Microscopic polyangiitis pathophysiology: Difference between revisions
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====Anti-neutrophil cytoplasmic antibodies (ANCA)==== | ====Anti-neutrophil cytoplasmic antibodies (ANCA)==== | ||
==Overview== | |||
The [[pathogenesis]] of [[Microscopic polyangiitis]] is currently not fully understood. However, certain [[Hypothesis|hypothesizes]] have been made to determine possible factors that may trigger the disease such as environmental factors and [[Anti-neutrophil cytoplasmic antibody|anti-neutrophil cytoplasmic antibodies.]] [[Capillary|Capillaries]] and [[venules]] are involved in the [[pathogenesis]] of [[microscopic polyangiitis]].The paucity of [[immunoglobulin]] deposition is shown in [[Immunohistochemical staining|Immunohistochemical]] staining. | |||
==Pathogenesis== | |||
====Pathogenesis==== | |||
*The exact [[etiology]] of [[Microscopic polyangiitis]] is not fully understood. | |||
*Certain [[Hypothesis|hypothesizes]] have been made to determine possible factors that may trigger the disease. | |||
*Triggers such as [[Environmental factor|environmental factors]] and [[Anti-neutrophil cytoplasmic antibody|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.<ref name="pmid180777832">{{cite journal| author=de Lind van Wijngaarden RA, van Rijn L, Hagen EC, Watts RA, Gregorini G, Tervaert JW et al.| title=Hypotheses on the etiology of antineutrophil cytoplasmic autoantibody associated vasculitis: the cause is hidden, but the result is known. | journal=Clin J Am Soc Nephrol | year= 2008 | volume= 3 | issue= 1 | pages= 237-52 | pmid=18077783 | doi=10.2215/CJN.03550807 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18077783 }}</ref> | |||
====Anti-neutrophil cytoplasmic antibodies (ANCA)<ref name="pmid15855209">{{cite journal |vauthors=Eisenberger U, Fakhouri F, Vanhille P, Beaufils H, Mahr A, Guillevin L, Lesavre P, Noël LH |title=ANCA-negative pauci-immune renal vasculitis: histology and outcome |journal=Nephrol. Dial. Transplant. |volume=20 |issue=7 |pages=1392–9 |date=July 2005 |pmid=15855209 |doi=10.1093/ndt/gfh830 |url=}}</ref>==== | |||
*The majority of patients who are affected with [[Microscopic polyangiitis]] are positive for [[Anti-neutrophil cytoplasmic antibody|anti-neutrophil cytoplasmic antibodies]] ([[ANCA]]) with [[myeloperoxidase]] antigen activity. | |||
*The presence of [[Anti-neutrophil cytoplasmic antibody|anti-neutrophil cytoplasmic antibodies]] ([[ANCA]]) activates neutrophil production do to [[proinflammatory]] [[Cytokine|cytokines]] such as ''[[Interleukin 1|interleukin-1]]'' and ''[[Tumor necrosis factor-alpha|tumor necrosis factor-α]](TNF-ALPHA).'' | |||
*Stimulation of ''[[Interleukin 1|interleukin-1]]'' and ''tumor necrosis factor-α(TNF-ALPHA)'' 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|endothelial]] cells results in [[necrotizing]] [[Crescentic glomerulonephritis|crescentic]] glomerulonephritis and [[Necrosis|necrotizing]] [[vasculitis]] of the pulmonary [[capillaries]].<ref name="pmid171332512">{{cite journal| author=Kallenberg CG, Heeringa P, Stegeman CA| title=Mechanisms of Disease: pathogenesis and treatment of ANCA-associated vasculitides. | journal=Nat Clin Pract Rheumatol | year= 2006 | volume= 2 | issue= 12 | pages= 661-70 | pmid=17133251 | doi=10.1038/ncprheum0355 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17133251 }}</ref> | |||
==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.<ref name="pmid235555272">{{cite journal| author=Tsuchiya N| title=Genetics of microscopic polyangiitis in the Japanese population. | journal=Ann Vasc Dis | year= 2012 | volume= 5 | issue= 3 | pages= 289-95 | pmid=23555527 | doi=10.3400/avd.ra.12.00062 | pmc=3595849 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23555527 }}</ref> | |||
==Gross Pathology== | |||
