scholarly journals Prevalence of large vessel vasculitis in ANCA-associated vasculitis: a retrospective cohort study

2021 ◽  
Author(s):  
Yann Coattrenec ◽  
Yannick D. Muller ◽  
David Spoerl ◽  
Johannes A. Lobrinus ◽  
Jörg D. Seebach
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Temporal Arteritis ◽  
Significant Proportion ◽  
Large Vessel Vasculitis ◽  
Large Vessel ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Anca Associated Vasculitis ◽  
Total Prevalence ◽  
Swiss Population ◽  
Eosinophilic Granulomatosis

AbstractANCA-associated vasculitis (AAV) in general involves small blood vessels and includes granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA). Although reported in a few studies, the prevalence of large vessel vasculitis (LVV) in patients with AAV remains to be further explored. The goal of the present study was to assess the prevalence of LVV in a cohort of patients with AAV and to characterize this population. We conducted a ten-year retrospective study of a single-center cohort of AAV, including 101 patients with GPA (n = 58), EGPA (n = 28), MPA (n = 15), and compared the groups with or without associated LVV. LVV was diagnosed in five patients, two with aortitis and three with temporal arteritis, corresponding to a total prevalence of 5.0% [95% CI 1.6–11.2%]. This value was significantly higher than the estimated prevalence of LVV in the normal Swiss population (OR 234.9 95% CI 91.18–605.2, p < 0.001). All five patients had GPA, whereas no cases with EGPA or MPA were identified. Anti-PR3 antibodies were detected in four out of five patients, anti-MPO in one patient. Since LVV can occur in a significant proportion of patients with GPA, evaluation for LVV may be considered systematically in the diagnostic workup of AAV.

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 EOSINOPHILIC STATE, ASTHMA, RADICULITIS, MYOSITIS, AND LARGE VESSEL OBSTRUCTION: ATYPICAL EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS?

2021 ◽  
Author(s):  
Jefferson Becker ◽  
Henrique Luiz Staub ◽  
Rafael Saidelles ◽  
Lucas Immich Gonçalves ◽  
Luís Eduardo Stefani Brusamarello ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Large Vessel ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Eosinophilic Granulomatosis

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 rare variant of ANCA-associated eosinophilic granulomatosis with polyangiitis complicated by diffuse alveolar hemorrage

2019 ◽  
Vol 29 (2) ◽  
pp. 229-234
Author(s):  
Anna S. Zaytseva ◽  
Igor' E. Stepanyan ◽  
Irina Yu. Shabalina ◽  
Lyudmila I. Dmitriyeva ◽  
Evgeniy I. Shmelev
Keyword(s):  
Clinical Case ◽  
Granulomatosis With Polyangiitis ◽  
Cardiac Injury ◽  
Diffuse Alveolar Hemorrhage ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Elderly Female ◽  
Anca Associated Vasculitis ◽  
Eosinophilic Granulomatosis ◽  
Limited Usefulness

Diagnostic criteria of vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA) are of limited usefulness as they involve resembling conditions and do not make possible distinguishing nosology of vasculitis. A challenging clinical case of an elderly female patient with ANCA-associated vasculitis, diffuse alveolar hemorrhage syndrome, cardiac injury and blood eosinophilia, but without bronchial asthma, is described in the article.

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.

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