Overlap Syndrome: Primary Sclerosing Cholangitis with Sjogren?s Syndrome

Dr. Manjiri Naik, Dr. Kartik Doshi, Dr. Rajiv Naik, Dr. Umar Quadri, Dr. Sushen Ghadge, Dr. Nilofer Patel, Dr. Arshad Ali, Dr. Abhay Bhosale, Dr. Tanvi Shingavi, Dr. Praful Kadam

Abstract: The co - occurrence of Small Duct Primary Sclerosing Cholangitis (PSC) and Sj?gren?s Syndrome presents a clinical puzzle that demands a refined lens for diagnosis. This case study of a 22 - year - old female, who presented with vague and overlapping symptoms like fatigue, jaundice, pruritus, and xerostomia, sheds light on a truly rare autoimmune overlap. Despite initial normal imaging and non - specific lab results, a strong clinical suspicion prompted a liver biopsy, which confirmed small duct PSC an entity less aggressive yet elusive in presentation. The concurrent positivity for anti - Ro and anti - La antibodies, alongside signs of glandular dysfunction, established the presence of Sj?gren?s Syndrome. What makes this case particularly meaningful is not just its rarity, but the way it illustrates how traditional diagnostic paths may fall short in detecting such overlap syndromes. It is evident that when faced with autoimmune ambiguity, a multidisciplinary approach anchored in persistence can make all the difference. The patient?s partial response to immunosuppressive and symptomatic therapy further emphasizes the need for vigilant long - term management and consideration for transplant in refractory cases. This suggests that while small duct PSC and Sj?gren?s may seem like distant clinical cousins, their intersection especially in the Indian context invites both curiosity and caution. Ultimately, this report does more than add to the literature; it underscores the necessity for broader awareness and nuanced protocols in handling rare autoimmune intersections.

Keywords: Small duct PSC, Sj?gren?s syndrome, autoimmune overlap, liver biopsy diagnosis