculitis.展开更多
To leptospirosis is the commonest spirocheatal infection in the tropical and temperate countries of Indian sub-continent and Africa and the most common zoonosis worldwide.The protean manifestation of this infectious d...To leptospirosis is the commonest spirocheatal infection in the tropical and temperate countries of Indian sub-continent and Africa and the most common zoonosis worldwide.The protean manifestation of this infectious disease is a challenge for practising clinicians across the world. In poor developing countries,at most clinical suspicion it is essential in the diagnosis of this disease.In this report,we are able to document two uncommon manifestations of leptospirosis, namely Sweet’s syndrome and central nervous system vasculitis.展开更多
Idiopathic hypereosinophilic syndrome(HES) is a rare disorder characterized by peripheral eosinophilia exceeding 1500/mm3, a chronic course, absence of secondary causes, and signs and symptoms of eosinophil-mediated t...Idiopathic hypereosinophilic syndrome(HES) is a rare disorder characterized by peripheral eosinophilia exceeding 1500/mm3, a chronic course, absence of secondary causes, and signs and symptoms of eosinophil-mediated tissue injury. One of the best-characterized forms of HES is the one associated with FIP1L1-PDGFRA gene rearrangement, which was recently demonstrated as responsive to treatment with the small molecule kinase inhibitor drug, imatinib mesylate. Here, we describe the case of a 51-year-old male, whose symptoms satisfied the clinical criteria for HES with cutaneous and cardiac involvement and who also presented with vasculitic brain lesions and retroperitoneal bleeding. Molecular testing, including fluorescence in situ hybridization, of bone marrow and peripheral blood showed no evidence of PDGFR rearrangements. The patient was initially treated with high-dose steroid therapy and then with hydroxyurea, but proved unresponsive to both. Upon subsequent initiation of imatinib mesilate, the patient showed a dramatic improvement in eosinophil count and progressed rapidly through clinical recovery. Long-term follow-up confirmed the efficacy of treatment with low-dose imatinib and with no need of supplemental steroid treatment, notwithstanding the absence of PDGFR rearrangement.展开更多
We report a very rare case of mitral valve dissection and aorticleft ventricular tunnel caused by possible autoimmune vasculitis.We suspected Behcet’s disease in this patient.There was no obvious clinical evidence of...We report a very rare case of mitral valve dissection and aorticleft ventricular tunnel caused by possible autoimmune vasculitis.We suspected Behcet’s disease in this patient.There was no obvious clinical evidence of infective endocarditis.Echocardiography is the diagnostic tool of choice to recognize valvular dysfunction,related pathology and possible complications.The patient may require immunosuppressive therapy due to the high likelihood of recurrence perioperation period.展开更多
Behet's disease is a chronic, relapsing, systemic vasculitis of unknown aetiology. Patients present manifestations of gastrointestinal complications, including mouth lesions, small and large intestinal lesions, ...Behet's disease is a chronic, relapsing, systemic vasculitis of unknown aetiology. Patients present manifestations of gastrointestinal complications, including mouth lesions, small and large intestinal lesions, and vascular lesions in the abdomen. In some cases, the intestinal ulcers of patients with Behet's disease are indistinguishable from those of Crohn's disease, tuberculosis, vasculitis and other diseases. In this article, we present a case of atypical Behet's disease with a complicated medical history and multisystem damage, for the purpose of better management of this disease.展开更多
Objective:Although relatively rare,adult immunoglobulin A vasculitis(IgAV)can lead to severe complications and longer hospitalization,and result in poor prognosis,when compared to childhood IgAV.Hence,early identifica...Objective:Although relatively rare,adult immunoglobulin A vasculitis(IgAV)can lead to severe complications and longer hospitalization,and result in poor prognosis,when compared to childhood IgAV.Hence,early identification and prevention for patients prone to develop systemic involvement are essential.The purpose of this study was to explore the correlations of common serological markers with the development of systemic involvement in adult IgAV.Methods:A retrospective analysis was performed for adult IgAV patients,who were hospitalized in Wuhan Union Hospital between January 2016 and December 2019.A total of 259 patients were enrolled,and