Abstract
Autoimmune Hepatitis – Primary Biliary Cirrhosis (AIH-PBC) Overlap Syndrome Unmasked During Anti-Tuberculosis Therapy
Tristan Paulo Madrigal, MD; Katrina Austero, MD; Mara Teresa Panlilio, MD
University of the Philippines – Philippine General Hospital
Background: Autoimmune hepatitis – primary biliary cirrhosis (AIH-PBC) overlap syndrome represents a significant yet underrecognized variant of autoimmune liver disorders, accounting for less than 10% of cases. Patients present with a mixed hepatocellular and cholestatic pattern of liver injury. The diagnosis is often delayed, particularly when drug-induced liver injury (DILI) is being considered, as it exhibits a similar pattern of liver test abnormalities.
Clinical Presentation: A 22-year-old female presented with a 4-month history of jaundice and pruritus after starting anti-tuberculosis therapy (ATT) for pulmonary tuberculosis. Three weeks into treatment, she developed jaundice and generalized pruritus, prompting discontinuation of the ATT. She was initially given silymarin and phospholipids, but her symptoms did not improve. She eventually consulted at our institution due to worsening symptoms and liver tests . Initial laboratory investigations revealed a mixed hepatocellular and cholestatic pattern of liver injury, raising suspicion for underlying chronic liver disease. MRCP showed a normal liver and biliary tree, and while ANA was positive, AMA, anti-LKM-1, and anti-SMA were negative. A liver biopsy revealed portal inflammation, ductular reaction, and interface hepatitis. She was treated with ursodeoxycholic acid and prednisone, resulting in clinical improvement and resolution of jaundice within weeks.
Conclusion: AIH-PBC overlap syndrome poses a diagnostic challenge, particularly when it mimics DILI. This case highlights the importance of excluding a chronic liver disease in cases of persistent liver injury even after discontinuing the suspected drug. Prompt recognition and initiation of immunosuppressive therapy and UDCA are crucial for improving outcomes and preventing disease progression.
Important Dates to Remember
| JRRE Activities 2023-2024 |
Proposed Dates |
|---|---|
| Resumption of face-to-face Evidence-Based Medicine Workshop Venue to be announced once finalized. |
July 21, 2023 (Friday), 1-5pm |
| Monthly Critical Appraisal of Topics Conferences to be gaciliated by Research Coordinators per Institution | Monthly Critical Appraisal of Topics Conferences per Institution |
| CAT Plenary/Liver Con Dates: September 23, 2023 December 16, 2023 March 2024 (During the JAC) Resumption of face-to-face Quarterly Critical Appraisal of Topics facilitated by the JRRE |
2 Clinical scenarios and articles are to be sent to institutions each on August 2023, November 2023, and February 2024; |
| Hybrid Research Workshop 4 week asynchronous via Moodle, followed by a 1 day on-site Onsite Workshop Research Protocol Presentation for Level I GI Fellows-In-Training - Research Protocol Presentation: August 12, 2023 - Research Forums of the Tripartite Societies |
Week 1: July 15-21, 2023 Week 2: July 22-28, 2023 Week 3: July 29-August 4, 2023 Week 4: August 5-11, 2023 |
| Research Manuscript Workshop for level II GI Fellows-In-Training | January 6, 2024 |