AB0702 Coexistence of systemic sclerosis and microscopic polyangitis associated with pulmonary renal syndrome: a case report and literature review

Background Systemic sclerosis is a chronic immune disease characterized by varying degrees of fibrosis skin and internal organs. Microscopic polyangitis, as subtype ANCA associated vasculitis, mainly involves small blood vessels, often manifested necrotizing glomerulonephritis pulmonary capillary vasculitis. Pulmonary renal syndrome diffuse alveolar hemorrhage based on vasculitis rapidly progressive glomerulonephritis, which can be derived from variety autoimmune diseases, accounts for about 60%[1]. The cases coexistence systemic microscopic polyangitis with in clinic are rare, dangerous easy to miss diagnosis or misdiagnosis. Objectives To investigate the clinical characteristics, treatment (SSC) polyangitis（MPA）associated (PRS). Methods data, process patient who has SSC combined MPA PRS were summarized analyzed. And literature was reviewed explore correlation pathogenesis experience complicated PRS. Results case middle-aged male diagnosed SSc due hardening both hands，Reynolds phenomenon, anti-scl-70 antibody positive. suffered repeated hemoptysis, dyspnea, severe anemia insufficiency, so he After giving glucocorticoid, immunosuppressant anti-infection treatment, his condition improved. A total 7 reports retrieved reviewing relevant literature.A patients reported.They first then PRS, 4 improved after 3 died. Among dead patients, 1 treated penicillamine years, remaining 2 only steroids without immunosuppressants.In SSc, P-ANCA closely related prognosis secondary may very poor. Most have kidney lung diseases.There certain between them pathogenesis. Glucocorticoids, immunosuppressants, biological agents, hemodialysis plasma exchange main treatments. Conclusion Although there still many reported,which reminds us: ①When new symptoms such insufficiency lungs, they should alert entities that other diseases avoid missed misdiagnosis.②ANCA detected at baseline, activity.③PRS rapid progress high mortality, whcih an emergency needs urgent treatment. Such immunosuppression immediately. However, if need extra careful when using high-dose steroids, increases risk crisis. References [1]de Groot K, Schnabel A. Das pulmorenale Syndrom [Pulmonary-renal syndrome]. Internist (Berl). 2005;46(7):769-782. doi:10.1007/s00108-005-1423-8. Figure 1. contrast chest CT before showed exudative lesions lungs significantly absorbed 2. Clinical data Acknowledgements Institute Research Center Gout Hyperuricemia Affiliated Hospital, North Sichuan Medical College Disclosure Interests None declared