the pre-treatment serological markers were comprehensively assessed.Results:In the present study,49.0% and 33.2% of patients developed renal and gastrointestinal(GI)involvement,respectively.Furthermore,the elevated levels of white blood cells count,D-Dimer(D-D),C-reactive protein(CRP)and neutrophil granulocyte ratio(NE%)>60% were significantly associated with GI involvement in the univariate analysis,while the decrease in high density lipoprotein level,and the elevated D-D and CRP levels were significantly associated with renal involvement(P<0.05).Moreover,a prediction model that combined multiple markers was established by performing a logistic regression analysis,and this presented a more favorable value of prediction than the individual serological markers.Conclusion:The present study suggests that common serological markers have close correlations with systemic involvement in adult IgAV,and that the establishment of a prediction model for systemic involvement may be helpful in facilitating personalized therapeutic strategies and clinical management for IgAV patients.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
Mycophenolic acid, the active metabolite for mycophenolate mofetil and mycophenolic sodium, is a strong, noncompetitive, reversible inhibitor of inosine monophosphate dehydrogenase, the key enzyme in de novo synthesis...Mycophenolic acid, the active metabolite for mycophenolate mofetil and mycophenolic sodium, is a strong, noncompetitive, reversible inhibitor of inosine monophosphate dehydrogenase, the key enzyme in de novo synthesis of guanosine nucleotides leading to selective inhibition of lymphocyte proliferation. Mycophenolic acid has been evaluated as induction and remission maintenance agent in the treatment of antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Since the course of disease of AAV usually requires long term immunosuppression, mycophenolate has been explored as a less toxic agent compared to cyclophosphamide and azathioprine. Mycophenolate is a potent immunosuppressive agent in the therapy of AAV, non-inferior to other available drugs with comparable side effect profile. Therefore, it could be a valuable alternative in cases of toxicity with life threatening side effects or intolerance to cyclophosphamide or azathioprine, in cases with high cumulative dose of cyclophosphamide, but also in cases with insufficient response. Several studies have shown a higher relapse rate following discontinuation of mycophenolate or in mycophenolate treated subjects that raises concerns about its usefulness in the treatment of AAV. This review describes the efficacy of mycophenolate in AAV as remission induction agent, as remission maintenance agent, and as therapeutic option in relapsing AAV disease, the relapse rate following discontinuation of mycophenolate, and the adverse events related to mycophenolate treatment.展开更多
BACKGROUND Systemic lupus erythematosus(SLE)and antineutrophil cytoplasmic antibodyassociated vasculitis(AAV)are classically thought to cause renal impairment and small vessel vasculitis with different pathophysiologi...BACKGROUND Systemic lupus erythematosus(SLE)and antineutrophil cytoplasmic antibodyassociated vasculitis(AAV)are classically thought to cause renal impairment and small vessel vasculitis with different pathophysiologies.Their overlap constitutes a rare rheumatologic disease.To date,only dozens of such cases with biopsyproven glomerulonephritis have been reported worldwide typically in women of childbearing age.Here,we present a unique clinical case due to its rarity and individualized treatment of a Chinese man in his eighth decade of life.CASE SUMMARY A 77-year-old man was admitted to several hospitals for shortness of breath and received nonspecific treatments over the past 3 years.As his symptoms were not completely relieved,he visited our hospital for further treatment.Laboratory examinations revealed kidney dysfunction,severe anaemia,hypocomplementemia,glomerular proteinuria,and microscopic haematuria.Antinuclear antibodies,as well as anti-dsDNA antibodies,were positive.Computed tomography of the chest showed right pleural effusion.Renal biopsy was performed,and histology suggested crescentic glomerulonephritis,pauci-immune type.After treatment with plasmapheresis,glucocorticoid,and cyclophosphamide,the disease was in remission,and the patient remained in a stable condition for over 3 years post-hospital discharge.CONCLUSION Due to its complexity and rarity,SLE and AAV overlap syndrome is easily misdiagnosed.An accurate diagnosis and treatment at the earliest stage may significantly improve the condition and reduce irreversible organ injury.展开更多
