Background： Primary anti-neutrophil cytoplasmic antibody-associated vasculitis （AAV） is a chronic autoimmune disease associated with multisystem dysfunction. Renal involvement is common and closely associated with ...Background： Primary anti-neutrophil cytoplasmic antibody-associated vasculitis （AAV） is a chronic autoimmune disease associated with multisystem dysfunction. Renal involvement is common and closely associated with outcome. The purpose of this study was to investigate the clinical determinants of mortality of patients with AAV-related renal injury in the first 2 years after diagnosis in a single West Chinese center. Methods： Demographic and laboratory parameters of 123 consecutive patients with AAV-related renal injury diagnosed in Renal Division and Institute ofNephrology, Sichuan Provincial People＇s Hospital between 2004 and 2012 were collected retrospectively. All patients were followed up for 2 years after diagnosis. Survivors were compared with nonsurvivors to identify the clinical baseline variables associated with mortality. Multivariate Cox regression model was used to determine the independent predictors of mortality. Results： Of the 123 patients, 46 （37.4%） died by the end of 2 years after diagnosis, with 41 （33.3%） patients dying within the first 12 months. In comparison with the survivors, Birmingham Vasculitis Activity Score （BVAS）, the incidence of pulmonary hemorrhage and digestive system （DS） involvement, serum creatinine, and erythrocyte sedimentation rate were significantly higher in nonsurvivors, whereas lymphocyte counts, hemoglobin, and complement 3 （C3） were significantly lower. Renal replacement therapy was more common in nonsurvivors. High BVAS （hazard ratio [HR] = 1.058, 95% confidence interval [CI]： 1.002-1. I17; P = 0.042）, pulmonary hemorrhage （MR = 1.970, 95% CI： 1.033-3.757; P = 0.04）, DS involvement （HR = 2.911,95% （7：1.212-6.911 ： P = 0.017）4 and serum creatinine 〉400 μmol/L （MR = 2.910, 95% CI： 1.271-6.664, P = 0.012） were independent predictors of death in patients with AAV-related renal injury. Conclusions： Patients with AAV-related renal injury have high early mortality. Those with high BVAS （particularly with pulmonary or DS involvement） and serious renal dysfunction should receive aggressive therapy and careful monitoring to reduce the occurrence of adverse events and improve prognosis.展开更多
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
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
AIM To investigate the prevalence and clinical significance of autoimmune liver disease(ALD)-related autoantibodies in patients with biliary atresia(BA).METHODS Sera of 124 BA patients and 140 age-matched non-BA contr...AIM To investigate the prevalence and clinical significance of autoimmune liver disease(ALD)-related autoantibodies in patients with biliary atresia(BA).METHODS Sera of 124 BA patients and 140 age-matched non-BA controls were assayed for detection of the following autoantibodies: ALD profile and specific anti-nuclear antibodies(ANAs), by line-blot assay; ANA and antineutrophil cytoplasmic antibody(ANCA), by indirect immunofluorescence assay; specific ANCAs and antiM2-3 E, by enzyme linked immunosorbent assay. Associations of these autoantibodies with the clinical features of BA(i.e., cytomegalovirus infection, degree of liver fibrosis, and short-term prognosis of Kasai procedure) were evaluated by Spearman's correlation coefficient.RESULTS The overall positive rate of serum autoantibodies in preoperative BA patients was 56.5%. ALD profile assay showed that the positive reaction to primary biliary cholangitis-related autoantibodies in BA patients was higher than that to autoimmune hepatitis-related autoantibodies. Among these autoantibodies, anti-BPO was detected more frequently in the BA patients than in the controls(14.8% vs 2.2%, P < 0.05). Accordingly, 32(25.8%) of the 124 BA patients also showed a high positive reaction for anti-M2-3 E. By comparison, the controls had a remarkably lower frequency of anti-M2-3 E(P < 0.05), with 6/92(8.6%) of patients with other liver diseases and 2/48(4.2%) of healthy controls. The prevalence of ANA in BA patients was 11.3%, which was higher than that in disease controls(3.3%, P < 0.05), but the reactivity to specific ANAs was only 8.2%. The prevalence of ANCAs(ANCA or specific ANCAs) in BA patients was also remarkably higher than that in the healthy controls(37.9% vs 6.3%, P < 0.05), but showed no difference from that in patients with other cholestasis. ANCA positivity was closely associated with the occurrence of postoperative cholangitis(r = 0.61, P < 0.05), whereas none of the autoantibodies showed a correlation to cytomegalovirus infection or the stages of liver fibrosis.CONCLUSION High prevalence of autoantibodies in the BA developmental process strongly reveals the autoimmunemediated pathogenesis. Serological ANCA positivity may be a useful predictive biomarker of postoperative cholangitis.展开更多
