THU0301 Safety following initiation of rituximab in granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA): interim analysis of the rituximab in anca-associated vasculitis registry (RAVER)

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is an autoimmune disorder which affects small- and, to a lesser degree, medium-sized vessels. ANCA-associated vasculitis encompasses three disease phenotypes: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). This classification is largely based on clinical presentations and has several limitations. Recent research provided evidence that genetic background, risk of relapse, prognosis, and co-morbidities are more closely related to the ANCA serotype, proteinase 3 (PR3)-ANCA and myeloperoxidase (MPO)-ANCA, compared to the disease phenotypes GPA or MPA. This finding has been extended to the investigation of biomarkers predicting disease activity, which again more closely relate to the ANCA serotype. Discoveries related to the immunopathogenesis translated into clinical practice as targeted therapies are on the rise. This review will summarize the current understanding of the immunopathogenesis of ANCA-associated vasculitis and the interplay between ANCA serotype and proposed disease biomarkers and illustrate how the extending knowledge of the immunopathogenesis will likely translate into development of a personalized medicine approach in the management of ANCA-associated vasculitis.

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CLINICAL AND LABORATORY FEATURES OF ANCA ASSOCIATED VASCULITIS: EXPERIENCE AT A TERTIARY CARE HOSPITAL IN LAHORE, PAKISTAN

Pakistan Armed Forces Medical Journal ◽

10.51253/pafmj.v71i1.3157 ◽

2021 ◽

Vol 71 (1) ◽

pp. 29-33

Author(s):

Muhammad Zeeshan Aslam ◽

Asadullah Kakkar ◽

Haseeb Ahmed Khan ◽

Saira Elaine Anwar Khan

Keyword(s):

Respiratory Symptoms ◽

Granulomatosis With Polyangiitis ◽

Microscopic Polyangiitis ◽

Renal Involvement ◽

Tertiary Care ◽

Care Hospital ◽

X Rays ◽

Neurologic Manifestation ◽

Anca Associated Vasculitis ◽

Laboratory Features

Objective: To determine the clinical and laboratory features during the disease course in patients of anticytoplasmic antibody (ANCA) associated vasculitis in Pakistani patients presenting to a tertiary care center. Study Design: Case series. Place and Duration of Study: Fatima Memorial Hospital Shadman Lahore, from Dec 2018 to Mar 2019. Methodology: A collection of 20 patients regarding demographic data, constitutional symptoms, mucocutaneous symptoms and signs, upper respiratory symptoms, lower respiratory symptoms, orbital and ocular manifestation, cardiovascular, peripheral vascular manifestation, central and peripheral neurologic manifestation, abdominal manifestation and renal manifestation Results: Sixteen patients (80%) had a diagnosis of Granulomatosis with Polyangiitis, and 4 patients (20%) wereof microscopic polyangiitis. The most common systemic involvement in descending order were constitutionalsymptoms (75%), ear nose and throat symptoms (50%), renal (50%), respiratory (45%), ocular (40%) and neurologic (40%). Most common laboratory abnormalities in our patients included leukocytosis (45%), anemia (35%), hematuria (50%), proteinuria (45%), and elevated serum creatinine (45%). Cytoplasmic-anti cytoplasmic antibody (C-ANCA) was positive in 11 (55%), all cases were of granulomatosis with polyangiitis, P-ANCA was positive in 5 (25%) of all patients, with 4 (100%) in Microscopic polyangiitis. Analysis of Granulomatosis with Polyangiitis according to gender and cytoplasmic-anti cytoplasmic antibody status showed correlation of renal involvement with cytoplasmic-anti cytoplasmic antibody status with statistical significance of p=0.036. Plain chest X-rays showed infiltrates in 2 (10%), nodularity 2 (10%), cavitation in 2 (10%), effusion in 1 (5%), and reticulonodular showing in 1 (5%) patients. High-resolution computed tomography findings included ground-glass opacification in 5............

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327. LONG-TERM SAFETY OF RITUXIMAB IN GRANULOMATOSIS WITH POLYANGIITIS OR MICROSCOPIC POLYANGIITIS: RESULTS OF THE FOUR-YEAR RITUXIMAB IN ANCA-ASSOCIATED VASCULITIS REGISTRY

Rheumatology ◽

10.1093/rheumatology/kez063.051 ◽

2019 ◽

Vol 58 (Supplement_2) ◽

Cited By ~ 1

Author(s):

John Niles ◽

Peter Merkel ◽

Lester Mertz ◽

Patricia Lehane ◽

Pooneh Pordeli ◽

...

