Sjögren's syndrome (SS) is a chronic autoimmune disorder primarily affecting exocrine glands, leading to symptoms such as dry mouth and dry eyes. While SS can occur as a primary condition, it may overlap with other autoimmune diseases, complicating management. Autoimmune hepatitis (AIH), a liver disorder characterized by elevated serum globulins and liver-specific autoantibodies, can co-occur with SS, although this overlap is rare. We report a case of a 45-year-old woman with primary SS who developed abdominal distension, pedal edema, and abnormal liver function tests, raising suspicion of an overlap syndrome. Autoimmune profiling confirmed primary SS, while liver biopsy indicated AIH and chronic liver disease with portal hypertension. The patient received immunosuppressive therapy, including corticosteroids and azathioprine, along with supportive care for cirrhosis led to stabilization. This case highlights the diagnostic challenges and the need for clinical vigilance in identifying autoimmune overlap syndromes to ensure appropriate management.
Keywords: anti-nuclear antibody; anti-ss-a antibodies; autoimmune hepatitis; autoimmune profiling; chronic liver disease; liver biopsy; overlap syndrome; portal hypertension; salivary gland disorders; sjögren's syndrome.
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