Adolescent ; Antibodies, Antineutrophil Cytoplasmic ; analysis ; Child ; Granulomatosis with Polyangiitis ; complications ; Humans ; Lung Diseases ; etiology ; Male

OBJECTIVE: To investigate the significance of a set of seven disease activities and extension measurements and their correlations between one and another for anti-neutrophil cytoplasmic autoantibody associated vasculitis (AAV). METHODS: A total of 121 patients from Peking University International Hospital and Fouth Medical Center of PLA General Hospital with confirmed diagnoses of AAV clinically were enrolled in the study, including 15 cases of eosinophilic granulomatous with polyangiitis (EGPA), 59 cases of granulomatous with polyangiitis (GPA) and 47 cases of microscopic polyangiitis (MPA). A hundred and twenty-one AAV patients were divided into death group and survival group according to their survival conditions. A set of seven disease assessment scales including Birmingham vasculitis activity score (BVAS)-1994, BVAS-2003, as well as BVAS/GPA, vasculitis damage index (VDI), disease extent index (DEI), five factor score (FFS)-1996, and FFS-2009 were measured and scored one by one, and their relationships which were represented by Spearman correlation coefficient were compared between one and another. RESULTS: BVAS-1994, BVAS-2003, as well as BVAS/GPA, VDI, DEI, and FFS, all of those seven evaluation indexes of the AAV patients in the death group were significantly higher than those in the survival group (P<0.05). Except for BVAS/GPA, all those above indicators in the patients with EGPA were lower than those in the patients with GPA and those in the patients with MPA, and those in all of the AAV patients as a whole group. There were high correlations among BVAS-2003, BVAS-1994 and BVAS/GPA (r values were 0.9 and 0.7, respectively); BVAS-1994 was fairly correlated with BVAS/GPA (r=0.69); FFS-1996 and FFS-2009 were highly correlated (r=0.73) with each other; BVAS-1994, BVAS-2003 and BVAS/GPA were fairly correlated with DEI (with r values of 0.62, 0.65, and 0.62, respectively); VDI was also fairly correlated with BVAS-1994 and with BVAS-2003 (r values were 0.49 and 0.52, respectively). CONCLUSION All of those seven AAV assessment indicators above can be used as indicators of disease activity and prognosis in AAV patients, most of which were relevant within one and another. There were high correlations among BVAS-2003, BVAS-1994 and BVAS/GPA, and besides, there were also high correlations between FFS-1996 and FFS-2009.
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis* ; Antibodies, Antineutrophil Cytoplasmic ; Autoantibodies ; Biomarkers/analysis* ; Humans ; Microscopic Polyangiitis ; Neutrophils

