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Objective: To describe an unusual case of autoimmune polyglandular syndrome (APS) type 3 and provide a brief review of the literature., Methods: We present the clinical course and laboratory data of a patient with silent thyroiditis, isolated corticotropin (adrenocorticotropic hormone or ACTH) deficiency, alopecia universalis, and ulcerative colitis with an associated hypercoagulable state. The related literature is also reviewed briefly., Results: A 43-year-old man who had a history of ulcerative colitis with an associated hypercoagulable state and alopecia universalis was referred to the endocrinology department for evaluation of fatigue and a mildly elevated level of thyrotropin (thyroid-stimulating hormone or TSH). He previously had mildly increased TSH levels, for which low-dose levothyroxine therapy had been prescribed. During use of this therapy, a suppressed TSH level developed, necessitating discontinuation of thyroid hormone therapy; a subsequent increase in TSH value was followed by a spontaneous return to euthyroidism. An ACTH stimulation test revealed adrenal insufficiency. His ACTH level was low, 21-hydroxylase antibodies were not present, and further testing demonstrated otherwise intact pituitary function. Magnetic resonance imaging of his pituitary gland showed normal findings. Treatment with hydrocortisone promptly decreased his fatigue. He was found to have an elevated factor VIII level as the cause of his hypercoagulable state. The patient continues to feel well with use of hydrocortisone therapy and has normal thyroid function., Conclusion: This patient's components of APS type 3 have not been previously reported; thus, the complex nature of the APS variants is supported.

Objective: To present 3 cases of cardiac tamponade and addisonian crisis as the presenting features of autoimmune polyglandular syndrome (APS) type II., Methods: We present the clinical features, physical examination findings, radiographic findings, and laboratory investigations in 3 patients and provide an overview of the published cases of pericarditis and APS type II found in a MEDLINE search., Results: Patient 1, a 34-year-old woman, and Patient 3, a 35-year-old woman, had known autoimmune thyroid disease. Patient 2 was a 58-year-old man with no known history of autoimmune disease. All presented with a history of long-standing lethargy, nausea, weight loss, dyspnea, chest pain, and striking hyperpigmentation. Physical examination, laboratory, and radiographic findings were consistent with cardiac tamponade and addisonian crisis. In all 3 patients, serum cortisol was either very low or undetectable with a subnormal response to cosyntropin (250 mcg) stimulation. Cells in the pericardial fluid were predominantly neutrophils, and findings from cytology, microbiology, and serology investigations were normal. Symptoms and pericardial fluid resolved during treatment with high-dose glucocorticoids and various anti-inflammatory agents. Over the follow-up period of at least 2 years, all 3 patients experienced recurrent pericarditis and/or pleuritis despite replacement doses of glucocorticoids, which resolved with high-dose anti-inflammatory therapy., Conclusions: Pericarditis is a rare, but life-threatening manifestation of APS type II that is currently not considered to be a key manifestation of the syndrome. Our experience suggests that APS should be considered in the differential diagnosis for patients presenting with unexplained pericarditis. In patients with established APS, one should be vigilant for the development of recurrent pericarditis.

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.