Article Text

  1. T. D. Nielsen,
  2. C. Steenbergen,
  3. S. D. Russell,
  4. W. J. Rogers
  1. University of Alabama at Birmingham, Birmingham


Background A case of non-ischemic cardiomyopathy associated with polyglandular autoimmune syndrome type II (PASII) is presented. This syndrome consists of Addison's disease in combination with either autoimmune thyroid disease or type 1 diabetes, and it commonly includes hypogonadism. Associations between PASII and multiple sclerosis, celiac disease, rheumatoid arthritis and primary biliary cirrhosis have been reported. While cardiomyopathy of presumed autoimmune origin has been associated with other autoimmune disorders including systemic lupus erythematosis and celiac disease, association with PASII has not previously been described.

Case The patient is a white male who from age 32-37 was diagnosed with type 1 diabetes mellitus, Crohn's disease, Addison's disease, and hypogonadism. At age 39 he presented with severe congestive heart failure (CHF). He was found to have normal coronary arteries, an ejection fraction of less than 15% with global hypokinesis, and trifascicular block with right bundle branch block, left anterior fascicular block, and 1st degree AV block. Anti-cardiac myosin antibodies were present. Treatment of his CHF was complicated by the coexisting Addison's disease. His heart failure progressed rapidly and he required a continuous dobutamine infusion, placement of an intra-aortic balloon pump, and later a left ventricular assist device. A donor heart eventually became available and cardiac transplant was performed. Pathology of his native heart confirmed a chronic autoimmune process.

Follow-up He has done well after transplant and at last follow-up had no complaints. He was recently able to take a 7-8 mile hike with his son and reported only mild dyspnea on walking up steep hills. He has had no severe rejection episodes. His Crohn's disease has been in complete remission, presumably due to his immunosuppressive medications.

Conclusions While autoimmune cardiomyopathy has been associated with other autoimmune disorders, this is the first known case of cardiomyopathy in association with a polyglandular autoimmune syndrome.

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