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Microscopic polyangiitis (microscopic polyarteritis)
J C Jennette1, D B Thomas, R J Falk
1Department of Pathology and Laboratory Medicine, University of North Carolina, Chapel Hill 27599-7525, USA.
Seminars in Diagnostic Pathology
|April 12, 2001
Summary
Microscopic polyangiitis is a necrotizing small vessel vasculitis. It is distinguished from other vasculitides by pauci-immune glomerulonephritis and pulmonary capillaritis, often without immune deposits.
Area of Science:
- Rheumatology
- Nephrology
- Pulmonology
Background:
- Microscopic polyangiitis (MPA) is a necrotizing small vessel vasculitis.
- MPA primarily affects venules, capillaries, arterioles, and small arteries.
- It is differentiated from immune complex vasculitis by the absence of significant immunoglobulin deposition.
Purpose of the Study:
- To clarify the distinct pathological and clinical features of microscopic polyangiitis.
- To differentiate MPA from other small and medium-sized vessel vasculitides.
- To highlight MPA's common renal and pulmonary manifestations.
Main Methods:
- Pathological examination of vessel walls, focusing on inflammation and immune deposits.
- Clinical assessment of patient symptoms, including renal and pulmonary involvement.
- Serological testing for antineutrophil cytoplasmic autoantibodies (ANCAs).
Main Results:
- MPA is characterized by pauci-immune necrotizing and crescentic glomerulonephritis.
- Hemorrhagic pulmonary capillaritis is a frequent finding in MPA.
- MPA shares pathological features with Wegener's granulomatosis and Churg-Strauss syndrome but lacks granulomatous inflammation or eosinophilia, respectively.
- Circulating ANCAs are associated with MPA, Wegener's granulomatosis, and Churg-Strauss syndrome.
Conclusions:
- Microscopic polyangiitis is a distinct vasculitic entity characterized by small vessel necrotizing inflammation.
- Pauci-immune glomerulonephritis and pulmonary capillaritis are hallmarks of MPA.
- Distinguishing MPA from other ANCA-associated vasculitides relies on the absence of granulomatous inflammation and eosinophilia.