Microscopic polyangiitis pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Ali Poyan Mehr, M.D. [2]Associate Editor(s)-in-Chief: Krzysztof Wierzbicki M.D. [3]
Overview
The pathogenesis of Microscopic polyangiitis is currently not fully understood. However, certain hypothesizes have been made to determine possible factors that may trigger the disease such as enviornmental factors and anti-neutrophilc cytoplasmic antibodies.
Pathogenesis
The etiology of Microscopic polyangiitis is not fully understood. However, certain hypothesizes have been made to determine possible factors that may trigger the disease. Triggers such as environmental factors and anti-neutrophil cytoplasmic antibodies.
Environmental factors
Environmental triggers such as exposure to silica have been found to influence the progression of the disease. However, its role in disease progression is not fully understood.[1]
Anti-neutrophil cytoplasmic antibodies (ANCA)
The majority of patients affected with Microscopic polyangiitis are positive for anti-neutrophil cytoplasmic antibodies with myeloperoxidase antigen activity. The presence of anti-neutrophil cytoplasmic antibodies activates neutrophil production do to proinflammatory cytokines (interleukin-1 and tumor necrosis factor-α), producing reactive oxygen species and causing the release of lytic enzymes. These two processes, induce detachment and lyses of the endothelium. The destruction of the endothelial cells results in necrotizing crescentic glomerulonephritis and necrotizing vasculitis of the pulmonary capillaries.
Genetics
Microscopic polyangiitis is correlated with HLA-DRB1*09:01-DQB1*03:03 haplotype in the Japanese population. This haplotype however, in the Caucasian population is not typically seen.[2]
Associated Conditions
Gross Pathology
On gross pathology, the following changes are typically seen:
Microscopic Pathology
References
- ↑ de Lind van Wijngaarden RA, van Rijn L, Hagen EC, Watts RA, Gregorini G, Tervaert JW; et al. (2008). "Hypotheses on the etiology of antineutrophil cytoplasmic autoantibody associated vasculitis: the cause is hidden, but the result is known". Clin J Am Soc Nephrol. 3 (1): 237–52. doi:10.2215/CJN.03550807. PMID 18077783.
- ↑ Tsuchiya N (2012). "Genetics of microscopic polyangiitis in the Japanese population". Ann Vasc Dis. 5 (3): 289–95. doi:10.3400/avd.ra.12.00062. PMC 3595849. PMID 23555527.