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 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

Antineutrophil Cytoplasmic Antibody–Positive Vasculitis

Asthma ◽  
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.

scholarly journals ANCA Status or Clinical Phenotype — What Counts More?

2021 ◽  
Vol 23 (6) ◽  
Author(s):  
Martin Windpessl ◽  
Erica L. Bettac ◽  
Philipp Gauckler ◽  
Jae Il Shin ◽  
Duvuru Geetha ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Granulomatosis With Polyangiitis ◽  
Clinical Phenotype ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Cardiovascular Death ◽  
Genetic Associations ◽  
Ongoing Debate ◽  
The Past ◽  
Anca Associated Vasculitis

Abstract Purpose of Review There is ongoing debate concerning the classification of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. That is, whether classification should be based on the serotype (proteinase 3 (PR3)- or myeloperoxidase (MPO)-ANCA) or on the clinical phenotype (granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA)). To add clarity, this review focused on integration of the most recent literature. Recent Findings Large clinical trials have provided evidence that a serology-based risk assessment for relapses is more predictive than distinction based on the phenotype. Research conducted in the past decade indicated that a serology-based approach more closely resembles the genetic associations, the clinical presentation (i.e., lung involvement), biomarker biology, treatment response, and is also predicting comorbidities (such as cardiovascular death). Summary Our review highlights that a serology-based approach could replace a phenotype-based approach to classify ANCA-associated vasculitides. In future, clinical trials and observational studies will presumably focus on this distinction and, as such, translate into a “personalized medicine.”

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 Granulomatosis with Polyangiitis Restricted to the Back Muscle: The First Case Report

Medicina ◽  
2020 ◽  
Vol 56 (9) ◽  
pp. 431
Author(s):  
Yu-Mi Lee ◽  
Sang Wan Chung
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Final Diagnosis ◽  
High Dose ◽  
Back Muscle ◽  
Anca Associated Vasculitis ◽  
First Case ◽  
Psoas Muscles ◽  
High Dose Methylprednisolone ◽  
Single Organ

Granulomatosis with polyangiitis (GPA) is an autoimmune disease which is a type of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis that frequently affects the lungs and kidneys. However, GPA limited to a single organ has also been reported. A 71-year-old man was admitted for back pain and fever. We detected elevated levels of inflammatory markers and myeloperoxidase-ANCA. Magnetic resonance imaging indicated diffuse inflammation of the back and psoas muscles. Histology showed degenerated muscle fibers and granulomatosis vasculitis with mixed lymphoplasma cell infiltration. High-dose methylprednisolone therapy improved his symptoms. A final diagnosis of GPA limited to the muscles was made.

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.

Incidence of Malignancy Prior to Antineutrophil Cytoplasmic Antibody–associated Vasculitis Compared to the General Population

2017 ◽  
Vol 44 (3) ◽  
pp. 314-318 ◽  
Author(s):  
Emma E. van Daalen ◽  
Chinar Rahmattulla ◽  
Ron Wolterbeek ◽  
Jan A. Bruijn ◽  
Ingeborg M. Bajema
Keyword(s):  
General Population ◽  
Causal Relationship ◽  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Malignancy Risk ◽  
Time Period ◽  
Study Results ◽  
Significant Difference ◽  
Overall Risk

Objective.Previous studies have reported an increased malignancy risk preceding antineutrophil cytoplasmic antibody–associated vasculitis (AAV), suggesting common pathogenic pathways in these 2 entities. However, the study results were conflicting and often limited to patients with granulomatosis with polyangiitis (GPA). Here, we study the malignancy risk prior to AAV diagnosis [either GPA or microscopic polyangiitis (MPA)] to elaborate on the putative association between malignancy and AAV.Methods.A total of 203 patients were selected for the current study. Malignancies prior to AAV diagnosis were identified using a nationwide pathology database, and their occurrence was verified by reviewing the medical files of 145 patients (71.4%). The malignancy incidence was compared to the general population by calculation of standardized incidence ratios (SIR), matching for sex, age, and time period. SIR were calculated for 2 intervals: < 2 years and ≥ 2 years prior to AAV diagnosis. Separate analyses were performed for GPA and MPA.Results.The overall risk for malignancy prior to AAV diagnosis was similar to that of the general population (SIR 0.96, 95% CI 0.55–1.57), as was true when risks were analyzed by malignancy type, including skin, bladder, kidney, lung, stomach, rectum, and uterus (SIR ranged from 1.64 to 4.14). We found no significant difference in malignancy risk between patients with GPA and MPA.Conclusion.Our findings do not support the hypothesis that preceding malignancies and AAV have a causal relationship or shared pathogenic pathways.

Sign in / Sign up

Export Citation Format

Share Document