Hepatic granulomas as a manifestation of ANCA-associated vasculitis:a systematic review

T. Ben Achour, Nesrine Ben Hadj Dahman, Maysam Jridi, Fatma Said, I. Ben Ghorbel, I. Naceur, Monia Smiti

Background ANCA-associated vasculitides (AAV) — granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA) — are rare small-vessel autoimmune diseases. Liver involvement in AAV is uncommon and generally manifests as biochemical hepatitis; true hepatic granulomatosis is exceedingly rare and diagnostically challenging. Methods We conducted a systematic scoping review following PRISMA-ScR guidelines and the Arksey & O’Malley framework, searching PubMed, Google Scholar, ScienceDirect, and Scopus without date restriction (through March 2026). A total of 7, 033 records were initially retrieved; after deduplication, title/abstract screening, and full-text review, five articles meeting strict inclusion criteria were included for qualitative synthesis. Results Five published cases of hepatic granulomas in AAV patients with no confirmed confounding etiology were identified. All were GPA or EGPA; no case of pure MPA was documented. Three patients were female and two were male, with a mean age of 57.6 years. Liver histology revealed non-necrotizing epithelioid granulomas (n = 2), granulomatous inflammation with necrosis (n = 1), incomplete septal cirrhosis with vasculopathic changes (n = 1), and incidental calcified granulomas (n = 1). Immunosuppressive therapy with corticosteroids and/or cyclophosphamide achieved clinical and biochemical improvement in all treated patients. Conclusions Hepatic granulomatosis is a rare but genuine extra-respiratory manifestation of AAV, most frequently reported in GPA. It may antedate the canonical ENT-pulmonary-renal triad, presenting as incidental hepatomegaly or unexplained liver function test elevation. Systematic exclusion of competing etiologies (sarcoidosis, tuberculosis, primary biliary cholangitis, drug-induced hepatitis) is mandatory before attributing granulomas to AAV. Liver biopsy remains pivotal in confirming the diagnosis. Immunosuppression is the therapeutic cornerstone, with generally favourable outcomes.