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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 abdomin
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