| 词条 | Microscopic polyangiitis |
| 释义 |
| synonyms = Micropolyangiitis | name = Microscopic polyangiitis | image = | caption = | pronounce = | field = | symptoms = | complications = | onset = | duration = | types = | causes = | risks = | diagnosis = | differential = | prevention = | treatment = | medication = | prognosis = | frequency = | deaths = }} Microscopic polyangiitis is an ill-defined autoimmune disease characterized by a systemic, pauci-immune, necrotizing, small-vessel vasculitis without clinical or pathological evidence of necrotizing granulomatous inflammation. Signs and symptomsClinical features may include constitutional symptoms like fever, loss of appetite, weight loss, fatigue, and kidney failure.[1] A majority of patients may have blood in the urine and protein in the urine. Rapidly progressive glomerulonephritis may occur. Because many different organ systems may be involved, a wide range of symptoms are possible in MPA. Purpura and livedo racemosa may be present.[2]CauseWhile the mechanism of disease has yet to be fully elucidated, the leading hypothesis is that the process is begun with an autoimmune process of unknown cause that triggers production of p-ANCA. These antibodies will circulate at low levels until a pro-inflammatory trigger — such as infection, malignancy, or drug therapy. The trigger upregulates production of p-ANCA. Then, the large number of antibodies make it more likely that they will bind a neutrophil. Once bound, the neutrophil degranulates. The degranulation releases toxins that cause endothelial injury.[3] Most recently, two different groups of investigators have demonstrated that anti-MPO antibodies alone can cause necrotizing and crescentic glomerulonephritis.[4] DiagnosisLaboratory 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[5] (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. Differential diagnosisThe 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. TreatmentThe customary treatment involves long term dosage of prednisone, alternated or combined with cytotoxic drugs, such as cyclophosphamide or azathioprine. Plasmapheresis may also be indicated in the acute setting to remove ANCA antibodies. Rituximab has been investigated,[6] and in April 2011 approved by the FDA when used in combination with glucocorticoids in adult patients.[7]See also
References1. ^{{cite journal |vauthors=Altaie R, Ditizio F, Fahy GT |title=Microscopic polyangitis presenting with sub-acute reversible optic neuropathy |journal=Eye (Lond) |volume=19 |issue=3 |pages=363–5 |date=March 2005 |pmid=15272290 |doi=10.1038/sj.eye.6701479 }} 2. ^{{cite journal |vauthors=Nagai Y, Hasegawa M, Igarashi N, Tanaka S, Yamanaka M, Ishikawa O |title=Cutaneous manifestations and histological features of microscopic polyangiitis |journal=Eur J Dermatol |volume= 19|issue= 1|pages= 57–60|date=December 2008 |pmid=19059827 |doi=10.1684/ejd.2008.0566 }} 3. ^{{cite journal |vauthors=Xiao H, Heeringa P, Hu P, etal |title=Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidase cause glomerulonephritis and vasculitis in mice |journal=J. Clin. Invest. |volume=110 |issue=7 |pages=955–63 |date=October 2002 |pmid=12370273 |pmc=151154 |doi=10.1172/JCI15918}} 4. ^{{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 |pmid=12089397 |url=http://jasn.asnjournals.org/cgi/pmidlookup?view=long&pmid=12089397}} 5. ^{{cite journal |vauthors=Seishima M, Oyama Z, Oda M |title=Skin eruptions associated with microscopic polyangiitis |journal=Eur J Dermatol |volume=14 |issue=4 |pages=255–8 |year=2004 |pmid=15319159 |url=http://www.john-libbey-eurotext.fr/medline.md?issn=1167-1122&vol=14&iss=4&page=255}} 6. ^{{cite journal |author=Jayne D |pmid=18281850 |doi=10.1097/BOR.0b013e3282f370d1 |title=Challenges in the management of microscopic polyangiitis: past, present and future |journal=Curr Opin Rheumatol |volume=20 |issue=1 |pages=3–9 |date=January 2008 |url=http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?an=00002281-200801000-00003 }} 7. ^Sources*{{cite press release |title=FDA approves Rituxan to treat two rare disorders |publisher=Food and Drug Administration |url=http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm251946.htm |accessdate=20 April 2011 |date=19 April 2011 }}*{{cite web |title=RITUXAN (rituximab) injection, solution |work=Daily Med |publisher=U.S. National Library of Medicine |url=http://dailymed.nlm.nih.gov/dailymed/lookup.cfm?setid=b172773b-3905-4a1c-ad95-bab4b6126563}} External links{{Medical resources| DiseasesDB = 8193 | ICD10 = {{ICD10|M|31|7|m|30}} | ICD9 = {{ICD9|446.0}} | ICDO = | OMIM = | MedlinePlus = | eMedicineSubj = med | eMedicineTopic = 2931 | MeshID = D055953 }}{{Systemic vasculitis}}{{Urologic disease}} 3 : Steroid-responsive inflammatory conditions|Systemic connective tissue disorders|Vascular-related cutaneous conditions |
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