A pancreatic tumor was suspected on the abdominal ultrasound of a 72-year-old man. Abdominal computed tomography showed pancreatic enlargement as well as a diffuse, poorly enhanced area in the pancreas; endoscopic ult...A pancreatic tumor was suspected on the abdominal ultrasound of a 72-year-old man. Abdominal computed tomography showed pancreatic enlargement as well as a diffuse, poorly enhanced area in the pancreas; endoscopic ultrasound-guided fine needle aspiration biopsy and endoscopic retrograde cholangiopancreatography failed to provide a definitive diagnosis. Based on the trend of improvement of the pancreatic enlargement, the treatment plan involved follow-up examinations. Later, he was hospitalized with an alveolar hemorrhage and rapidly progressive glomerulonephritis; he tested positive for myeloperoxidase-anti-neutrophil cytoplasmic antibody(ANCA) and was diagnosed with ANCArelated vasculitis, specifically microscopic polyangiitis. It appears that factors such as thrombus formation caused by the vasculitis in the early stages of ANCArelated vasculitis cause abnormal distribution of the pancreatic blood flow, resulting in non-uniform pancreatitis. Pancreatic lesions in ANCA-related vasculitis are very rare. Only a few cases have been reported previously. Therefore, we report our case and a review of the literature.展开更多
Immune-mediated mechanisms are involved in the pathogenesis of both cerebral vasculitis and Parkinson's disease(PD, brainstem-predominant Lewy pathology), but the presentation of cerebral vasculitis with comorbid ...Immune-mediated mechanisms are involved in the pathogenesis of both cerebral vasculitis and Parkinson's disease(PD, brainstem-predominant Lewy pathology), but the presentation of cerebral vasculitis with comorbid Lewy pathology has not yet been reported. Here we present a case of pathologically confirmed vasculitis in a 73-year-old male patient whose postmortem examination revealed Lewy pathology diagnostic of PD. This case study suggests a comorbidity of cerebral vasculitis and Lewy pathology, as well as potential pathogenic interactions between these two disorders with immune-mediated mechanisms.展开更多
BACKGROUND Infection by the hepatitis C virus(HCV) is currently considered to be a global health issue, with a high worldwide prevalence and causing chronic disease in afflicted individuals. The disease largely involv...BACKGROUND Infection by the hepatitis C virus(HCV) is currently considered to be a global health issue, with a high worldwide prevalence and causing chronic disease in afflicted individuals. The disease largely involves the liver but it can affect other organs, including the skin. While leukocytoclastic vasculitis has been reported as one of the dermatologic manifestations of HCV infection, there are no reports of this condition as the first symptom of HCV recurrence after liver transplantation.CASE SUMMARY We report here a case of leukocytoclastic vasculitis in a liver transplant recipient on maintenance immunosuppression. The condition presented as a palpable purpura in both lower extremities. Blood and urine cultures were negative and all biochemical tests were normal, excepting evidence of anemia and hypocomplementemia. Imaging examination by computed tomography showed a small volume of ascites, diffuse thickening of bowel walls, and a small bilateral pleural effusion. Skin biopsy showed leukocytoclasia and fibrinoid necrosis.Liver biopsy was suggestive of HCV recurrence in the graft, and HCV polymerase chain reaction yielded 11460 copies/mL and identified the genotype as 1 A. Treatment of the virus with a 12-wk direct-acting antiviral regimen of ribavirin, sofosbuvir and daclatasvir led to regression of the symptoms within the first 10 d and subsequent complete resolution of the symptoms.CONCLUSION This case highlights the difficulties of diagnosing skin lesions caused by HCVinfection in immunosuppressed patients.展开更多
文摘The clinical spectrum of immunoglobulin A vasculitis nephritis(IgAVN)ranges from the relatively common transitory microscopic hematuria and/or low-grade proteinuria to nephritic or nephrotic syndrome,rapidly progressive glomerulonephritis,or even renal failure.Clinical and experimental studies have shown a multifactor pathogenesis:Infection triggers,impaired glycosylation of IgA1,complement activation,Toll-like-receptor activation and B cell proliferation.This knowledge can identify IgAVN patients at a greater risk for adverse outcome and increase the evidence for treatment recommendations.
基金the Ethics Committee of Tongji Hospital,Huazhong University of Science and Technology,China(TJ-IRB20191012).