To compare the clinical and pathological manifestations of patients with antineutrophil cytoplasmic autoantibodies (ANCA) directed against proteinas e 3 (anti PR3) or myeloperoxidase (anti MPO). Methods. One hundred a...To compare the clinical and pathological manifestations of patients with antineutrophil cytoplasmic autoantibodies (ANCA) directed against proteinas e 3 (anti PR3) or myeloperoxidase (anti MPO). Methods. One hundred and forty patients with ANCA were detected for anti PR3 a nd anti MPO by ELISA. The clinical features at presentation, histopathological characteristics and outcome of all patients who were tested positive for anti P R3 or anti MPO were analysed.Results. In anti PR3 group (n=21), 16 cases (76.2%) had systemic vasculitis , in which Wegener’s granulomatosis prevailed (13 cases, 61.9%). In anti MPO g roup (n=31), 19 cases (61.3%) were diagnosed as systemic vasculitis and 12 case s (38.7%) as microscopic angiitis. For vasculitic patients with anti PR3 and a nti MPO, the disease duration at diagnosis was 9.6±2.0m and 4.4±0.9m respecti vely, P< 0.05;vasculitis activity index (BVAS) and mean number of affected organ were 22.5±2.1, 5.0±0.4 and 25.1±1.7, 4.8±0.4 respectively, P >0.05;upper r espiratory tract, eye and joint involvements were 11(68.8%), 7(43.8%), 11(68.8 %) and 7(36.8%), 2(10.5%), 5(26.3%) respectively, P< 0.05.Although there was no statistical difference in renal involvement between these two groups, patien ts with serum creatine >500 μmol/L were more commonly seen in anti MPO group t han in anti PR3 group, which were 8(42.1%) and 2(12.5%) respectively, P< 0.05 . Ten relapses were seen in anti PR3 group and only 2 in anti MPO group, but t he acute mortality rate in anti MPO group (5/19, 27.4%) was much higher than t hat in anti PR3 group (1/16, 6.3%). Conclusions. Anti PR3 and anti MPO occurred mainly in systemic vasculitis. A large divergence was seen in the disease spectrum between patients with anti PR 3 and those with anti MPO. In particular, upper respiratory tract, eye and join t involvements, granuloma formation and relapse were more prominent in anti PR3 patients. By contrast, the anti MPO patients had a more acute disease onset, m ore rapid progressive renal involvement and a higher acute mortality rate.展开更多
Objective:In recent years,an increasing number of drugs have been proved to be associated with the induction of anti-neutrophil cytoplasmic antibody(ANCA)-associated vasculitis(AAV).This article reviews the latest res...Objective:In recent years,an increasing number of drugs have been proved to be associated with the induction of anti-neutrophil cytoplasmic antibody(ANCA)-associated vasculitis(AAV).This article reviews the latest research progress on drug-induced AAV.Data sources:We conducted a comprehensive and detailed search of the PubMed database.The search terms mainly included druginduced,ANCA,and vasculitis.Study selection:We summarized the original articles and reviews on drug-induced AAV in recent years.The extracted information included the definition,epidemiology,associated drugs,pathogenesis,clinical features,diagnosis,treatment,and prognosis of druginduced AAV.We also focused on the differences between drug-induced AAV and primary vasculitis.Results:The offending drugs leading to drug-induced AAV are almost from pharmacologic categories and we need to be vigilant when using these drugs.The pathogenesis of drug-induced AAV might be multifactorial.The formation of neutrophil extracellular traps is an important mechanism for the development of drug-induced AAV.The clinical features of drug-induced AAV are similar to those of primary AAV.Understanding the difference between drug-induced AAV and primary AAV is helpful to identify druginduced AAV.Stopping the offending drug at once after diagnosis may be sufficient for those patients with mild symptoms.Immunosuppressive therapy should only be used in patients with vital organs involvement.Conclusions:Patients with drug-induced AAV usually have a good prognosis if they stop using the offending drug immediately.Recent advances in research on AAV are expected to help us better understand the pathogenesis of drug-induced AAV.展开更多
