# A Case Report of Combined Type 2 Autoimmune Hepatitis and Antiphospholipid Syndrome Presenting With Intracranial Hemorrhage: Diagnostic and Therapeutic Dilemma

## TL;DR

This case report describes a rare combination of type 2 autoimmune hepatitis and antiphospholipid syndrome leading to severe complications and death.

## Contribution

Highlights the rare association of type 2 AIH and APS, and the challenges in managing their combined complications.

## Key findings

- The patient had type 2 AIH and APS, a rare combination.
- PSAE improved thrombocytopenia but led to intracranial hemorrhage.
- Platelet dysfunction was identified despite normal coagulation tests.

## Abstract

Background: Autoimmune hepatitis (AIH) is very frequently associated with many autoimmune diseases. Antiphospholipid syndrome (APS) is frequently associated with type 1 AIH but rarely seen in type 2 AIH patients. In this case, the association between type 2 AIH and APS is seen, along with complications, which made the management difficult.

Case presentation: This patient, who had a history of recurrent abortions, presented with complaints of recurrent epistaxis with severe thrombocytopenia. She was diagnosed with APS with concomitant type 2 AIH. After failing with conservative treatment, partial splenic artery embolization (PSAE) for recurrent thrombocytopenia was done, which led to improvement in platelet count and the resolution of recurrent epistaxis. But after a few days, the patient developed an intracranial hemorrhage (ICH) with a normal platelet count and coagulation profile. A platelet function study was done, and platelet dysfunction was found. Finally, after a prolonged period of admission, the patient developed sepsis and disseminated intravascular coagulation (DIC) and succumbed to death.

Conclusion: From this case, it may be kept in mind the possibility of the association of both types of AIH when dealing with a patient with APS. Unconventional methods such as partial splenic artery embolization can be useful in patients with recurrent thrombocytopenia. A platelet function study is a very important parameter to look for whenever a patient has bleeding with a normal coagulation profile and platelet count with isolated raised activated partial thromboplastin time (aPTT).