Keyword(s):

Granulomatosis With Polyangiitis ◽

Microscopic Polyangiitis ◽

Anca Associated Vasculitis

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Efficacy and Safety of Rituximab Treatment in Patients with Antineutrophil Cytoplasmic Antibody-associated Vasculitides: Results from a German Registry (GRAID)

The Journal of Rheumatology ◽

10.3899/jrheum.120482 ◽

2012 ◽

Vol 39 (11) ◽

pp. 2153-2156 ◽

Cited By ~ 23

Author(s):

PETRA ROLL ◽

EVA OSTERMEIER ◽

MARION HAUBITZ ◽

SVJETLANA LOVRIC ◽

LEONORE UNGER ◽

...

Keyword(s):

Treatment Option ◽

Clinical Efficacy ◽

Granulomatosis With Polyangiitis ◽

Microscopic Polyangiitis ◽

Safety Data ◽

Antineutrophil Cytoplasmic Antibody ◽

National Registry ◽

Efficacy And Safety ◽

Anca Associated Vasculitis ◽

German Registry

Objective.Rituximab (RTX) therapy is a treatment option in patients with refractory antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We investigated the tolerability and clinical efficacy of RTX in a cohort of patients with refractory AAV.Methods.Clinical and safety data of patients with AAV treated with RTX were retrospectively assessed from the data of a German national registry.Results.In total, 58 patients were included in this analysis (50/58 with granulomatosis with polyangiitis; 8/58 with microscopic polyangiitis who received at least 1 cycle, 17 patients who received 2 cycles, and 3 patients who received 3 cycles of RTX). Response was classified as complete and partial in 22 (40%) and in 29 cases (52.7%), respectively. Four patients (7.3%) were classified as nonresponders.Conclusion.RTX was well tolerated with good clinical efficacy in patients with refractory AAV.

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Spectrum and Prognosis of Antineutrophil Cytoplasmic Antibody–associated Vasculitis-related Bronchiectasis: Data from 61 Patients

The Journal of Rheumatology ◽

10.3899/jrheum.190313 ◽

2019 ◽

Vol 47 (10) ◽

pp. 1522-1531 ◽

Cited By ~ 1

Author(s):

Raphael Lhote ◽

Marie Chilles ◽

Matthieu Groh ◽

Xavier Puéchal ◽

Philippe Guilpain ◽

...

Keyword(s):

Median Time ◽

Granulomatosis With Polyangiitis ◽

Microscopic Polyangiitis ◽

Fold Increase ◽

Antineutrophil Cytoplasmic Antibody ◽

Mononeuritis Multiplex ◽

Biological Features ◽

Anca Associated Vasculitis ◽

Worse Prognosis

ObjectiveTo report on a large series of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and bronchiectasis, with a specific focus on the timeline of occurrence of both features.MethodsRetrospective nationwide multicenter study of patients diagnosed with both AAV and bronchiectasis.ResultsSixty-one patients were included, among whom 27 (44.25%) had microscopic polyangiitis (MPA), 27 (44.25%) had granulomatosis with polyangiitis (GPA), and 7 (11.5%) had eosinophilic GPA. Thirty-nine (64%) had myeloperoxidase (MPO)-ANCA and 13 (21%) had proteinase 3–ANCA. The diagnosis of bronchiectasis either preceded (n = 25; median time between both diagnoses: 16 yrs, IQR 4–54 yrs), was concomitant to (n = 12), or followed (n = 24; median time between both diagnoses: 1, IQR 0–6 yrs) that of AAV. Patients in whom bronchiectasis precedes the onset of AAV (B-AAV group) have more frequent mononeuritis multiplex, MPA, MPO-ANCA, and a 5-fold increase of death. The occurrence of an AAV relapse tended to be protective against bronchiectasis worsening (HR 0.6, 95% CI 0.4–0.99, P = 0.049), while a diagnosis of bronchiectasis before AAV (HR 5.8, 95% CI 1.2–28.7, P = 0.03) or MPA (HR 18.1, 95% CI 2.2–146.3, P = 0.01) were associated with shorter survival during AAV follow-up.ConclusionThe association of bronchiectasis with AAV is likely not accidental and is mostly associated with MPO-ANCA. Patients in whom bronchiectasis precedes the onset of AAV tend to have distinct clinical and biological features and could carry a worse prognosis.