Antithyroid drugs are molecules known as thionamides that inhibit thyroid hormone synthesis by interfering with thyroid peroxidase mediated iodination of tyrosine residues in thyroglobulin. These extensively used drug...Antithyroid drugs are molecules known as thionamides that inhibit thyroid hormone synthesis by interfering with thyroid peroxidase mediated iodination of tyrosine residues in thyroglobulin. These extensively used drugs are associated with a variety of well-known side effects such as anti-neutrophil cytoplasmic antibody （ANCA）-positive vasculitis, granulocytopenia and aplastic anemia. Recently, an atypical hematological finding -- bone marrow plasmacytosis, related to the use of methimazole -- was reported twice in English literatures, but bone marrow plasmacytosis with the use of propylthiouracil （PTU） has hardly been reported so far. Herein we present a case of a patient with Graves＇ disease who was initially investigated for plasma cell dyscrasia but finally diagnosed as PTU-induced bone marrow plasmacytosis with granulocytopenia and ANCA-associated vasculitis.展开更多
BACKGROUND Eosinophilic granulomatosis with polyangiitis(EGPA),formerly known as Churg-Strauss syndrome,is a rare form of anti-neutrophil cytoplasmic antibodyassociated vasculitis characterized by asthma,vasculitis,an...BACKGROUND Eosinophilic granulomatosis with polyangiitis(EGPA),formerly known as Churg-Strauss syndrome,is a rare form of anti-neutrophil cytoplasmic antibodyassociated vasculitis characterized by asthma,vasculitis,and eosinophilia.CASE SUMMARY We report an atypical case of EGPA in a 20-year-old female patient.Unlike previously reported cases of EGPA,this patient’s initial symptom was asthma associated with a respiratory infection.This was followed by Loeffler endocarditis and cardiac insufficiency.She received treatment with methylprednisolone sodium succinate,low molecular weight heparin,recombinant human brain natriuretic peptide,furosemide,cefoperazone sodium/sulbactam sodium,and acyclovir.Despite prophylactic anticoagulation,she developed a large right ventricular thrombus.EGPA diagnosis was confirmed based on ancillary test results and specialty consultations.Subsequent treatment included mycophenolate mofetil.Her overall condition improved significantly after treatment,as evidenced by decreased peripheral blood eosinophils and cardiac markers.She was discharged after 17 d.Her most recent follow-up showed normal peripheral blood eosinophil levels,restored cardiac function,and a reduced cardiac mural thrombus size.CONCLUSION This case illustrates the swift progression of EGPA and underscores the significance of early detection and immediate intervention to ensure a favorable prognosis.展开更多
Background:Follistatin-like 1(FSTL1)plays both pro-inflammatory and anti-inflammatory roles in the inflammatory processes.We investigated whether serum FSTL1 could predict the current anti-neutrophil cytoplasmic antib...Background:Follistatin-like 1(FSTL1)plays both pro-inflammatory and anti-inflammatory roles in the inflammatory processes.We investigated whether serum FSTL1 could predict the current anti-neutrophil cytoplasmic antibody-associated vasculitis(AAV)-specific indices.Methods:We randomly selected 74 patients with AAV from a prospective and observational cohort of Korean patients with AAV.Clinical and laboratory data and AAV-specific indices were recorded.FSTL1 concentration was determined using the stored sera.The lowest tertile of the short-form 36-item health survey(SF-36)was defined as the current low SF-36.The cutoffs of serum FSTL1 for the current low SF-36 physical component summary(PCS)and SF-36 mental component summary(MCS)were extrapolated by the receiver operator characteristic curve.Results:The median age was 62.5 years(55.4%were women).Serum FSTL1 was significantly correlated with SF-36 PCS(r=-0.374),SF-36 MCS(r=-0.377),and C-reactive protein(CRP)(r=0.307),but not with Birmingham vasculitis activity score(BVAS).In the multivariable linear regression analyses,BVAS,CRP,and serum FSTL1 were independently associated with the current SF-36 PCS(β=-0.255,β=-0.430,andβ=-0.266,respectively)and the current SF-36 MCS(β=-0.234,β=-0.229,andβ=-0.296,respectively).Patients with serum FSTL1≥779.8 pg/mL and those with serum FSTL1≥841.6 pg/mL exhibited a significantly higher risk of having the current low SF-36 PCS and SF-36 MCS than those without(relative risk 7.583 and 6.200,respectively).Conclusion:Serum FSTL1 could predict the current functional status in AAV patients.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