文摘Antineutrophil cytoplasmic autoantibody(ANCA)-associated vasculitis(AAV)is a rare and severe systemic autoimmune disease characterized by pauci-immune necrotizing inflammation of small blood vessels.AAV involves multiple organ systems throughout the body.Our knowledge of the pathogenesis of AAV has increased considerably in recent years,involving cellular,molecular and genetic factors.Because of the controlled environment with no other confounding factors,animal models are beneficial for studying the mechanistic details of disease development and for providing novel therapeutic targets with fewer toxic side effects.However,the complexity and heterogeneity of AAV make it very difficult to establish a single animal model that can fully represent the entire clinical spectrum found in patients.The aim of this review is to overview the current status of animal models for AAV,outline the pros and cons of methods,and propose potential directions for future research.
文摘BACKGROUND Patients with proteinase 3-antineutrophil cytoplasmic antibody associated vasculitis(AAV)experience different manifestations at the initial onset and relapse.However,such cases of different initial and relapse manifestations have not been reported in myeloperoxidase(MPO)-AAV patients.CASE SUMMARY A 52-year-old woman was admitted to our hospital because of headache.Laboratory findings indicated nephrotic range proteinuria and microscopic hematuria,serum creatinine of 243μmol/L,anti-MPO antibody titer of>400 RU/mL,and positive perinuclearantineutrophil cytoplasmic antibody.Renal biopsy showed pauci-immune crescentic glomerulonephritis.The cerebrospinal fluid examination and brain magnetic resonance imaging did not show any abnormality.Therefore,MPO-AAV was diagnosed.Corticosteroids,plasmapheresis,and cyclophosphamide as induction therapy and mycophenolate mofetil(MMF)as maintenance therapy were administered.The patient’s headache disappeared;serum creatinine returned to normal;complete remission of microscopic hematuria and proteinuria was observed.Anti-MPO antibody titer reached normal limits after immunosuppressive treatment.Twenty-five months after stopping the immunosuppressive treatment,the patient relapsed with arthralgia,without neurological or renal involvement.The patient’s arthralgia improved after treatment with prednisone and MMF.CONCLUSION We have reported a rare case of MPO-AAV who initially presented with headache and kidney involvement.However,relapse presented with only arthralgia,which was completely different from the initial manifestations.This case suggests that AAV relapse should be highly suspected in MPO-AAV patients after remission,when clinical manifestations at relapse are different from those at onset.Prednisone and MMF may provide a good choice for refractory arthralgia during relapse in MPO-AAV patients.
文摘Objective: According to clinical phenotypic classification, there is a significant overlap of clinical features between different anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV), and disease classification based on ANCA subtype helps to differentiate clinical phenotypes. We investigated the clinical features and outcomes of patients based on ANCA serotype classification. Methods: Clinical and laboratory data were collected retrospectively. We compared clinical manifestations and organ involvement based on serotype. The risk factors for death and renal survival were investigated with univariate and multivariate Cox regression models. Results: Patients with MPO-ANCA were predominant, whose median age and lung involvement at diagnosis was higher than that of the PR3-ANCA patients. Compared to the AAV patients without renal involvement, those with renal involvement have older, anemia, low complement C3, and hypoproteinemia, more likely to have cardiovascular and abdominal involvement, and have less lung involvement. Multivariate Cox analysis revealed that age ≥ 65 years (HR = 2.611, p p = 0.019), BVAS ≥ 15 (HR = 1.943, p = 0.001), low C3 (HR = 1.696, p = 0.008), and hypoproteinemia (HR = 1.438, p = 0.044) were associated with mortality. SCR ≥ 500 μmol/L (HR = 13.583, p p = 0.020), low C3 (HR = 1.506, p = 0.049) were independent detrimental factors for renal survival, and immunosuppressive treatment was a protective factor for renal survival (HR = 0.523, p = 0.003). Conclusions: Clinical manifestations varied by AAV categories. Age, SCR, BVAS, low C3 and hypoproteinemia at diagnosis were independent predictors of mortality. BVAS, low C3, SCR at diagnosis and immunosuppressive treatment were independently related to renal survival in ANCA positive patients.
