Recurrent Pericarditis As an Initial Manifestation of Autoimmune Polyglandular Syndrome Type 2

Presentation Number: SAT 398
Date of Presentation: April 1st, 2017

Boram Han*1, Sira Korpaisarn1, Jirat Chenbhanich1 and Samir Malkani2
1Metrowest Medical Center, Framingham, MA, 2University of Massachusetts Medical School, Worcester, MA


A 43-year-old Caucasian male with a past history of adrenal insufficiency and type 1 diabetes, was admitted in October 2016 with a 3 week history of left-sided pleuritic chest pain. The patient was noted to have tanned skin with diffuse hyperpigmentation. His blood pressure was 69/44 mmHg, pulse was 114/min and temperature 98.0F. Lung and heart exam were normal without friction rubs. Thyroid and neurologic exam were normal. EKG showed inferior lead ST elevation, along with elevation of troponin. Echocardiogram showed small pericardial effusion. Coronary arteries were normal on cardiac catheterization. His blood pressure responded to aggressive hydration and stress dose steroids. He had been non-compliant with adrenal replacement therapy at admission but was discharged on hydrocortisone, fludrocortisones and anti-inflammatory medications. Admission laboratory data: ESR 35mm/h (0-15), CRP 39.2 mg/L (0.0-8.0); Hgb 12.1 g/dL, WBC 10.9x109/L; sodium 149 mEq/L, potassium 4.0 mEq/L; creatinine 1.42 mg/dL; normal liver functions; HbA1c 6.7%. Anti TPO elevated, and TSH normal. Ds-DNA, c-ANCA, p-ANCA, anti-Smith antibody, TTG-IgA, and intrinsic factor antibody were all negative. Complement C3 and C4 were normal. Adrenal CT was unremarkable. The patient was diagnosed with acute pericarditis due to unknown cause.

 Prior records revealed admissions in 2008 and 2012 for left pleuritic chest pain. EKGs, cardiac enzymes and nuclear stress tests were negative. Echocardiogram revealed small pericardial effusion, and he also had a pleural effusion on chest X-ray. At that time, the patient had no signs of adrenal insufficiency with normal blood pressure and electrolytes. Adrenal insufficiency was diagnosed in 2013, when he presented with headache, hypotension, hyperpigmentation, hyponatremia and hyperkalemia. Plasma ACTH was 1250 pg/mL (9-52) and baseline cortisol <1.9 mcg/dL (normal 8am cortisol: 8-19) with no response to cosyntropin stimulation. The patient was started on hydrocortisone and fludrocortisones replacement, but he stopped these after several months.

 Autoimmune polyglandular syndrome (APS) type 2, is defined by the co-occurence of autoimmune adrenal insufficiency, thyroid disease and/or type 1 diabetes mellitus. Other manifestations include hypogonadism, vitiligo and pernicious anemia. So far, the definition does not include pericarditis. In the literature, there have been few reported cases of pericarditis associated with Addison’s disease. In APS, the occurrence of pericarditis prior to the development of adrenal insufficiency, as in this patient who only had type 1 diabetes when he presented with the initial two episodes of pericarditis, has only been reported once before. We propose that pericarditis can be the initial manifestation of APS type 2, and that the mechanism and pathophysiology of this is not currently understood.


Nothing to Disclose: BH, SK, JC, SM