Autoimmune Hemolytic Anemia with Systemic Lupus Erythematosus and Primary Biliary Cholangitis Overlap Syndrome in a 25-year-old Female: A Rare Case Report

Abstract

Autoimmune hemolytic anemia (AIHA) is an uncommon hematologic manifestation of systemic autoimmune diseases, most frequently associated with systemic lupus erythematosus (SLE). Primary biliary cholangitis (PBC) is a chronic autoimmune cholestatic liver disorder that rarely coexists with SLE. An overlap syndrome involving AIHA, SLE, and PBC is exceptionally rare and poses significant diagnostic and therapeutic challenges. We report the case of a 25-year-old female presenting with progressive jaundice, fatigue, and pallor. Laboratory evaluation revealed hemolytic anemia with elevated lactate dehydrogenase, reticulocytosis, and a positive direct Coombs test, consistent with AIHA. Autoimmune serology demonstrated high-titer antinuclear antibody, anti-double-stranded deoxyribonucleic acid positivity, and hypocomplementemia, supporting SLE. Persistent cholestatic liver enzyme elevation, AMA-M2 positivity, and supportive imaging findings indicated PBC. The patient improved following corticosteroids, immunosuppressive therapy, and ursodeoxycholic acid. This case emphasizes the importance of early recognition of multisystem autoimmune overlap syndromes to guide appropriate management and improve outcomes.