Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) encompass a group of rare autoimmune diseases characterized by granuloma formation and/or inflammation of small vessels. The clinical spectrum of AAV varies widely. Liver involvement is exceptional, posing diagnostic challenges. Moreover, AAV can co-exist with other systemic diseases, further complicating the diagnosis. We herein present a unique case of AAV overlapping with Sjögren’s syndrome (SS) with an uncommon onset of the disease.
A 47-year-old female was admitted for hiatal hernia surgery. During the intervention, nodular hepatomegaly was observed. A liver biopsy was performed, showing non-necrotizing epithelioid and central giant cell granulomas. Computed tomography (CT) scan showed perilymphatic pulmonary micronodules with bilateral hilar lymphadenopathies, raising the suspicion of sarcoidosis. Minor salivary gland biopsy revealed Chisholm grade 3 sialadenitis, which, along with the patient’s dry eye and mouth symptoms, confirmed SS. Immunological workup showed negative antinuclear antibodies and positive anti-myeloperoxidase (MPO) antibodies. A year later, the patient presented with asthma flare-ups and ear, nose, and throat (ENT) symptoms. A nasal biopsy showed signs of eosinophilic leukocytoclastic vasculitis, confirming the diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA). Oral steroid therapy was initiated, which resulted in clinical improvement. We present a case highlighting that EGPA is a protean disease, possibly mimicking or co-occurring with other autoimmune disorders. Thus, it is important to consider the differential diagnoses and carefully monitor for any new symptom or organ dysfunction.