文摘BACKGROUND Single-organ vasculitis(SOV)is characterized by inflammation of a blood vessel,affecting one organ,such as the skin,genitourinary system,or the aorta without systemic features.Gastrointestinal SOV is rare,with hepatic artery involvement reported only in two prior published cases.Herein,we presented a case of isolated hepatic artery vasculitis presenting after Pfizer-BioNTech mRNA corona virus disease 2019(COVID-19)vaccination.CASE SUMMARY A 50-year-old woman with hypertension presented to our Emergency Department with recurrent diffuse abdominal pain that localized to the epigastrium and emesis without diarrhea that began eight days after the second dose of the PfizerBioNTech COVID-19 vaccine.Blood work revealed an elevated C-reactive protein(CRP)of 19 mg/L(normal<4.8 mg/L),alkaline phosphatase 150 U/L(normal 25-105 U/L),gamma-glutamyl transferase(GGT)45 U/L(normal<43 U/L)and elevated immunoglobulins(Ig)G 18.4 g/L(normal 7-16 g/L)and IgA 4.4 g/L(normal 0.7-4 g/L).An abdominal computed tomography revealed findings in keeping with hepatic artery vasculitis.A detailed review of her history and examination did not reveal infectious or systemic autoimmune causes of her presentation.An extensive autoimmune panel was unremarkable.COVID-19polymerase chain reaction nasopharyngeal swab,human immunodeficiency virus,viral hepatitis and Heliobacter pylori serology were negative.At six months,the patient’s symptoms,and blood work spontaneously normalized.CONCLUSION High clinical suspicion of SOV is required for diagnosis in patients with acute abdominal pain and dyspepsia.
文摘Vasculitis is an inflammation of vessel walls,followed by alteration of the blood flow and damage to the dependent organ.Vasculitis can cause local or diffuse pathologic changes in the gastrointestinal (GI) tract.The variety of GI lesions includes ulcer,submucosal edema,hemorrhage,paralytic ileus,mesenteric ischemia,bowel obstruction,and life-threatening perforation.The endoscopic and radiographic features of GI involvement in vasculitisare reviewed with the emphasis on small-vessel vasculitis by presenting our typicalcases,including Churg-Strauss syndrome,HenochSch nlein purpura,systemic lupus erythematosus,and Beh et's disease.Important endoscopic features are ischemic enterocolitis and ulcer.Characteristic computed tomographic findings include bowel wall thickening with the target sign and engorgement of mesenteric vessels with comb sign.Knowledge of endoscopic and radiographic GI manifestations can help make an early diagnosis and establish treatment strategy.
文摘Henoch-Sch?nlein purpura(HSP) is generally a selflimited vasculitis disease and has a good prognosis. We report a 4-year-old Thai boy who presented with palpable purpura, abdominal colicky pain, seizure, and eventually developed intestinal ischemia and perforation despite adequate treatment, including corticosteroid and intravenous immunoglobulin therapy. Imaging modalities, including ultrasonography and contrastenhanced computed tomography, could not detect intestinal ischemia prior to perforation. In this patient, we also postulated that vasculitis-induced mucosal ischemia was a cause of the ulcer, leading to intestinal perforation, and high-dose corticosteroid could have been a contributing factor since the histopathology revealed depletion of lymphoid follicles. Intestinal perforation in HSP is rare, but life-threatening. Close monitoring and thorough clinical evaluation are essential to detect bowel ischemia before perforation, particularly in HSP patients who have hematochezia, persistent localized abdominal tenderness and guarding. In highly suspicious cases, exploratory laparotomy may be needed for the definite diagnosis and prevention of further complications.
基金The Institutional Review Board of National Cheng Kung University Hospital approved this study(No.B-ER-105-108).
