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https://www.selleckchem.com/products/sumatriptan.html Anti-neutrophil cytoplasmic autoantibody (ANCA)-positive retroperitoneal fibrosis (RPF) is extremely rare. This study aimed to clarify the clinical characteristics and prognosis of patients with ANCA-positive RPF. We conducted a systematic literature review of articles reporting on ANCA-positive RPF from the database inception dates until March 8, 2020. We identified 19 patients with ANCA-positive RPF with a mean age of 62 years; a male dominance (68.4%) was noted. Most patients presented with systemic symptoms and/or lower back or abdominal pain. Proteinase 3 (PR3) -ANCA positivity was predominant compared with myeloperoxidase (MPO)-ANCA (63.2% vs. 36.8%, respectively), and all patients showed elevated serum C-reactive protein levels. Of note, 26.7% of patients had isolated RPF without any other ANCA-associated systemic organ involvement. Regarding typical manifestations of ANCA- associated vasculitis, ear, nose, and throat involvement occurred in 26.3%, lung involvement in 36.8%, and kidney involvemen as an "isolated" involved organ in one-third of patients. Immunosuppressive treatments are effective, but the disease can recur, particularly in PR3-ANCA-positive patients.Patients with antiphospholipid syndrome (APS) present with clinical features of recurrent thrombosis and pregnancy morbidity and persistently test positive for the presence of antiphospholipid antibodies (aPL). At least one clinical (vascular thrombosis or pregnancy morbidity) and one lab-based (positive test result for lupus anticoagulant, anticardiolipin antibodies and/or anti-β2-glycoprotein 1 antibodies) criterion have to be met for a patient to be classified as having APS. Nevertheless, the clinical variety of APS encompasses additional signs and symptoms, potentially affecting any organ, that cannot be explained exclusively by a prothrombotic state. Those manifestations, also known as extra-criteria manifestations, include haematologic (th

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