文摘Background： Primary anti-neutrophil cytoplasmic antibody-associated vasculitis （AAV） is a chronic autoimmune disease associated with multisystem dysfunction. Renal involvement is common and closely associated with outcome. The purpose of this study was to investigate the clinical determinants of mortality of patients with AAV-related renal injury in the first 2 years after diagnosis in a single West Chinese center. Methods： Demographic and laboratory parameters of 123 consecutive patients with AAV-related renal injury diagnosed in Renal Division and Institute ofNephrology, Sichuan Provincial People＇s Hospital between 2004 and 2012 were collected retrospectively. All patients were followed up for 2 years after diagnosis. Survivors were compared with nonsurvivors to identify the clinical baseline variables associated with mortality. Multivariate Cox regression model was used to determine the independent predictors of mortality. Results： Of the 123 patients, 46 （37.4%） died by the end of 2 years after diagnosis, with 41 （33.3%） patients dying within the first 12 months. In comparison with the survivors, Birmingham Vasculitis Activity Score （BVAS）, the incidence of pulmonary hemorrhage and digestive system （DS） involvement, serum creatinine, and erythrocyte sedimentation rate were significantly higher in nonsurvivors, whereas lymphocyte counts, hemoglobin, and complement 3 （C3） were significantly lower. Renal replacement therapy was more common in nonsurvivors. High BVAS （hazard ratio [HR] = 1.058, 95% confidence interval [CI]： 1.002-1. I17; P = 0.042）, pulmonary hemorrhage （MR = 1.970, 95% CI： 1.033-3.757; P = 0.04）, DS involvement （HR = 2.911,95% （7：1.212-6.911 ： P = 0.017）4 and serum creatinine 〉400 μmol/L （MR = 2.910, 95% CI： 1.271-6.664, P = 0.012） were independent predictors of death in patients with AAV-related renal injury. Conclusions： Patients with AAV-related renal injury have high early mortality. Those with high BVAS （particularly with pulmonary or DS involvement） and serious renal dysfunction should receive aggressive therapy and careful monitoring to reduce the occurrence of adverse events and improve prognosis.
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.
基金the Guangdong Provincial Science and Technology Planning Project,No.2014A020212520the Guangzhou Science and Technology Project,No.201707010014
文摘AIM To investigate the prevalence and clinical significance of autoimmune liver disease(ALD)-related autoantibodies in patients with biliary atresia(BA).METHODS Sera of 124 BA patients and 140 age-matched non-BA controls were assayed for detection of the following autoantibodies: ALD profile and specific anti-nuclear antibodies(ANAs), by line-blot assay; ANA and antineutrophil cytoplasmic antibody(ANCA), by indirect immunofluorescence assay; specific ANCAs and antiM2-3 E, by enzyme linked immunosorbent assay. Associations of these autoantibodies with the clinical features of BA(i.e., cytomegalovirus infection, degree of liver fibrosis, and short-term prognosis of Kasai procedure) were evaluated by Spearman's correlation coefficient.RESULTS The overall positive rate of serum autoantibodies in preoperative BA patients was 56.5%. ALD profile assay showed that the positive reaction to primary biliary cholangitis-related autoantibodies in BA patients was higher than that to autoimmune hepatitis-related autoantibodies. Among these autoantibodies, anti-BPO was detected more frequently in the BA patients than in the controls(14.8% vs 2.2%, P < 0.05). Accordingly, 32(25.8%) of the 124 BA patients also showed a high positive reaction for anti-M2-3 E. By comparison, the controls had a remarkably lower frequency of anti-M2-3 E(P < 0.05), with 6/92(8.6%) of patients with other liver diseases and 2/48(4.2%) of healthy controls. The prevalence of ANA in BA patients was 11.3%, which was higher than that in disease controls(3.3%, P < 0.05), but the reactivity to specific ANAs was only 8.2%. The prevalence of ANCAs(ANCA or specific ANCAs) in BA patients was also remarkably higher than that in the healthy controls(37.9% vs 6.3%, P < 0.05), but showed no difference from that in patients with other cholestasis. ANCA positivity was closely associated with the occurrence of postoperative cholangitis(r = 0.61, P < 0.05), whereas none of the autoantibodies showed a correlation to cytomegalovirus infection or the stages of liver fibrosis.CONCLUSION High prevalence of autoantibodies in the BA developmental process strongly reveals the autoimmunemediated pathogenesis. Serological ANCA positivity may be a useful predictive biomarker of postoperative cholangitis.
