Microscopic polyangiitis pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Vamsikrishna Gunnam M.B.B.S [2], 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 environmental factors and anti-neutrophil cytoplasmic antibodies. Capillaries and venules are involved in the pathogenesis of microscopic polyangiitis.The paucity of immunoglobulin deposition is shown in immunohistochemical staining.
Pathogenesis
Pathogenesis
- The exact etiology of Microscopic polyangiitis is not fully understood.
- 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 triggers
- Environmental triggers such as exposure to silica have been found to influence the progression of the disease.But, its role in disease progression is not fully understood.[1]
Anti-neutrophil cytoplasmic antibodies (ANCA)
Key-points regarding ANCA in microscopic polyangiitis include:[2][3][4][5]
- The majority of patients who are affected with microscopic polyangiitis are positive for anti-neutrophil cytoplasmic antibodies (ANCA) with myeloperoxidase antigen activity.
- The presence of anti-neutrophil cytoplasmic antibodies (ANCA) activates neutrophil production do to proinflammatory cytokines such as interleukin-1 and tumor necrosis factor-α (TNF-α).
- Stimulation of interleukin-1 and tumor necrosis factor-α (TNF-α) results in 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.[6]
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.[7]
Gross Pathology
- On gross pathology, the characteristic findings of microscopic polyangiitis incude:[8][9]
- Hemorrhagic necrotizing alveolar capillaritis
- Fibrinoid necrosis of the lung
- Intra-alveolar hemosiderosis
Microscopic Pathology
- On microscopic histopathological analysis of the kidney in patients with microscopic polyangiitis, characteristic findings include:[10][11]
- Focal segmental necrotizing glomerulonephritis
- Crescents of glomeruli
- Minimal deposition of immunoglobulins
- Compliment in glomeruli and renal vasculature can be seen
- The majority of patients who are affected with Microscopic polyangiitis are positive for anti-neutrophil cytoplasmic antibodies (ANCA) with myeloperoxidase antigen activity.
- The presence of anti-neutrophil cytoplasmic antibodies (ANCA) activates neutrophil production do to proinflammatory cytokines such as interleukin-1 and tumor necrosis factor-α (TNF-α).
- Stimulation of interleukin-1 and tumor necrosis factor-α (TNF-α) results in 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.[12]
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.
- ↑ Eisenberger U, Fakhouri F, Vanhille P, Beaufils H, Mahr A, Guillevin L, Lesavre P, Noël LH (July 2005). "ANCA-negative pauci-immune renal vasculitis: histology and outcome". Nephrol. Dial. Transplant. 20 (7): 1392–9. doi:10.1093/ndt/gfh830. PMID 15855209.
- ↑ Falk RJ, Nachman PH, Hogan SL, Jennette JC (May 2000). "ANCA glomerulonephritis and vasculitis: a Chapel Hill perspective". Semin. Nephrol. 20 (3): 233–43. PMID 10855933.
- ↑ Vizjak A, Rott T, Koselj-Kajtna M, Rozman B, Kaplan-Pavlovcic S, Ferluga D (March 2003). "Histologic and immunohistologic study and clinical presentation of ANCA-associated glomerulonephritis with correlation to ANCA antigen specificity". Am. J. Kidney Dis. 41 (3): 539–49. doi:10.1053/ajkd.2003.50142. PMID 12612976.
- ↑ Neumann I, Regele H, Kain R, Birck R, Meisl FT (March 2003). "Glomerular immune deposits are associated with increased proteinuria in patients with ANCA-associated crescentic nephritis". Nephrol. Dial. Transplant. 18 (3): 524–31. PMID 12584274.
- ↑ Kallenberg CG, Heeringa P, Stegeman CA (2006). "Mechanisms of Disease: pathogenesis and treatment of ANCA-associated vasculitides". Nat Clin Pract Rheumatol. 2 (12): 661–70. doi:10.1038/ncprheum0355. PMID 17133251.
- ↑ 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.
- ↑ Gómez-Puerta JA, Espinosa G, Morlà R, Cid MC, Cervera R (2009). "Interstitial lung disease as a presenting manifestation of microscopic polyangiitis successfully treated with mycophenolate mofetil". Clin Exp Rheumatol. 27 (1): 166–7. PMID 19327249.
- ↑ Chung SA, Seo P (2010). "Microscopic polyangiitis". Rheum Dis Clin North Am. 36 (3): 545–58. doi:10.1016/j.rdc.2010.04.003. PMC 2917831. PMID 20688249.
- ↑ Savage CO, Winearls CG, Evans DJ, Rees AJ, Lockwood CM (1985). "Microscopic polyarteritis: presentation, pathology and prognosis". Q J Med. 56 (220): 467–83. PMID 4048389.
- ↑ Chung SA, Seo P (2010). "Microscopic polyangiitis". Rheum Dis Clin North Am. 36 (3): 545–58. doi:10.1016/j.rdc.2010.04.003. PMC 2917831. PMID 20688249.
- ↑ Kallenberg CG, Heeringa P, Stegeman CA (2006). "Mechanisms of Disease: pathogenesis and treatment of ANCA-associated vasculitides". Nat Clin Pract Rheumatol. 2 (12): 661–70. doi:10.1038/ncprheum0355. PMID 17133251.