文摘Human hepatitis viruses(HHVs)include hepatitis A virus,hepatitis B virus(HBV),hepatitis C virus(HCV),hepatitis delta virus,and hepatitis E virus and can cause liver inflammation in their common human host.Usually,HHV is rapidly cleared by the immune system,following acute HHV invasion.The morbidities associated with hepatitis A virus and hepatitis E virus infection occur shortly after their intrusion,in the acute stage.Nevertheless,the viral infectious process can persist for a long period of time,especially in HBV and HCV infection,leading to chronic hepatitis and further progressing to hepatic cirrhosis and liver cancer.HHV infection brings about complications in other organs,and both acute and chronic hepatitis have been associated with clinical presentations outside the liver.Vascular involvement with cutaneous and systemic vasculitis is a well-known extrahepatic presentation;moreover,there is growing evidence for a possible causal relationship between viral pathogens and vasculitis.Except for hepatitis delta virus,other HHVs have participated in the etiopathogenesis of cutaneous and systemic vasculitis via different mechanisms,including direct viral invasion of vascular endothelial cells,immune complex-mediated vessel wall damage,and autoimmune responses with stimulation of autoreactive B-cells and impaired regulatory T-cells.Cryoglobulinemic vasculitis and polyarteritis nodosa are recognized for their association with chronic HHV infection.Although therapeutic guidelines for HHV-associated vasculitis have not yet been established,antiviral therapy should be initiated in HBV and HCV-related systemic vasculitis in addition to the use of corticosteroids.Plasma exchange and/or combined cyclophosphamide and corticosteroid therapy can be considered in patients with severe life-threatening vasculitis manifestations.
基金the Special Foundation of Autonomous Region Key Laboratory of China(grant number 2014KL014)and the National Natural Science Foundation of China(grant number 81360051).
文摘Objectives:Malignant hypertension(MHT)is defi ned as severe hypertension accompanied by ischemic failure of one or more organs.The aims of this study were to evaluate the current clinical and etiologic profi les of MHT.Methods:As a retrospective study,we selected all patients admitted to our center from January 2013 to December 2016.Seventy patients with MHT were included.Results:The average age of the patients was 40 years,and more than half of the patients were male(78.57%).There were 24 patients with essential hypertension,accounting for 34.29%of the patients,and 46 with secondary hypertension,accounting for 65.71%of the patients.For secondary MHT,systemic vasculitis(25.57%)was the most common cause,followed by severe obstructive sleep apnea syndrome(15.71%),primary renal parenchymal hypertension(11.43%),primary aldosteronism(7.14%),and Cushing syndrome(1.43%)and nutcracker phenomenon(1.43%).Twenty patients with systemic vasculitis were characterized by severe hypertension accompanied by damage to two or more target organs of differing severity.The levels of white blood cells,hypersensitive C-reactive protein,serum creatinine,and 24-hour urinary protein were above their normal range.Conclusion:Systemic vasculitis may be one of the main causes of MHT,and has been underestimated in the past.In future clinical work,clinicians need to pay more attention to patients with systemic vasculitis.
文摘To leptospirosis is the commonest spirocheatal infection in the tropical and temperate countries of Indian sub-continent and Africa and the most common zoonosis worldwide.The protean manifestation of this infectious disease is a challenge for practising clinicians across the world. In poor developing countries,at most clinical suspicion it is essential in the diagnosis of this disease.In this report,we are able to document two uncommon manifestations of leptospirosis, namely Sweet’s syndrome and central nervous system vasculitis.
文摘Idiopathic hypereosinophilic syndrome(HES) is a rare disorder characterized by peripheral eosinophilia exceeding 1500/mm3, a chronic course, absence of secondary causes, and signs and symptoms of eosinophil-mediated tissue injury. One of the best-characterized forms of HES is the one associated with FIP1L1-PDGFRA gene rearrangement, which was recently demonstrated as responsive to treatment with the small molecule kinase inhibitor drug, imatinib mesylate. Here, we describe the case of a 51-year-old male, whose symptoms satisfied the clinical criteria for HES with cutaneous and cardiac involvement and who also presented with vasculitic brain lesions and retroperitoneal bleeding. Molecular testing, including fluorescence in situ hybridization, of bone marrow and peripheral blood showed no evidence of PDGFR rearrangements. The patient was initially treated with high-dose steroid therapy and then with hydroxyurea, but proved unresponsive to both. Upon subsequent initiation of imatinib mesilate, the patient showed a dramatic improvement in eosinophil count and progressed rapidly through clinical recovery. Long-term follow-up confirmed the efficacy of treatment with low-dose imatinib and with no need of supplemental steroid treatment, notwithstanding the absence of PDGFR rearrangement.
