Primary Sjogren’s syndrome presenting as acute pancreatitis and interstitial nephritis: A diagnostic challenge

Primary Sjogren’s syndrome (pSS) is an autoimmune disorder primarily affecting the exocrine glands, with systemic involvement being increasingly recognized. Presentation with gastrointestinal involvement is a rare presentation of the disease. We report the case of a 72-year-old woman who presented with cholestatic jaundice, fever, and acute pancreatitis. During hospital stay, she also developed pulmonary involvement and an active urinary sediment, which significantly complicated the diagnosis. Serological testing revealed a strongly positive antinuclear antibody (ANA) titer along with anti-Smith antibodies, raising a strong suspicion for systemic lupus erythematosus (SLE). However, renal biopsy demonstrated a plasma cell predominant tubulointerstitial nephritis without immune complex deposition. This pattern effectively ruled out lupus nephritis and established the diagnosis of primary Sjogren’s syndrome. This case highlights the diagnostic challenge posed by overlapping autoimmune features and reinforces the importance of tissue biopsy in differentiating autoimmune mimics. Early recognition of atypical extra-glandular manifestations of pSS is essential for appropriate management and improved outcomes.