Nonischemic cardiomyopathy associated with autoimmune polyglandular syndrome type II.

OBJECTIVE

To report a case of nonischemic cardiomyopathy associated with autoimmune polyglandular syndrome type II (APS-II).

METHODS

We describe our patient's clinical features, evaluation, and outcome. In addition, a literature review of cardiomyopathy associated with polyendocrinopathy syndromes is presented.

RESULTS

The component disorders of APS-II are Addison's disease in combination with either autoimmune thyroid disease or type 1 (insulin-dependent) diabetes. Although numerous other autoimmune conditions have been reported in conjunction with APS-II, cardiomyopathy has not been previously described as part of this syndrome. The current patient was a 32-year-old man who, during a 5-year period, was diagnosed as having type 1 diabetes mellitus, Crohn's disease, and Addison's disease. In 2001, he presented with severe heart failure that progressed rapidly and eventually necessitated cardiac transplantation.

CONCLUSION

Although autoimmune cardiomyopathy has been associated with other autoimmune disorders, to our knowledge this is the first reported case of cardiomyopathy in association with an autoimmune polyglandular syndrome. Patients with this syndrome should undergo clinical evaluation for heart failure.