scholarly journals 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 ◽  
2019 ◽  
Vol 58 (Supplement_2) ◽  
Author(s):  
John Niles ◽  
Peter Merkel ◽  
Lester Mertz ◽  
Patricia Lehane ◽  
Pooneh Pordeli ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Anca Associated Vasculitis

scholarly journals Granulomatosis with Polyangiitis: Recurrence or Treatment Consequences?

2021 ◽  
Author(s):  
Andreia Diegues ◽  
Joana Tavares ◽  
Diogo Sá ◽  
João Oliveira ◽  
Diana Fernandes ◽  
...  
Keyword(s):  
Intracranial Hypertension ◽  
Clinical Improvement ◽  
Granulomatosis With Polyangiitis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Lung Nodules ◽  
Rare Form ◽  
Anca Associated Vasculitis

Granulomatosis with polyangiitis (GPA) is the most common antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We describe the case of a 38-year-old woman with relapsing GPA who presented with intracranial hypertension, followed by the appearance of cavitated lung nodules despite treatment with azathioprine. Clinical improvement and ANCA titre reduction were observed after rituximab treatment. We report a rare form of GPA relapse and highlight the challenge of following-up patients with GPA, in whom can be hard to distinguish relapse from the consequences of long-term immunosuppression.

scholarly journals Long‐Term Safety of Rituximab in Granulomatosis with Polyangiitis or Microscopic Polyangiitis

2020 ◽  
Author(s):  
Peter A. Merkel ◽  
John L. Niles ◽  
Lester E. Mertz ◽  
Patricia B. Lehane ◽  
Pooneh Pordeli ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis

scholarly journals Immunopathogenesis of ANCA-Associated Vasculitis

2020 ◽  
Vol 21 (19) ◽  
pp. 7319
Author(s):  
Andreas Kronbichler ◽  
Keum Hwa Lee ◽  
Sara Denicolo ◽  
Daeun Choi ◽  
Hyojeong Lee ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Autoimmune Disorder ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Proteinase 3 ◽  
Disease Biomarkers ◽  
Disease Phenotypes ◽  
Anca Associated Vasculitis ◽  
Clinical Presentations ◽  
Eosinophilic Granulomatosis

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.

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

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............

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

2012 ◽  
Vol 39 (11) ◽  
pp. 2153-2156 ◽  
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.

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

2019 ◽  
Vol 47 (10) ◽  
pp. 1522-1531 ◽  
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.

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

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.

scholarly journals A case of ANCA associated vasculitis presented with mononeuritis multiplex

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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