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Natural Killer Cells in Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - A Review of the Literature

Frontiers in Immunology ◽

10.3389/fimmu.2021.796640 ◽

2022 ◽

Vol 12 ◽

Author(s):

Sina Fuchs ◽

Andrea Scheffschick ◽

Iva Gunnarsson ◽

Hanna Brauner

Keyword(s):

Nk Cells ◽

Natural Killer ◽

Granulomatosis With Polyangiitis ◽

Microscopic Polyangiitis ◽

Lymphoid Cells ◽

Eosinophilic Granulomatosis With Polyangiitis ◽

Systemic Autoimmune Diseases ◽

Anca Associated Vasculitis ◽

And Function ◽

Eosinophilic Granulomatosis

Anti-neutrophil cytoplasmic antibody (ANCA)- associated vasculitis (AAV) is a group of systemic autoimmune diseases characterized by inflammation of small- and medium-sized vessels. The three main types of AAV are granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). A growing number of studies focus on natural killer (NK) cells in AAV. NK cells are innate lymphoid cells with important roles in anti-viral and anti-tumor defense, but their roles in the pathogenesis of autoimmunity is less well established. In this review, we will present a summary of what is known about the number, phenotype and function of NK cells in patients with AAV. We review the literature on NK cells in the circulation of AAV patients, studies on tissue resident NK cells and how the treatment affects NK cells.

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A case of ANCA associated vasculitis presented with mononeuritis multiplex

BIRDEM Medical Journal ◽

10.3329/birdem.v9i3.43094 ◽

2019 ◽

Vol 9 (3) ◽

pp. 257-260

Author(s):

Tanbin Rahman ◽

Md Rashedul Islam ◽

Mohammad Sakhawat Hossen Khan ◽

Sharif Mohammad Ruhul Quddus ◽

Dilruba Alam ◽

...

Keyword(s):

Inflammatory Cell ◽

Granulomatosis With Polyangiitis ◽

Microscopic Polyangiitis ◽

Systemic Vasculitis ◽

Antineutrophil Cytoplasmic Antibody ◽

Mononeuritis Multiplex ◽

Good Clinical Response ◽

Standard Regimen ◽

Anca Associated Vasculitis ◽

Common Manifestation

Mononeuritis multiplex is a common manifestation of many illnesses which includes diabetes, leprosy, malignancy and certain types of systemic vasculitis. The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of rare diseases which show typical characteristic inflammatory cell infiltration and blood vessel wall necrosis. AAV syndromes include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilicgranulomatosis with polyangiitis (EGPA). Here we present a patient who presented with mononeuritis multiplex and had features ofEGPA. The patient was treated with standard regimen of steroids and pulsed cyclophosphamide and she achieved good clinical response. Birdem Med J 2019; 9(3): 257-260

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Clinical outcomes of treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis based on ANCA type

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2015-208073 ◽

2015 ◽

Vol 75 (6) ◽

pp. 1166-1169 ◽

Cited By ~ 100

Author(s):

Sebastian Unizony ◽

Miguel Villarreal ◽

Eli M Miloslavsky ◽

Na Lu ◽

Peter A Merkel ◽

...

Keyword(s):

Disease Activity Score ◽

Granulomatosis With Polyangiitis ◽

Microscopic Polyangiitis ◽

Proteinase 3 ◽

Successful Completion ◽

Anca Associated Vasculitis ◽

Registration Number ◽

Treatment Responses ◽

New Onset

ObjectiveTo evaluate whether the classification of patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) according to ANCA type (anti-proteinase 3 (PR3) or anti-myeloperoxidase (MPO) antibodies) predicts treatment response.MethodsTreatment responses were assessed among patients enrolled in the Rituximab in ANCA-associated Vasculitis trial according to both AAV diagnosis (granulomatosis with polyangiitis (GPA)/microscopic polyangiitis (MPA)) and ANCA type (PR3-AAV/MPO-AAV). Complete remission (CR) was defined as disease activity score of 0 and successful completion of the prednisone taper.ResultsPR3-AAV patients treated with rituximab (RTX) achieved CR at 6 months more frequently than did those randomised to cyclophosphamide (CYC)/azathioprine (AZA) (65% vs 48%; p=0.04). The OR for CR at 6 months among PR3-AAV patients treated with RTX as opposed to CYC/AZA was 2.11 (95% CI 1.04 to 4.30) in analyses adjusted for age, sex and new-onset versus relapsing disease at baseline. PR3-AAV patients with relapsing disease achieved CR more often following RTX treatment at 6 months (OR 3.57; 95% CI 1.43 to 8.93), 12 months (OR 4.32; 95% CI 1.53 to 12.15) and 18 months (OR 3.06; 95% CI 1.05 to 8.97). No association between treatment and CR was observed in the MPO-AAV patient subset or in groups divided according to AAV diagnosis.ConclusionsPatients with PR3-AAV respond better to RTX than to CYC/AZA. An ANCA type-based classification may guide immunosuppression in AAV.Trial registration numberNCT00104299; post-results.

