ANCA associated vasculitis (AAV): a review for internists
Kinanah Yaseen1, Brian F Mandell1
1Department of Rheumatic and Immunologic Diseases, Cleveland Clinic, Cleveland, Ohio, USA.
Postgraduate Medicine
|July 14, 2022
Summary
Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) involves rare autoimmune disorders affecting blood vessels. Advances in understanding AAV have led to improved treatments and survival rates for patients with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA).
Area of Science:
- Rheumatology and Immunology
- Vascular Medicine
Background:
- Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) encompasses rare necrotizing small to medium vessel vasculitides, including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA).
- AAV is characterized by autoantibodies against neutrophil proteins, such as proteinase 3 (PR3-ANCA) and myeloperoxidase (MPO-ANCA), which trigger inflammation and damage to vessel walls.
- Clinical manifestations range from non-severe to life-threatening multi-organ involvement, emphasizing the critical need for early diagnosis and intervention.
Purpose of the Study:
- To provide a narrative review focusing on granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), two major forms of ANCA-associated vasculitis.
- To highlight key aspects of AAV, including clinical manifestations, diagnostic approaches, disease monitoring strategies, and current treatment paradigms.
Main Methods:
- This review synthesizes current knowledge on AAV, focusing on GPA and MPA.
- It examines recent advances in understanding the pathophysiology, diagnosis, and management of these conditions.
Main Results:
- Significant progress in understanding AAV pathophysiology has driven the development of novel treatments over the past two decades.
- These therapeutic advancements have led to substantial improvements in patient outcomes and survival rates for AAV.
- The review details the clinical spectrum, diagnostic criteria, and monitoring tools for GPA and MPA.
Conclusions:
- Early recognition and diagnosis of AAV are crucial for effective management and improved patient prognosis.
- Advances in treatment strategies have markedly enhanced the outlook for individuals diagnosed with GPA and MPA.
- Continued research into AAV pathogenesis and therapeutic interventions remains vital for further improving patient care.
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