Factors Associated With Relapses and Performance of the French Vasculitis Study Group Relapse Score in a Cohort of Mexican Patients With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.

AB0461 ANCA-ASSOCIATED VASCULITIS: CLINICAL FEATURES, RELAPSE, ORGAN DAMAGE AND SURVIVAL IN 197 PATIENTS

47-Year-Old Woman With Bilateral Flank Pain

AB0475 CLINICAL RELEVANCE OF CLINICOPATHOLOGICAL PHENOTYPE AND ANTIBODY SPECIFICITY IN ANCA-ASSOCIATED VASCULITIS

OP0232 Outcomes Following Renal Transplantation (RTX) in Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis (AAV): An Analysis of 46 Patients

Comparative proteomic analysis of neutrophils from patients with microscopic polyangiitis and granulomatosis with polyangiitis

Background and Aims We investigated the incidence rate and type of cancer in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in our Institute. Method We retrospectively included 210 patients with AAV [106 patients with microscopic polyangiitis (MPA), 93 with granulomatosis with polyangiitis (GPA) and 11 patients with eosinophilic GPA (EGPA)], and reviewed their medical records. We collected demographic, clinical and laboratory data. We selected patients with history of cancer, patients diagnosed with paraneoplastic AAV and patients who developed malignant disease after AAV. We reviewed the use of glucocorticoid and immunosuppressive drugs. Results Between 1993-2024 there were 16 patients with AAV who had cancer during their life in our Department. There were eight patients with malignancy-associated AAV. Two patients were simultaneously diagnosed with kidney carcinoma and vasculitis. One patient's serum was positive for cytoplasmatic ANCA, the other patient's for pANCA. Complete resection of cancer and renal biopsy was performed, they got steroid monotherapy. Five patients were diagnosed with pANCA vasculitis a few months (1-5) after the diagnosis of malignancies (two pts with breast carcinoma, two pts with ovarium carcinoma, one patient with ventricular carcinoma). In one case the pAAV was established after 5 months of oropharyngeal tumor's resection. Two patients had history of malignancy (one pts had colon carcinoma 4 ys before the appearance of AAV, the other patient had breast carcinoma 10 ys before the AAV). In both cases there were examinations for malignancy with negative results. In these cases the vasculitis was not associated with carcinoma. The therapeutic approach included the use of corticosteroids, plasmapheresis, and cyclophosphamide or rituximab. Six patients were observed malignancy 0.5-10 years after the diagnosis of AAV. There was one pancreas, one ventricular, two lung and two bladder carcinoma. Conclusion In our study we focused on the relationship of malignancy and AAV. Between the 210 patients with AAV eight cases (3.8%) were malignancy-associated, in one case cANCA associated, the other cases pANCA was presented. Treating paraneoplastic vasculitis can be challenging. Individualized treatment strategy tailored to patients' needs is crucial. Six patients were diagnosed with solid tumor during the follow-up. The cumulative overall cancer incidence rates were 1.4% and 2.8% at 5 and 10 years, respectively. Only in one patient with bladder cancer was treated with the cumulative CYC doses ≥ 36 g. No leukemia or non melanotic skin cancer (NMSC) was represented contrary to other studies.

BackgroundIn 2022 the American College of Rheumatology (ACR) and European Alliance of Associations for Rheumatology (EULAR) presented new classification criteria for the three subsets of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis...

BackgroundAnti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis is a life- or organ-threatening condition in which patients experience severe inflammation of small- to medium-sized blood vessels. Clinical relapses are common in ANCA-associated vasculitis...

