Mixed cholangiopathy with eosinophilic and autoimmune features: a case report

Eosinophilic cholangitis and autoimmune cholangiopathy are two rare immune-mediated biliary disorders with distinct demographic, clinical, and histopathological features. Eosinophilic cholangitis typically presents in younger men with eosinophilia and biliary wall changes, while autoimmune cholangiopathy more often affects women and is characterized by positive autoimmune serology. We present a case of a young adult male who exhibited clinical and histological features of both diseases which presented a significant diagnostic and management challenge.

An 18-year-old Caucasian man from Australia with obesity presented with 2 weeks of abdominal pain, lethargy, anorexia, and subjective fevers. He was afebrile, hemodynamically stable, and physical examination was unremarkable. Laboratory tests revealed eosinophilia, elevated inflammatory markers, and cholestatic liver enzyme derangement. Autoimmune serology was notable for high-titer antinuclear antibodies and anti-smooth muscle antibodies, as well as myeloperoxidase anti-neutrophil cytoplasmic antibody, and anti-mitochondrial antibodies with hypergammaglobulinemia. Infective and parasitic screens were negative. Imaging demonstrated only mild biliary prominence without strictures or obstructive lesions. Liver biopsy revealed bile duct destruction, eosinophilic infiltration, ductular proliferation, and periportal fibrosis, suggestive of eosinophilic cholangitis. Given the strong autoimmune profile, an overlap with autoimmune cholangiopathy was considered. The patient improved rapidly with corticosteroids but remained steroid-dependent despite azathioprine. Long-term treatment with azathioprine, budesonide, and ursodeoxycholic acid resulted in sustained biochemical improvement and clinical stability over 2 years.

This represents the first reported case of overlapping eosinophilic and autoimmune cholangiopathy. It highlights the heterogeneity of immune-mediated biliary disorders and the importance of integrating clinical, serological, radiological, and histological data. Management remains centered on immunosuppression, with careful longitudinal monitoring required to guide therapy and prevent relapse.