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Published on: August 26, 2017
Microscopic polyangiitis
1Division of Rheumatology, Department of Medicine, Rosalind Russell Medical Research Center for Arthritis, University of California, San Francisco, CA, USA.
Abstract:
In 1923, Friedrich Wohlwill described two patients with a "microscopic form of periarteritis nodosa," which was distinct from the classical form. This disease, now known as microscopic polyangiitis (MPA), is a primary systemic vasculitis characterized by inflammation of the small-caliber blood vessels and the presence of circulating antineutrophil cytoplasmic antibodies. Typically, microscopic polyangiitis presents with glomerulonephritis and pulmonary capillaritis, although involvement of the skin, nerves, and gastrointestinal tract is not uncommon. Treatment of MPA generally requires use of a cytotoxic agent (such as cyclophosphamide) in addition to high-dose glucocorticoids. Recent research has focused on identifying alternate treatment strategies that minimize or eliminate exposure to cytotoxic agents. This article reviews the history, pathogenesis, clinical manifestations, and treatment of MPA.
Insights
Microscopic polyangiitis (MPA) is a small-vessel vasculitis often treated with cytotoxic agents and glucocorticoids. Current research seeks alternative therapies to reduce reliance on these potent drugs.
Area of Science:
- Rheumatology
- Immunology
- Pathology
Background:
- Microscopic polyangiitis (MPA) is a systemic vasculitis distinct from classical periarteritis nodosa.
- It involves small-caliber blood vessels and is associated with antineutrophil cytoplasmic antibodies.
- MPA commonly affects the kidneys (glomerulonephritis) and lungs (pulmonary capillaritis).
Purpose of the Study:
- To review the history, pathogenesis, clinical manifestations, and treatment of microscopic polyangiitis.
- To highlight current research into alternative treatment strategies for MPA.
Main Methods:
- Literature review of historical descriptions and recent research.
- Analysis of pathogenesis, clinical presentation, and treatment modalities.
Main Results:
- MPA is characterized by small-vessel inflammation and specific autoantibodies.
- Standard treatment involves cytotoxic agents and high-dose glucocorticoids.
- Emerging research focuses on minimizing cytotoxic agent exposure.
Conclusions:
- Understanding MPA's history and pathogenesis is crucial for effective management.
- Current treatment paradigms are being re-evaluated to improve patient outcomes.
- Future research aims to develop safer and equally effective MPA therapies.
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