文摘We report a very rare case of mitral valve dissection and aorticleft ventricular tunnel caused by possible autoimmune vasculitis.We suspected Behcet’s disease in this patient.There was no obvious clinical evidence of infective endocarditis.Echocardiography is the diagnostic tool of choice to recognize valvular dysfunction,related pathology and possible complications.The patient may require immunosuppressive therapy due to the high likelihood of recurrence perioperation period.
基金Supported by Major Foundation of Xiamen Gastroenterology Center,No.200702
文摘Behet's disease is a chronic, relapsing, systemic vasculitis of unknown aetiology. Patients present manifestations of gastrointestinal complications, including mouth lesions, small and large intestinal lesions, and vascular lesions in the abdomen. In some cases, the intestinal ulcers of patients with Behet's disease are indistinguishable from those of Crohn's disease, tuberculosis, vasculitis and other diseases. In this article, we present a case of atypical Behet's disease with a complicated medical history and multisystem damage, for the purpose of better management of this disease.
基金supported by the National Natural Science Foundation of China(No.81772913).
文摘Objective:Although relatively rare,adult immunoglobulin A vasculitis(IgAV)can lead to severe complications and longer hospitalization,and result in poor prognosis,when compared to childhood IgAV.Hence,early identification and prevention for patients prone to develop systemic involvement are essential.The purpose of this study was to explore the correlations of common serological markers with the development of systemic involvement in adult IgAV.Methods:A retrospective analysis was performed for adult IgAV patients,who were hospitalized in Wuhan Union Hospital between January 2016 and December 2019.A total of 259 patients were enrolled,and the pre-treatment serological markers were comprehensively assessed.Results:In the present study,49.0% and 33.2% of patients developed renal and gastrointestinal(GI)involvement,respectively.Furthermore,the elevated levels of white blood cells count,D-Dimer(D-D),C-reactive protein(CRP)and neutrophil granulocyte ratio(NE%)>60% were significantly associated with GI involvement in the univariate analysis,while the decrease in high density lipoprotein level,and the elevated D-D and CRP levels were significantly associated with renal involvement(P<0.05).Moreover,a prediction model that combined multiple markers was established by performing a logistic regression analysis,and this presented a more favorable value of prediction than the individual serological markers.Conclusion:The present study suggests that common serological markers have close correlations with systemic involvement in adult IgAV,and that the establishment of a prediction model for systemic involvement may be helpful in facilitating personalized therapeutic strategies and clinical management for IgAV patients.
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Mycophenolic acid, the active metabolite for mycophenolate mofetil and mycophenolic sodium, is a strong, noncompetitive, reversible inhibitor of inosine monophosphate dehydrogenase, the key enzyme in de novo synthesis of guanosine nucleotides leading to selective inhibition of lymphocyte proliferation. Mycophenolic acid has been evaluated as induction and remission maintenance agent in the treatment of antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Since the course of disease of AAV usually requires long term immunosuppression, mycophenolate has been explored as a less toxic agent compared to cyclophosphamide and azathioprine. Mycophenolate is a potent immunosuppressive agent in the therapy of AAV, non-inferior to other available drugs with comparable side effect profile. Therefore, it could be a valuable alternative in cases of toxicity with life threatening side effects or intolerance to cyclophosphamide or azathioprine, in cases with high cumulative dose of cyclophosphamide, but also in cases with insufficient response. Several studies have shown a higher relapse rate following discontinuation of mycophenolate or in mycophenolate treated subjects that raises concerns about its usefulness in the treatment of AAV. This review describes the efficacy of mycophenolate in AAV as remission induction agent, as remission maintenance agent, and as therapeutic option in relapsing AAV disease, the relapse rate following discontinuation of mycophenolate, and the adverse events related to mycophenolate treatment.
