Autoimmune hepatitis and myositis as presenting manifestation of mixed connective tissue disease – A case report

Abstract

In the course of mixed connective tissue disease (MCTD), hepatitis is very rare. Most commonly liver steatosis or elevated liver enzymes are reported and only a few cases of MCTD associated with autoimmune hepatitis were described previously. We report a case of 30-year-old female, who initially presented with autoimmune hepatitis and was treated with oral Prednisolone. Few weeks later, she presented with lower motor neuron weakness of all four limbs with normal reflexes and sensory findings. Clinically, the pattern of weakness was suggestive of a myopathic process which was confirmed with a high creatine phosphokinase levels and electromyography. She had history of Raynaud’s phenomenon with minimal skin thickening involving the extremities and face. Thus, a diagnosis of overlap syndrome/MCTD was kept. MCTD has been confirmed on high titers positive anti U-1 ribonucleoprotein antibodies. Autoimmune hepatitis and myositis as the presenting manifestation are very rare. Here, we highlight a patient of MCTD with initial symptoms of autoimmune hepatitis and myositis.