To explore the clinical diagnostic efficacy of antineutrophil cytoplasmic antibody associated vasculitis (AAV) by comparing the consistency and coincidence rate of serum anti-myeloperoxidase (MPO) antibody and anti-protease 3 (PR3) antibody detected by digital liquid chip method (DLCM) and enzyme-linked immunosorbent assay (ELISA). To provide reference for the selection of detection methods of anti-MPO antibody and anti-PR3 antibody in clinical laboratory. This study is a cross-sectional study, a total of 307 cases of antineutrophil cytoplasmic antibodies were detected in the Department of Clinical Immunology, West China Hospital of Sichuan University from January to March 2021. The serum samples and related clinical information were collected. At the same time, the levels of anti-MPO antibody and anti-PR3 antibody in serum samples were detected by ELISA and DLCM, indirect immunofluorescence (IIF) was used to re-test the differential samples between the two methods. SPSS 26.0 was used to analyze the test results, Cohen's kappa coefficient analysis was used to compare the consistency of the two methods, and paired chi-square test was used to compare the sensitivity and specificity of the two methods to AAV. The results showed that the positive cases of anti-MPO antibody detected by ELISA and DLCM were 63 and 44, and the negative cases were 244 and 263; the positive cases of anti-PR3 antibody detected by ELISA and DLCM were 34 and 28, and the negative cases were 273 and 279. The results of anti-MPO antibody and anti-PR3 antibody detected by the two methods had good consistency and coincidence rate, in which the total coincidence rate of anti-MPO antibody was 92.51%, the positive coincidence rate was 66.67%, and the negative coincidence rate was 99.18%. The results of consistency analysis showed that kappa=0.741 had well consistency. The total coincidence rate of anti-PR3 antibody is 96.74%, the positive coincidence rate is 76.47%, and the negative coincidence rate is 99.27%. The consistency analysis results show that kappa=0.821 had strong consistency. The results of IIF re-test of differential samples showed that the coincidence rate between DLCM and IIF was higher. The results of comparative analysis of anti-MPO antibody and anti-PR3 antibody showed that the specificity of DLCM was better than that of ELISA, and its sensitivity was lower than that of ELISA. In conclusion, the results of anti-MPO antibody and anti-PR3 antibody detected by DLCM were consistent with those of ELISA. In the combined detection of anti-MPO antibody and anti-PR3 antibody, the specificity of DLCM is better than that of ELISA.
Humans ; Antibodies, Antineutrophil Cytoplasmic/analysis* ; Myeloblastin ; Cross-Sectional Studies ; Sensitivity and Specificity ; Fluorescent Antibody Technique, Indirect ; Enzyme-Linked Immunosorbent Assay/methods*

OBJECTIVES: Platelet-to-lymphocyte ratio (PLR) has recently been investigated as a new inflammatory marker in many inflammatory diseases, including systemic lupus erythematosus and immunoglobulin A vasculitis. However, there were very few reports regarding the clinical role of PLR in patients with anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis. This study was thus undertaken to investigate the relationship between inflammatory response and disease activity in Chinese patients with myeloperoxidase-anti-neutrophil cytoplasmic antibody (MPO-ANCA) associated vasculitis. Furthermore, we evaluated whether PLR predicts the progression of end stage of renal disease (ESRD) and all-cause mortality. METHODS: The clinical, laboratory and pathological data, and the outcomes of MPO-ANCA associated vasculitis patients were collected. The Spearman correlation coefficient was computed to examine the association between 2 continuous variables. Cox regression analysis was used to estimate the association between PLR and ESRD or all-cause mortality. RESULTS: A total of 190 consecutive patients with MPO-ANCA associated vasculitis were included in this study. Baseline PLR was positively correlated with CRP (r=0.333, P<0.001) and ESR (r=0.218, P=0.003). PLR had no obvious correlation with Birmingham Vasculitis Activity Score (BVAS). Patients having PLR≥330 exhibited better cumulative renal survival rates than those having PLR<330 (P=0.017). However, there was no significant difference in the cumulative patient survival rates between patients with PLR≥330 and those with PLR<330 at diagnosis (P>0.05). In multivariate analysis, PLR is associated with the decreased risk of ESRD (P=0.038, HR=0.518, 95% CI 0.278 to 0.963). We did not find an association between PLR with all-cause mortality using multivariate analysis (HR=1.081, 95% CI 0.591 to 1.976, P=0.801). CONCLUSIONS PLR is positively correlated with CRP and ESR. Furthermore, PLR may independently predict the risk of ESRD.
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis* ; Antibodies, Antineutrophil Cytoplasmic/analysis* ; China/epidemiology* ; Humans ; Kidney Failure, Chronic/complications* ; Lymphocytes ; Peroxidase ; Retrospective Studies

'Pauci-immune' glomerulonephritis has been recognized as an important cause of rapidly progressive glomerulonephritis. The paucity of immune deposits can be separated from the other two major immunohistologic variants of crescentic glomerulonephritis, ie, antiglomerular basement membrane (GBM) antibody-mediated and immune complex-mediated glomerulonephritis. Here we describe the case of a 42-year-old woman with pauci-immune' glomerulonephritis and vasculitis presenting as rapidly progressive renal failure with characteristic pathologic and immunohistologic findings. And in this case, despite oliguria and rapid deterioration of renal function, the renal function recovered partially and continued to be stabilized with a favourable response to hemodialysis and combined system immunosuppressive therapy.
Adult ; Antibodies, Antineutrophil Cytoplasmic ; Autoantibodies/analysis ; Cyclophosphamide/therapeutic use ; Drug Therapy, Combination ; Female ; Glomerulonephritis/*complications/immunology/pathology/therapy ; Humans ; Kidney/ultrastructure ; Prednisolone/therapeutic use ; Renal Dialysis ; Vasculitis/*complications

