A 24-years old 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 meantime
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.