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Venous Thrombotic Events in ANCA-Associated Vasculitis: Incidence and Risk Factors

Kidney360 ◽

10.34067/kid.0000572019 ◽

2020 ◽

Vol 1 (4) ◽

pp. 258-262 ◽

Cited By ~ 2

Author(s):

Bradley Isaacs ◽

Eric J. Gapud ◽

Brendan Antiochos ◽

Philip Seo ◽

Duvuru Geetha

Keyword(s):

Risk Factors ◽

Granulomatosis With Polyangiitis ◽

Multivariate Analyses ◽

Microscopic Polyangiitis ◽

Common Complication ◽

Birmingham Vasculitis Activity Score ◽

Factors Associated ◽

Anca Associated Vasculitis ◽

The Mean ◽

Univariate Analyses

BackgroundThe incidence of venous thromboembolism (VTE) is increased in ANCA-associated vasculitis (AAV). We assessed the frequency of VTE observed among patients with AAV evaluated at our center and identified risk factors.MethodsPatients from the Johns Hopkins Vasculitis Center cohort who were evaluated between 1998 and 2018 and had a diagnosis of granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) were eligible for analysis. Baseline demographics and clinical and serologic data were extracted. Univariate and multivariate analyses were performed to identify factors associated with VTE in AAV.ResultsA total of 162 patients with AAV were identified, 105 (65%) with GPA; 22 (14%) of these patients had a recorded VTE with a median time to VTE of 1 month. The mean (SD) age in the VTE versus non-VTE groups was 54±20 versus 55±17 years (P=0.99), 64% versus 60% female (P=0.93), 82% versus 49% PR3-ANCA positive (P=0.01), with a total mean BMI of 33.3±5.7 versus 28.3±6.1 kg/m2, (P<0.001) respectively. The median Birmingham Vasculitis Activity Score (BVAS version 3) was 19 versus 14 (P=0.02). Univariate analyses identified PR3-ANCA, rapidly progressive GN (RPGN), and hypoalbuminemia. In multivariate analysis, the significant associations with VTE included PR3-ANCA (OR, 4.77; P=0.02), hypoalbuminemia (OR, 4.84; P=0.004), and BMI (OR, 1.18; P<0.001).ConclusionsVTE is a surprisingly common complication of AAV. PR3-ANCA and hypoalbuminemia are risk factors for developing VTEs. Further studies are needed to confirm these findings.PodcastThis article contains a podcast at https://www.asn-online.org/media/podcast/K360/2020_04_30_KID0000572019.mp3

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Antineutrophil Cytoplasmic Antibody–Positive Vasculitis

Asthma ◽

10.1093/med/9780199918065.003.0002 ◽

2014 ◽

pp. 12-20

Author(s):

Lanny J. Rosenwasser ◽

Dennis K. Ledford

Keyword(s):

Granulomatosis With Polyangiitis ◽

Microscopic Polyangiitis ◽

Upper Airway ◽

Airway Disease ◽

Antineutrophil Cytoplasmic Antibody ◽

Eosinophilic Granulomatosis With Polyangiitis ◽

Anca Associated Vasculitis ◽

Granulomatous Polyangiitis ◽

Strauss Syndrome ◽

Eosinophilic Granulomatosis

The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis syndromes— eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome or allergic angiitis with granulomatosis), granulomatous polyangiitis (Wegener’s granulomatosis), and microscopic polyangiitis—are serious comorbidities of asthma or upper respiratory disease, or the symptoms of the vasculitis may resemble asthma or associated upper airway disease. ANCA-associated vasculitis is potentially fatal but is responsive to a variety of treatments, if the vasculitis is recognized before serious organ dysfunction. The role of ANCA in these conditions has not been defined because eosinophilic granulomatosis with polyangiitis, granulomatous polyangiitis, or microscopic polyangiitis may occur without ANCA, and the titer of ANCA does not predict clinical course. Clinical suspicion and consideration of tissue biopsy are important for recognition before irreversible complications occur. Corticosteroid therapy for asthma or suspected asthma may modify the presentations of ANCA-positive vasculitis.

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