A case is presented of Wegener's granulomatosis limited to the testis and epididymis, simultaneously, in a 69-year-old man. Orchiectomy was carried out through an inguinal incision under the presumptive diagnosis of a right testicular tumor. A hard, irregular mass occupied the upper testicle and a portion of the epididymal head was visualized. Histopathologic examination of the specimen showed granulomatous inflammation of the testis and epididymis with prominent angiocentric granulomata in the walls of arteries, veins and foci of fibrinoid necrosis, surrounded by palisading inflammatory cells with a few giant cells. The diagnosis of limited Wegener's granulomatosis was considered, although antineutrophil cytoplasmic antibody (c-ANCA) test was negative 2 weeks after orchiectomy. The patient showed an excellent response after local complete excision. He remains free of disease 18 months after orchiectomy.
Aged ; Antibodies, Antineutrophil Cytoplasmic ; analysis ; Epididymis ; Genital Diseases, Male ; pathology ; surgery ; Granulomatosis with Polyangiitis ; pathology ; surgery ; Humans ; Male ; Orchiectomy ; Testicular Diseases ; pathology ; surgery

OBJECTIVETo investigate the clinical characteristics of childhood antineutrophil cytoplasmic autoantibody (ANCA)-associated rapidly progressive glomerulonephritis.

METHODSThe medical data, including clinical manifestations, laboratory findings, and kidney pathology, of 7 children with ANCA-associated rapidly progressive glomerulonephritis were retrospectively studied.

RESULTSThe 7 patients (6 girls and 1 boy) ranged in age from 3.5-14 years, with a mean age of 9 years. A diversity of major complaints and clinical symptoms was presented in the patients. Laboratory findings were not specific. All patients had elevated ESR, BUN and serum creatinine levels as well as anaemia, hematuria and proteinuria. Urinary protein electrophoresis showed mixed proteinuria in the 7 cases. C3 was normal in 3 cases and slightly decreased in 4 cases. All were MPO-ANCA positive, and 1 out of the 7 cases was positive for PR3-ANCA. Renal biopsy displayed extensive crescentic formations and necrotic glomerulus capillary loop. A great quantity of inflammatory cell infiltration and swollen endotheliocytes of small vessels as well as vessel wall edema or necrosis were found in the interstitium. Immunofluorescence showed no or little amounts of immune complex depositions in the renal glomeruli and vessel walls. Renal function was recovered and hematuria/proteinuria disappeared or greatly relieved in 3 patients after methylprednisone and cyclophosphamide pulse therapy.

CONCLUSIONSChildren with ANCA-positive rapidly progressive glomerulonephritis present with various clinical manifestations. The diagnosis of this disorder may be difficult due to a lack of specificity in its clinical manifestations. It is important to enhance our understanding of this disorder to effectively make an early diagnosis.

Adolescent ; Antibodies, Antineutrophil Cytoplasmic ; analysis ; Biopsy ; Child ; Child, Preschool ; Disease Progression ; Female ; Glomerulonephritis ; drug therapy ; immunology ; pathology ; Humans ; Kidney ; pathology ; Male ; Prednisone ; therapeutic use

Antibodies, Antineutrophil Cytoplasmic ; analysis ; immunology ; Antibodies, Antinuclear ; analysis ; immunology ; Antigens, Nuclear ; immunology ; Arthritis, Juvenile ; immunology ; Autoantibodies ; analysis ; immunology ; Humans ; Monitoring, Physiologic ; methods ; Nucleosomes ; immunology ; Phospholipids ; immunology ; Rheumatic Diseases ; immunology ; physiopathology