*On gross pathology, the following changes are typically seen:<ref name="pmid193272492">{{cite journal| author=Gómez-Puerta JA, Espinosa G, Morlà R, Cid MC, Cervera R| title=Interstitial lung disease as a presenting manifestation of microscopic polyangiitis successfully treated with mycophenolate mofetil. | journal=Clin Exp Rheumatol | year= 2009 | volume= 27 | issue= 1 | pages= 166-7 | pmid=19327249 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19327249 }}</ref><ref name="pmid206882493">{{cite journal| author=Chung SA, Seo P| title=Microscopic polyangiitis. | journal=Rheum Dis Clin North Am | year= 2010 | volume= 36 | issue= 3 | pages= 545-58 | pmid=20688249 | doi=10.1016/j.rdc.2010.04.003 | pmc=2917831 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20688249 }}</ref> | |||
**[[Hemorrhagic]] [[necrotizing]] [[alveolar]] capillaritis. | |||
**[[Fibrinoid necrosis|Fibrinoid]] necrosis of the lung. | |||
**Intra-alveolar [[hemosiderosis]]. | |||
==Microscopic Pathology== | |||
*On [[microscopic]] histopathological analysis of the [[Renal|kidney]]<ref name="pmid40483892">{{cite journal| author=Savage CO, Winearls CG, Evans DJ, Rees AJ, Lockwood CM| title=Microscopic polyarteritis: presentation, pathology and prognosis. | journal=Q J Med | year= 1985 | volume= 56 | issue= 220 | pages= 467-83 | pmid=4048389 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4048389 }}</ref><ref name="pmid2068824922">{{cite journal| author=Chung SA, Seo P| title=Microscopic polyangiitis. | journal=Rheum Dis Clin North Am | year= 2010 | volume= 36 | issue= 3 | pages= 545-58 | pmid=20688249 | doi=10.1016/j.rdc.2010.04.003 | pmc=2917831 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20688249 }}</ref> | |||
**Focal [[Segmental analysis (biology)|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 antibody|anti-neutrophil cytoplasmic antibodies]] ([[ANCA]]) with [[myeloperoxidase]] antigen activity. | * The majority of patients who are affected with [[Microscopic polyangiitis]] are positive for [[Anti-neutrophil cytoplasmic antibody|anti-neutrophil cytoplasmic antibodies]] ([[ANCA]]) with [[myeloperoxidase]] antigen activity. | ||
* The presence of [[Anti-neutrophil cytoplasmic antibody|anti-neutrophil cytoplasmic antibodies]] ([[ANCA]]) activates neutrophil production do to [[proinflammatory]] [[Cytokine|cytokines]] such as ''[[Interleukin 1|interleukin-1]]'' and ''[[Tumor necrosis factor-alpha|tumor necrosis factor-α]](TNF-ALPHA).'' | * The presence of [[Anti-neutrophil cytoplasmic antibody|anti-neutrophil cytoplasmic antibodies]] ([[ANCA]]) activates neutrophil production do to [[proinflammatory]] [[Cytokine|cytokines]] such as ''[[Interleukin 1|interleukin-1]]'' and ''[[Tumor necrosis factor-alpha|tumor necrosis factor-α]](TNF-ALPHA).'' | ||
Revision as of 19:38, 18 April 2018
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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]Vamsikrishna Gunnam M.B.B.S [4]
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)
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.[2]
Anti-neutrophil cytoplasmic antibodies (ANCA)[3]
- 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-ALPHA).
- Stimulation of interleukin-1 and tumor necrosis factor-α(TNF-ALPHA) 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.[4]
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.[5]
Gross Pathology
- On gross pathology, the following changes are typically seen:[6][7]
- Hemorrhagic necrotizing alveolar capillaritis.
- Fibrinoid necrosis of the lung.
- Intra-alveolar hemosiderosis.
Microscopic Pathology
- On microscopic histopathological analysis of the kidney[8][9]
- 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-ALPHA).
- Stimulation of interleukin-1 and tumor necrosis factor-α(TNF-ALPHA) 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.[10]
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.[11]
Gross Pathology
- On gross pathology, the following changes are typically seen:[12][13]
- Hemorrhagic necrotizing alveolar capillaritis.
- Fibrinoid necrosis of the lung.
- Intra-alveolar hemosiderosis.
Microscopic Pathology
- On microscopic histopathological analysis of the kidney[14][15]
- Focal segmental necrotizing glomerulonephritis.
- Crescents of glomeruli.
- Minimal deposition of immunoglobulins.
- Compliment in glomeruli and renal vasculature can be seen.
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.
- ↑ 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.
- ↑ 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.
- ↑ 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.