Recurrent Tamponade – a Rare Presentation of Autoimmune Polyglandular Syndrome Type II

Presentation Number: SUN 367
Date of Presentation: April 2nd, 2017

Perihan Dimachkie*1, Kamaldeep Panach2, Minnie Keon3 and Ugis Gruntmanis4
1University Of Texas Southwestern Medical Center, Dallas, TX, 2UTSW Medical Center, Dallas, TX, 3UT Southwestern Medical School, Dallas, TX, 4UT Southwestern Medical Center, Dallas, TX

Abstract

Background: This case reports an unusual presentation of autoimmune polyglandular syndrome type II (APS II) with recurrent cardiac tamponade due to pericarditis in a young man.

Clinical case: Our patient is a 29-year-old Hispanic man with a history of primary hypothyroidism and vitiligo who presented to the emergency department complaining of chest pain. He was found to have a pericardial effusion with tamponade physiology. He rapidly became hemodynamically unstable and underwent pericardiocentesis, after which he was admitted to the ICU. The patient gradually recovered and was discharged after 15 days. The underlying etiology of his illness remained unclear. Four months later, he presented with similar symptoms and was diagnosed with recurrent cardiac tamponade. Once again he became hemodynamically unstable and underwent pericardiocentesis. During this presentation, he reported a one year history of poor appetite secondary to nausea, abdominal pain, and a 40 lb weight loss. Given these complaints in addition to notable hyperpigmentation on physical exam; a cortisol level was checked. His serum cortisol level was found to be undetectable, and endocrinology were consulted. Further work up confirmed autoimmune etiology of adrenal insufficiency with positive 21-OH antibodies (9 U/ml, ref : <1 U/ml). The presence of hypothyroidism with positive anti- TPO (52.5 IU/ml, ref: <9 IU/ml) and of primary autoimmune adrenal insufficiency suggested the diagnosis of APS type II. Further labs revealed positive GAD antibodies (>250 IU/ml , ref: 0-5 IU/ml); but no evidence of diabetes thus far.

The patient was later discharged from the hospital on maintenance doses of hydrocortisone and fludrocortisone. He was also discharged on indomethacin and colchicine for the treatment of pericarditis. The patient followed up with cardiovascular thoracic surgery and eventually had a pericardial window given the recurrent nature of his disease.

Conclusion: Five case reports consisting of a total of ten patients have previously reported cardiac tamponade as a serious manifestation of APS II. Our case provides another example that cardiac tamponade is a rare but clinically significant presentation. Our case also suggests the recurrent nature of cardiac tamponade in patients with APS II that may require definitive therapy. Accurate diagnosis and timely hormone replacement with monitoring for cardiac complications can prevent fatal outcomes in these patients.

 

Nothing to Disclose: PD, KP, MK, UG