文摘To compare the clinical and pathological manifestations of patients with antineutrophil cytoplasmic autoantibodies (ANCA) directed against proteinas e 3 (anti PR3) or myeloperoxidase (anti MPO). Methods. One hundred and forty patients with ANCA were detected for anti PR3 a nd anti MPO by ELISA. The clinical features at presentation, histopathological characteristics and outcome of all patients who were tested positive for anti P R3 or anti MPO were analysed.Results. In anti PR3 group (n=21), 16 cases (76.2%) had systemic vasculitis , in which Wegener’s granulomatosis prevailed (13 cases, 61.9%). In anti MPO g roup (n=31), 19 cases (61.3%) were diagnosed as systemic vasculitis and 12 case s (38.7%) as microscopic angiitis. For vasculitic patients with anti PR3 and a nti MPO, the disease duration at diagnosis was 9.6±2.0m and 4.4±0.9m respecti vely, P< 0.05;vasculitis activity index (BVAS) and mean number of affected organ were 22.5±2.1, 5.0±0.4 and 25.1±1.7, 4.8±0.4 respectively, P >0.05;upper r espiratory tract, eye and joint involvements were 11(68.8%), 7(43.8%), 11(68.8 %) and 7(36.8%), 2(10.5%), 5(26.3%) respectively, P< 0.05.Although there was no statistical difference in renal involvement between these two groups, patien ts with serum creatine >500 μmol/L were more commonly seen in anti MPO group t han in anti PR3 group, which were 8(42.1%) and 2(12.5%) respectively, P< 0.05 . Ten relapses were seen in anti PR3 group and only 2 in anti MPO group, but t he acute mortality rate in anti MPO group (5/19, 27.4%) was much higher than t hat in anti PR3 group (1/16, 6.3%). Conclusions. Anti PR3 and anti MPO occurred mainly in systemic vasculitis. A large divergence was seen in the disease spectrum between patients with anti PR 3 and those with anti MPO. In particular, upper respiratory tract, eye and join t involvements, granuloma formation and relapse were more prominent in anti PR3 patients. By contrast, the anti MPO patients had a more acute disease onset, m ore rapid progressive renal involvement and a higher acute mortality rate.
文摘Objective:In recent years,an increasing number of drugs have been proved to be associated with the induction of anti-neutrophil cytoplasmic antibody(ANCA)-associated vasculitis(AAV).This article reviews the latest research progress on drug-induced AAV.Data sources:We conducted a comprehensive and detailed search of the PubMed database.The search terms mainly included druginduced,ANCA,and vasculitis.Study selection:We summarized the original articles and reviews on drug-induced AAV in recent years.The extracted information included the definition,epidemiology,associated drugs,pathogenesis,clinical features,diagnosis,treatment,and prognosis of druginduced AAV.We also focused on the differences between drug-induced AAV and primary vasculitis.Results:The offending drugs leading to drug-induced AAV are almost from pharmacologic categories and we need to be vigilant when using these drugs.The pathogenesis of drug-induced AAV might be multifactorial.The formation of neutrophil extracellular traps is an important mechanism for the development of drug-induced AAV.The clinical features of drug-induced AAV are similar to those of primary AAV.Understanding the difference between drug-induced AAV and primary AAV is helpful to identify druginduced AAV.Stopping the offending drug at once after diagnosis may be sufficient for those patients with mild symptoms.Immunosuppressive therapy should only be used in patients with vital organs involvement.Conclusions:Patients with drug-induced AAV usually have a good prognosis if they stop using the offending drug immediately.Recent advances in research on AAV are expected to help us better understand the pathogenesis of drug-induced AAV.