文摘BACKGROUND Systemic lupus erythematosus(SLE)and antineutrophil cytoplasmic antibodyassociated vasculitis(AAV)are classically thought to cause renal impairment and small vessel vasculitis with different pathophysiologies.Their overlap constitutes a rare rheumatologic disease.To date,only dozens of such cases with biopsyproven glomerulonephritis have been reported worldwide typically in women of childbearing age.Here,we present a unique clinical case due to its rarity and individualized treatment of a Chinese man in his eighth decade of life.CASE SUMMARY A 77-year-old man was admitted to several hospitals for shortness of breath and received nonspecific treatments over the past 3 years.As his symptoms were not completely relieved,he visited our hospital for further treatment.Laboratory examinations revealed kidney dysfunction,severe anaemia,hypocomplementemia,glomerular proteinuria,and microscopic haematuria.Antinuclear antibodies,as well as anti-dsDNA antibodies,were positive.Computed tomography of the chest showed right pleural effusion.Renal biopsy was performed,and histology suggested crescentic glomerulonephritis,pauci-immune type.After treatment with plasmapheresis,glucocorticoid,and cyclophosphamide,the disease was in remission,and the patient remained in a stable condition for over 3 years post-hospital discharge.CONCLUSION Due to its complexity and rarity,SLE and AAV overlap syndrome is easily misdiagnosed.An accurate diagnosis and treatment at the earliest stage may significantly improve the condition and reduce irreversible organ injury.
文摘A pancreatic tumor was suspected on the abdominal ultrasound of a 72-year-old man. Abdominal computed tomography showed pancreatic enlargement as well as a diffuse, poorly enhanced area in the pancreas; endoscopic ultrasound-guided fine needle aspiration biopsy and endoscopic retrograde cholangiopancreatography failed to provide a definitive diagnosis. Based on the trend of improvement of the pancreatic enlargement, the treatment plan involved follow-up examinations. Later, he was hospitalized with an alveolar hemorrhage and rapidly progressive glomerulonephritis; he tested positive for myeloperoxidase-anti-neutrophil cytoplasmic antibody(ANCA) and was diagnosed with ANCArelated vasculitis, specifically microscopic polyangiitis. It appears that factors such as thrombus formation caused by the vasculitis in the early stages of ANCArelated vasculitis cause abnormal distribution of the pancreatic blood flow, resulting in non-uniform pancreatitis. Pancreatic lesions in ANCA-related vasculitis are very rare. Only a few cases have been reported previously. Therefore, we report our case and a review of the literature.
文摘Immune-mediated mechanisms are involved in the pathogenesis of both cerebral vasculitis and Parkinson's disease(PD, brainstem-predominant Lewy pathology), but the presentation of cerebral vasculitis with comorbid Lewy pathology has not yet been reported. Here we present a case of pathologically confirmed vasculitis in a 73-year-old male patient whose postmortem examination revealed Lewy pathology diagnostic of PD. This case study suggests a comorbidity of cerebral vasculitis and Lewy pathology, as well as potential pathogenic interactions between these two disorders with immune-mediated mechanisms.
文摘BACKGROUND Infection by the hepatitis C virus(HCV) is currently considered to be a global health issue, with a high worldwide prevalence and causing chronic disease in afflicted individuals. The disease largely involves the liver but it can affect other organs, including the skin. While leukocytoclastic vasculitis has been reported as one of the dermatologic manifestations of HCV infection, there are no reports of this condition as the first symptom of HCV recurrence after liver transplantation.CASE SUMMARY We report here a case of leukocytoclastic vasculitis in a liver transplant recipient on maintenance immunosuppression. The condition presented as a palpable purpura in both lower extremities. Blood and urine cultures were negative and all biochemical tests were normal, excepting evidence of anemia and hypocomplementemia. Imaging examination by computed tomography showed a small volume of ascites, diffuse thickening of bowel walls, and a small bilateral pleural effusion. Skin biopsy showed leukocytoclasia and fibrinoid necrosis.Liver biopsy was suggestive of HCV recurrence in the graft, and HCV polymerase chain reaction yielded 11460 copies/mL and identified the genotype as 1 A. Treatment of the virus with a 12-wk direct-acting antiviral regimen of ribavirin, sofosbuvir and daclatasvir led to regression of the symptoms within the first 10 d and subsequent complete resolution of the symptoms.CONCLUSION This case highlights the difficulties of diagnosing skin lesions caused by HCVinfection in immunosuppressed patients.