BACKGROUND/AIMS: Combined measurement of perinuclear antineutrophil cytoplasmic autoantibodies (pANCA) and anti-Saccharomyces cereviseae mannan antibodies (ASCA) has recently been suggested as a valuable diagnostic approach to inflammatory bowel disease (IBD) in the pediatric age group. The aim of this study was to test the accuracy of the assay using pANCA and ASCA in diagnosing pediatric ulcerative colitis (UC) and Crohn's disease (CD). METHODS: Sera were collected from 25 patients with IBD (17 with CD, 8 with UC) and 32 healthy controls. The levels of pANCA and ASCA were determined by using a standard indirect immunofluorescence technique on ethanol-fixed granulocytes and an ELISA assay, respectively. RESULTS: In patients with UC, the sensitivity, specificity, and positive predictive value of the pANCA test were 38%, 88%, and 60%, respectively. Such values were not changed significantly in the case of positive pANCA and negative ASCA. The sensitivity, specificity, and positive predictive value of ASCA test in diagnosing CD were 71%, 88%, and 92%, respectively. The combination of pANCA negative and ASCA positive was not significant. CONCLUSIONS: ASCA and pANCA assays are highly disease specific for CD and UC, respectively. These serological tests can assist clinicians in diagnosing and categorizing patients with IBD and may be useful in making therapeutic decisions.
Adolescent ; Antibodies, Antineutrophil Cytoplasmic/*analysis ; Antibodies, Fungal/*analysis ; Child ; Child, Preschool ; Colitis, Ulcerative/*diagnosis ; Crohn Disease/*diagnosis ; Enzyme-Linked Immunosorbent Assay ; Female ; Fluorescent Antibody Technique ; Humans ; Male ; Mannans/immunology ; Predictive Value of Tests ; Saccharomyces cerevisiae/*immunology ; Sensitivity and Specificity

OBJECTIVETo compare the clinical and pathological manifestations of patients with antineutrophil cytoplasmic autoantibodies (ANCA) directed against proteinase 3 (anti-PR3) or myeloperoxidase (anti-MPO).

METHODSOne hundred and forty patients with ANCA were detected for anti-PR3 and anti-MPO by ELISA. The clinical features at presentation, histopathological characteristics and outcome of all patients who were tested positive for anti-PR3 or anti-MPO were analysed.

RESULTSIn 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 group (n = 31), 19 cases (61.3%) were diagnosed as systemic vasculitis and 12 cases (38.7%) as microscopic angiitis. For vasculitic patients with anti-PR3 and anti-MPO, the disease duration at diagnosis was 9.6 +/- 2.0 m and 4.4 +/- 0.9 m respectively, 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 respiratory 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, patients with serum creatine > 500 micromol/L were more commonly seen in anti-MPO group than 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 the acute mortality rate in anti-MPO group (5/19, 27.4%) was much higher than that in anti-PR3 group (1/16, 6.3%).

CONCLUSIONSAnti-PR3 and anti-MPO occurred mainly in systemic vasculitis. A large divergence was seen in the disease spectrum between patients with anti-PR3 and those with anti-MPO. In particular, upper respiratory tract, eye and joint involvements, granuloma formation and relapse were more prominent in anti-PR3 patients. By contrast, the anti-MPO patients had a more acute disease onset, more rapid progressive renal involvement and a higher acute mortality rate.

Antibodies, Antineutrophil Cytoplasmic ; analysis ; Autoantibodies ; analysis ; Follow-Up Studies ; Granulomatosis with Polyangiitis ; drug therapy ; immunology ; pathology ; Humans ; Immunosuppressive Agents ; therapeutic use ; Kidney ; pathology ; Myeloblastin ; Peroxidase ; immunology ; Respiratory System ; pathology ; Serine Endopeptidases ; immunology ; Vasculitis ; drug therapy ; immunology ; pathology