Recurring posterior reversible encephalopathy syndrome in a patient with polymyositis/systemic sclerosis overlap syndrome triggered by scleroderma renal crisis.

Abstract

In posterior reversible encephalopathy syndrome (PRES) triggered by scleroderma renal crisis (SRC), modulation and adherence in immunosuppressive therapy are key for avoiding recurrence, complications, and death. A patient with polymyositis (PM)/systemic sclerosis (SSc) overlap syndrome developed PRES triggered by SRC. To our knowledge, this is the first report of a case with PRES associated with PM/SSc overlap syndrome. This manifested as altered mental status and headaches. Vasogenic edema was seen by magnetic resonance imaging in the brainstem and cerebral white matter. Antihypertension therapy resulted in improvement in both neurological symptoms and blood pressure (BP). Reversible clinical course and radiological change were consistent with PRES diagnosis. Here, the importance of BP maintenance and removal of precipitating factors of PRES is shown.