Pulmonary hypertension in a patient with Schmidt syndrome.

We describe a 26-year-old patient with long-standing autoimmune hypothyroidism. She was doing well until she developed Addisonian crisis accompanied by severe metabolic acidosis, hypoglycemia, hypomagnesemia, and hypokalemia. Subsequently she developed a life-threatening cardiac arrhythmia due to QT prolongation secondary to electrolyte imbalance. The association of autoimmune hypothyroidism and adrenal insufficiency in our patient suggests the diagnosis of autoimmune polyglandular syndrome type II or Schmidt syndrome. An echocardiography that was performed detected pulmonary hypertension without apparent cardiac or lung pathology. The association of pulmonary hypertension and Schmidt syndrome is rare and may be explained by a generalized immune activation leading to pulmonary endothelial damage or dysfunction.