Objective: 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. Methods: We conducted a systematic literature review of articles reporting on ANCA-positive RPF from the database inception dates until March 8, 2020. Results: 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 involvement (rapidly progressive glomerulonephritis) in 31.6% of patients. Necrosis and granulomatous inflammation, vasculitis, and multinucleated giant cells were pathologically observed in tissue sections of RPF, whereas tertiary lymphoid organ formation was not identified. Glucocorticoids with or without other immunosuppressive treatments were effective in most patients, but 4 patients experienced disease relapse during the clinical course. All relapsed patients were positive for PR3-ANCA. Conclusion: Clinical features of ANCA-positive RPF are associated with systemic inflammatory components such as fever and elevated serum C-reactive protein levels. ANCA-. positive RPF presents 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.
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