Correspondence to Author: Rahul Siwach, Parveen Malhotra, Bibin CF, Avani Sharma, Abhishek Yadav, Chitrakshi Bhardwaj, Himanshu, Shivanshu, Rajasvi, Taranjot Singh.. 

Department of Medical Gastroenterology, PGIMS, Rohtak, Haryana, India.

DOI: 10.52338/jjogastro.2026.5510

Abstract:

Introduction: Autoimmune hepatitis–primary biliary cholangitis (AIH–PBC) M overlap syndrome affects 10–20% of patients with either disease, blending features of both and complicating therapy. Long-term outcomes include cirrhosis, portal hypertension, and variceal bleeding.
Case report: A thirty-three-year-old female, not a known case of any chronic illness presented nine years back with abdominal pain which was symptomatically treated and ultrasonogram abdomen done at that point of time revealed mild splenomegaly, for which no definitive treatment was started. One year later, she again developed pain abdomen, generalized fatigue and abdominal distension. She was seen by local private practitioner, on whose advice, routine evaluation revealed anemia, thrombocytopenia, mildly raised transaminitis with ratio reversal, low serum albumin level. The ultrasound abdomen showed small coarse liver, splenomegaly and ascites. After stabilization, liver biopsy was done which confirmed the diagnosis of Primary Biliary Cirrhosis with stage-2, and on serology workup overlap syndrome with autoimmune hepatitis was confirmed (ANA 166.42, weakly positive AMA 1:20, IgG 2118 mg/dL). She was started on ursodeoxycholic acid, immunosuppressive therapy, diuretics, beta-blockers in addition to symptomatic therapy. She had two endoscopic variceal band ligations for large oesophageal varices. She remained stable for next nine years but stopped immunosuppressive treatment on her own for few months and later on again presented with increased decompensation in form of excessive fatigue, menorrhagia, ascites, and pedal edema. She was restarted on immunosuppressive therapy, in addition to rest previously going treatment. She got stabilized, pedal edema & ascites subsided and fatigue decreased and was discharged under haemodynamically stable condition on above therapy. This case illustrates need of regular long-term treatment, including immunosuppressive treatment, even in cirrhotic stage for preventing recurrent decompensation.
Conclusion: AIH–PBC overlap syndrome requires lifelong, multimodal management. Despite initial control, recurrent varices and decompensation underscore the need for vigilant endoscopic surveillance, optimized beta-blockade, and consideration of adjunctive therapies (e.g., TIPS) in refractory portal hypertension.

Citation:

Parveen Malhotra, Overlap Syndrome Of Autoimmune Hepatitis And Primary Biliary Cholangitis: Nine Year Follow-Up. Japanese Journal of Gastroenterology 2026.