Autoimmune Polyendocrine Syndrome complicated by Pulmonary Hypertension.

A 24-yo female was admitted for acute renal failure, melanoderma, hyponatremia and hyperkalemia. The clinical suspicion of Addison's disease was confirmed by laboratory test and the appropriate replacement therapy with corticosteroids and fludrocortisone was started. In the mean-time primary hypothyroidism and diabetes mellitus type 1 were disclosed and treated, thus fulfilling a diagnosis of autoimmune polyendocrine syndrome type 2. Eighteen months later she was admitted for right sided heart failure. The work-up allowed to diagnose pulmonary arterial hypertension. Here we report the clinical course and discuss the putative link between these two rare diseases.