文摘Antithyroid drugs are molecules known as thionamides that inhibit thyroid hormone synthesis by interfering with thyroid peroxidase mediated iodination of tyrosine residues in thyroglobulin. These extensively used drugs are associated with a variety of well-known side effects such as anti-neutrophil cytoplasmic antibody （ANCA）-positive vasculitis, granulocytopenia and aplastic anemia. Recently, an atypical hematological finding -- bone marrow plasmacytosis, related to the use of methimazole -- was reported twice in English literatures, but bone marrow plasmacytosis with the use of propylthiouracil （PTU） has hardly been reported so far. Herein we present a case of a patient with Graves＇ disease who was initially investigated for plasma cell dyscrasia but finally diagnosed as PTU-induced bone marrow plasmacytosis with granulocytopenia and ANCA-associated vasculitis.
基金Supported by the 2020 National Natural Science Foundation Cultivation Project of Guizhou Medical University Affiliated Hospital,No.gyfynsfc[2020]-14.
文摘BACKGROUND Eosinophilic granulomatosis with polyangiitis(EGPA),formerly known as Churg-Strauss syndrome,is a rare form of anti-neutrophil cytoplasmic antibodyassociated vasculitis characterized by asthma,vasculitis,and eosinophilia.CASE SUMMARY We report an atypical case of EGPA in a 20-year-old female patient.Unlike previously reported cases of EGPA,this patient’s initial symptom was asthma associated with a respiratory infection.This was followed by Loeffler endocarditis and cardiac insufficiency.She received treatment with methylprednisolone sodium succinate,low molecular weight heparin,recombinant human brain natriuretic peptide,furosemide,cefoperazone sodium/sulbactam sodium,and acyclovir.Despite prophylactic anticoagulation,she developed a large right ventricular thrombus.EGPA diagnosis was confirmed based on ancillary test results and specialty consultations.Subsequent treatment included mycophenolate mofetil.Her overall condition improved significantly after treatment,as evidenced by decreased peripheral blood eosinophils and cardiac markers.She was discharged after 17 d.Her most recent follow-up showed normal peripheral blood eosinophil levels,restored cardiac function,and a reduced cardiac mural thrombus size.CONCLUSION This case illustrates the swift progression of EGPA and underscores the significance of early detection and immediate intervention to ensure a favorable prognosis.
基金supported by a faculty research grant from the Yonsei University College of Medicine(No.6-2019-0184)a grant from the Korea Health Technology R&D Project through the Korea Health Industry Development Institute,funded by the Ministry of Health and Welfare,Republic of Korea(No.HI14C1324)。
文摘Background:Follistatin-like 1(FSTL1)plays both pro-inflammatory and anti-inflammatory roles in the inflammatory processes.We investigated whether serum FSTL1 could predict the current anti-neutrophil cytoplasmic antibody-associated vasculitis(AAV)-specific indices.Methods:We randomly selected 74 patients with AAV from a prospective and observational cohort of Korean patients with AAV.Clinical and laboratory data and AAV-specific indices were recorded.FSTL1 concentration was determined using the stored sera.The lowest tertile of the short-form 36-item health survey(SF-36)was defined as the current low SF-36.The cutoffs of serum FSTL1 for the current low SF-36 physical component summary(PCS)and SF-36 mental component summary(MCS)were extrapolated by the receiver operator characteristic curve.Results:The median age was 62.5 years(55.4%were women).Serum FSTL1 was significantly correlated with SF-36 PCS(r=-0.374),SF-36 MCS(r=-0.377),and C-reactive protein(CRP)(r=0.307),but not with Birmingham vasculitis activity score(BVAS).In the multivariable linear regression analyses,BVAS,CRP,and serum FSTL1 were independently associated with the current SF-36 PCS(β=-0.255,β=-0.430,andβ=-0.266,respectively)and the current SF-36 MCS(β=-0.234,β=-0.229,andβ=-0.296,respectively).Patients with serum FSTL1≥779.8 pg/mL and those with serum FSTL1≥841.6 pg/mL exhibited a significantly higher risk of having the current low SF-36 PCS and SF-36 MCS than those without(relative risk 7.583 and 6.200,respectively).Conclusion:Serum FSTL1 could predict the current functional status in AAV patients.
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
