A Rare Case of Sjögren's Syndrome Associated with Autoimmune Hemolytic Anemia, Palindromic Rheumatism, and Hepatitis B

Abstract

 

Background: Sjögren syndrome (SS) is a chronic systemic autoimmune disease that primarily affects exocrine glands, leading to dryness of the eyes and mouth, although multiple organ systems may also be involved. Hematological manifestations such as autoimmune hemolytic anemia (AIHA) are uncommon and may obscure the diagnosis when they predominate at disease onset. We report a case of SS presenting with severe AIHA in association with chronic hepatitis B virus (HBV) infection, where immunosuppressive therapy posed a potential risk for HBV reactivation.

Case Description: A 32-year-old woman presented with severe generalized weakness for 15 days, jaundice, and migratory joint pain. Clinical examination revealed marked pallor, icterus, tachycardia (heart rate 110 beats/min), and hepatosplenomegaly. Laboratory investigations showed hemoglobin of 2.7 g/dL with significant reticulocytosis, total bilirubin of 18 mg/dL (indirect bilirubin 14 mg/dL), elevated transaminases (AST 228 U/L, ALT 235 U/L), and a direct Coombs test positive for IgG and C3d. Autoimmune evaluation demonstrated positive antinuclear antibodies (ANA) and strongly positive SS-A, SS-B, and Ro52 antibodies. Hepatitis screening revealed positive HBsAg with a moderate HBV DNA load of 2,261 IU/mL. A diagnosis of SS associated with AIHA, palindromic rheumatism, and chronic HBV infection was established. The patient received pulse methylprednisolone for three consecutive days, followed by tapering doses of oral prednisolone. Tenofovir and hydroxychloroquine were also initiated. Gradual improvement was observed in hemoglobin levels and liver function parameters.

Conclusion: SS may present with severe AIHA as the initial and predominant manifestation, particularly in young women without classical sicca symptoms, resulting in delayed diagnosis. Concurrent evaluation for autoimmune disorders and chronic viral infections is recommended before initiating immunosuppressive therapy, especially in hepatitis B carriers, to identify potential risk factors and optimize management.