Microscopic polyangiitis: Difference between revisions
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==Cause== |
==Cause== |
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While the mechanism of disease has yet to be fully elucidated, the leading hypothesis is that |
While the mechanism of the disease has yet to be fully elucidated, the leading hypothesis is that AAV develops in patients with a genetic predisposition when an unknown cause triggers the production of [[p-ANCA]]. These antibodies will circulate at low levels until an environmental trigger—such as infection, malignancy, or drug therapy, causes the upregulation of neutrophils. The neutrophils bind to p-ANCAs and subsequently release inflammatory cytokines, reactive oxygen species and lytic enzymes that cause endothelial injury resulting to inflammation and necrosis of the small vessels <ref>{{Cite journal |last=Nakazawa |first=Daigo |last2=Masuda |first2=Sakiko |last3=Tomaru |first3=Utano |last4=Ishizu |first4=Akihiro |date=2019-02 |title=Pathogenesis and therapeutic interventions for ANCA-associated vasculitis |url=https://www.nature.com/articles/s41584-018-0145-y |journal=Nature Reviews Rheumatology |language=en |volume=15 |issue=2 |pages=91–101 |doi=10.1038/s41584-018-0145-y |issn=1759-4804}}</ref>. The damage that is caused in the kidneys is specifically called necrotizing and crescentic glomerulonephritis.<ref>{{cite journal |vauthors=Falk RJ, Jennette JC |title=ANCA are pathogenic—oh yes they are! |journal=J. Am. Soc. Nephrol. |volume=13 |issue=7 |pages=1977–9 |date=July 2002 |doi=10.1681/ASN.V1371977 |pmid=12089397 |url=http://jasn.asnjournals.org/cgi/pmidlookup?view=long&pmid=12089397}}</ref> |
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==Diagnosis== |
==Diagnosis== |
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Revision as of 19:09, 10 March 2023
| Microscopic polyangiitis | |
|---|---|
| Other names | Micropolyangiitis |
| Specialty | Immunology, rheumatology  |
Microscopic polyangiitis is an autoimmune disease characterized by a systemic, pauci-immune, necrotizing, small-vessel vasculitis without clinical or pathological evidence of granulomatous inflammation.
Signs and symptoms
Clinical features may include constitutional symptoms like fever, arthralgia, myalgia, loss of appetite, weight loss and fatigue. A variety of organs can be affected, which causes a wide range of symtoms such as cough, shortness of breath, hemoptysis (coughing up of blood), symtoms of kidney failure, skin manifestations (palpable purpura and livedo racemosa[1]), seizures or peripheral neuropathy, abdominal pain [2]
The kidneys are affected in up to 80% of cases with signs of blood and protein in the urine and the injury can lead to either rapidly or slowly progressive kidney failure. The lungs are affected in 20-50% of cases with findings of pulmonary hemorrhage, or chronic pulmonary fibrosis leading to respiratory failure.[3]
Cause
While the mechanism of the disease has yet to be fully elucidated, the leading hypothesis is that AAV develops in patients with a genetic predisposition when an unknown cause triggers the production of p-ANCA. These antibodies will circulate at low levels until an environmental trigger—such as infection, malignancy, or drug therapy, causes the upregulation of neutrophils. The neutrophils bind to p-ANCAs and subsequently release inflammatory cytokines, reactive oxygen species and lytic enzymes that cause endothelial injury resulting to inflammation and necrosis of the small vessels [4]. The damage that is caused in the kidneys is specifically called necrotizing and crescentic glomerulonephritis.[5]
Diagnosis
Laboratory tests may reveal an increased sedimentation rate, elevated CRP, anemia and elevated creatinine due to kidney impairment. An important diagnostic test is the presence of perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) with myeloperoxidase specificity[6] (a constituent of neutrophil granules), and protein and red blood cells in the urine.
In patients with neuropathy, electromyography may reveal a sensorimotor peripheral neuropathy. [citation needed]
Differential diagnosis
The signs and symptoms of microscopic polyangiitis may resemble those of granulomatosis with polyangiitis (GPA) (another form of small-vessel vasculitis) but typically lacks the significant upper respiratory tract involvement (e.g., sinusitis) frequently seen in people affected by GPA.[citation needed]
Treatment
Immunsuppressive treatment is the gold standard management in order to achieve remission of the blood vessel inflammation that occurs in active microscopic polyangitis. The current immunosuppressive protocols consists of a combination of high dose of glucocorticoids in combination with either cyclophosphamide or Rituximab.[7] In cases of life threatening disease treatment with plasmapheresis can also be applied.
The immunosuppressive treatment is slowly tapered down under a period of several months but there is at the moment no consensus about the total duration of the therapy. Discontinuation of immunsuppression can be related to increased risk for disease flares.
See also
- ANCA-associated vasculitides
- Polyarteritis nodosa
- List of cutaneous conditions
- Granulomatosis with polyangitis
References
- ^ Nagai Y, Hasegawa M, Igarashi N, Tanaka S, Yamanaka M, Ishikawa O (December 2008). "Cutaneous manifestations and histological features of microscopic polyangiitis". Eur J Dermatol. 19 (1): 57–60. doi:10.1684/ejd.2008.0566. PMID 19059827.
- ^ Chung, Sharon A.; Seo, Philip (August 2010). "Microscopic Polyangiitis". Rheumatic Disease Clinics of North America. 36 (3): 545–558. doi:10.1016/j.rdc.2010.04.003. ISSN 0889-857X. PMC 2917831. PMID 20688249.
- ^ Karras, Alexandre (August 2018). "Microscopic Polyangiitis: New Insights into Pathogenesis, Clinical Features and Therapy". Seminars in Respiratory and Critical Care Medicine. 39 (4): 459–464. doi:10.1055/s-0038-1673387. ISSN 1069-3424. PMID 30404112. S2CID 53207328.
- ^ Nakazawa, Daigo; Masuda, Sakiko; Tomaru, Utano; Ishizu, Akihiro (2019-02). "Pathogenesis and therapeutic interventions for ANCA-associated vasculitis". Nature Reviews Rheumatology. 15 (2): 91–101. doi:10.1038/s41584-018-0145-y. ISSN 1759-4804.
{{cite journal}}: Check date values in:|date=(help) - ^ Falk RJ, Jennette JC (July 2002). "ANCA are pathogenic—oh yes they are!". J. Am. Soc. Nephrol. 13 (7): 1977–9. doi:10.1681/ASN.V1371977. PMID 12089397.
- ^ Seishima M, Oyama Z, Oda M (2004). "Skin eruptions associated with microscopic polyangiitis". Eur J Dermatol. 14 (4): 255–8. PMID 15319159.
- ^ Geetha, Duvuru; Jefferson, J. Ashley (2020-01-01). "ANCA-Associated Vasculitis: Core Curriculum 2020". American Journal of Kidney Diseases. 75 (1): 124–137. doi:10.1053/j.ajkd.2019.04.031. ISSN 0272-6386. PMID 31358311. S2CID 198983998.