Autoimmune Polyendocrine Syndrome Complicated by Pulmonary Hypertension

Endocr Metab Immune Disord Drug Targets. 2021;21(3):561-565. doi: 10.2174/1871530320666200801033106.

Abstract

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.

Keywords: Autoimmune polyendocrine syndrome; diabetes mellitus; hypoadrenalism; hypothyroidism; pulmonary arterial hypertension.

Publication types

  • Case Reports

MeSH terms

  • Addison Disease / complications*
  • Addison Disease / diagnosis
  • Addison Disease / physiopathology
  • Addison Disease / therapy
  • Diabetes Mellitus, Type 1 / complications*
  • Diabetes Mellitus, Type 1 / diagnosis
  • Diabetes Mellitus, Type 1 / physiopathology
  • Female
  • Heart Failure / diagnosis
  • Heart Failure / etiology
  • Heart Failure / physiopathology
  • Hemodynamics
  • Humans
  • Hypothyroidism / complications*
  • Hypothyroidism / diagnosis
  • Hypothyroidism / physiopathology
  • Hypothyroidism / therapy
  • Polyendocrinopathies, Autoimmune / complications*
  • Polyendocrinopathies, Autoimmune / diagnosis
  • Polyendocrinopathies, Autoimmune / physiopathology
  • Polyendocrinopathies, Autoimmune / therapy
  • Pulmonary Arterial Hypertension / diagnosis
  • Pulmonary Arterial Hypertension / etiology*
  • Pulmonary Arterial Hypertension / physiopathology
  • Pulmonary Arterial Hypertension / therapy
  • Treatment Outcome
  • Ventricular Function, Right
  • Young Adult