Background/Purpose:Comparisons of pediatric ANCA‐associated vasculitis subtypes (AAV) are limited by the paucity of reported cases, standardized definitions, and overlapping classification criteria. Published work from ARChiVe (A Registry for Childhood Vasculitis) demonstrated modifications of validated classification algorithms applied to pediatric patients with AAV can classify each patient to mutually exclusive diagnostic categories. We compared presenting features of children with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) classified according to this methodology.Methods:A pediatric modification of the European Medicines Agency (EMA) algorithm for classifying AAV and polyarteritis nodosa (incorporating the EULAR/PRINTO/PRES pediatric classification criteria for GPA) was applied to patients in ARChiVe censored to April 2012. We compared characteristics of patients classified as having MPA and GPA. STATA (Statcorp, 2013) was used to calculate frequencies, percentages, and chi‐squared with fisher's exact for categorical variables and means, standard deviations, and t‐tests for continuous variables.Results:One hundred fifty‐two of 227 children in ARChiVe met criteria for diagnosis of MPA (n = 22) or GPA (n = 130). Characteristics and presenting features are shown in Table . Children with MPA were younger at diagnosis (mean diff. 2.7y, p = <0.01). Renal involvement was predominant in both groups. Renal biopsies in 40% of both groups were consistent with pauci‐immune, necrotizing glomerulonephritis. Children with MPA had higher rates of nephrosis, renal failure requiring dialysis, and abnormal creatinine clearance (Table ). Upper and lower airway involvement was more prevalent among those with GPA largely in accordance with surrogate GPA features used to differentiate GPA and MPA in the EMA algorithm. The majority of patients presented with constitutional symptoms, however, other organ systems were less frequently involved. Most patients received combination therapy corticosteroids and cytoxan (64% MPA, 81% GPA) with additional plasmapheresis (29% MPA, 21% GPA), rituximab (14% MPA, 3% GPA) or methotrexate (7% MPA, 1% GPA). The remainder of children received combination corticosteroids and methotrexate or rituximab, without cytoxan (12% MPA, 11% GPA). A larger proportion of patients with MPA received antihypertensive agents and/or ACE inhibitors (64% vs 35%, p = 0.01). Characteristics & Presenting Clinical Features of children with microscopic polyangiitis or granulomatosis with polyangiitis in the ARChiVe cohort (n = 152) Algorithm‐derived diagnosis Characteristic/Feature MPA (n = 22) GPA (n = 130) p‐value Female, n(%) 15 (68) 83 (64) 0.81 Caucasian, n (%) 11 (50) 85 (66) 0.231 Age at diagnosis, yrs, mean (sd) 11.6 (5) 14.2 (3) <0.01* Median (range) 12.8 (9–15) 14.9 (4–19) Symptom duration prior to diagnosis, mos, mean (sd) 4.4 (9) 5.2 (10) 0.72 Median (range) 3.4 (0–35.8) 4.8 (0–66.8) MD‐assigned diagnosis (clinical diagnosis) MPA or isolated MPA 8 (36) 18 (14) 0.027* WG or limited WG 7 (32) 107 (82) <0.01* ANCA 3 (14) 1 (1) 0.01* Unclassified 4 (18) 4 (3) 0.02* General Features Fatigue 19 (86) 113 (87) 1.00 Fever 10 (46) 71 (55) 0.49 Weight loss 10 (46) 60 (46) 1.00 Renal 18 (82) 103 (79) 1.00 Hypertension 8 (36) 28 (22) 0.17 Hematuria and proteinuria with red blood cell casts 16 (73) 97 (75) 0.80 Nephrotic range proteinuria with edema 6 (27) 14 (11) 0.04* Renal failure requiring dialysis 7 (32) 17 (13) 0.05* Creatinine clearance >25% lower limit of normal 8 (89) 18 (36) <0.01* Biopsy‐proven glomerulonephritis 6 (27) 28 (22) 0.58 Pulmonary 9 (41) 105 (81) <0.01* Chronic cough 6 (27) 79 (61) <0.01* Shortness of breath 4 (18) 63 (49) 0.01* Hemoptysis/alveolar hemorrhage 3 (14) 54 (42) 0.02* Fixed pulmonary infiltrates ± cavitations, nodules 4 (18) 99 (76) <0.01* Head, Ear, Nose, & Throat 4 (18) 97 (75) <0.01* Bloody nasal discharge ± crusting 0 (0) 68 (52) <0.01* Chronic sinusitis, otitis, or mastoiditis 0 (0) 63 (48) <0.01* Subglottic involvement 0 (0) 16 (12) 0.13 Cranial bone ± cartilage destruction 0 (0) 10 (8) 0.36 Acute hearing loss 0 (0) 15 (12) 0.13 Red ± painful eye conditions 2 (9) 34 (26) Gastrointestinal/Abdominal 15 (68) 51 (39) 0.01* Chronic nausea 9 (41) 17 (13) <0.01* Skin 10 (45) 71 (55) 0.49 Musculoskeletal 80 (62) 14 (64) 1.00 Nervous System 35 (27) 6 (27) 1.00 Cardiovascular 9 (7) 1 (5) 1.00 ANCA Serology pANCA or anti‐MPO 14 (70) 35 (29) <0.01 cANCA or anti‐PR3 6 (30) 86 (71) <0.01Conclusion:Children with AAV had predominantly renal and constitutional manifestations. Younger age and a more severe renal disease phenotype may characterize MPA with patients requiring additional treatment for the consequences of kidney disease. The wide variations in time to diagnosis continue to suggest that pediatric AAV is poorly recognized. Ongoing biomarker‐driven studies may complement systems for subclassifying patients with AAV.

ANCA-associated renal vasculitis

BackgroundAnti-B-cell therapy with rituximab (RTX) plays an important role in the induction and maintenance therapy of ANCA- associated vasculitis (AAV). Late-onset neutropenia (LON) has been reported following RTX therapy.ObjectivesBased on...

ObjectiveTo develop a score assessing the probability of relapse in granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA).MethodsLong-term follow-up data from GPA and MPA patients included in five consecutive randomised...

Background:In spite of significantly improved therapy during the last years, long-term morbidity and mortality has remained high in ANCA-associated vasculitis (AAV). The main causes of death within the first year...

AB0153 Comparative proteomic analysis of neutrophils from patients with microscopic polyangiitis and granulomatosis with polyangiitis

BackgroundAntineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have overlapping manifestations. Classifications based on clinical criteria or ANCA specificity have emerged to individualize homogenized group of patients in terms of clinical forms...