Abstract

Systemic sclerosis is a multisystem autoimmune rheumatic disorder characterized by fibrosis in the skin and internal organs but rarely with hepatic or brain involvement. Reversible posterior leukoencephalopathy syndrome is characterized by a unique pattern of brain vasogenic edema in the setting of neurotoxicity predominantly in the parietal and occipital regions. We report a case of systemic sclerosis associated with probable autoimmune hepatitis that progressed rapidly to reversible posterior leukoencephalopathy syndrome with loss of vision, seizures, and coagulopathy. Brain computed tomography showed faint low density in the bilateral occipital lobes and posterior parietal lobes with edematous change. Fortunately, the patients clinical condition considerably improved 2 days following the initiation of 100 mg intravenous hydrocortisone infusion.