Endocrine Abstracts

Introduction: Addison Disease is a rare condition with an estimated incidence in the developed world of 0.8 cases per 100 000 population. It is associated with relevant morbidity and mortality rates, but once the diagnosis is made it can be easily managed. When Addison Disease is associated with other autoimmune diseases, the diagnosis of polyglandular autoimmune syndrome can be set. The recognition of symptoms and signs can be challenging and clinicians should be aware of atypical presentations, since they can be fatal if not correctly identified.

Case report: A 24-year-old man was admitted to a coronary intensive care unit due to acute pericarditis complicated with tamponade and shock requiring aminergic support. After introduction of methylprednisolone 125 mg twice daily, the condition improved. The patient had history of septic shock 2 months before and episcleritis with 2 years of evolution. One month later, when the patient was on 8 mg of methylprednisolone, a new episode of pericarditis occurred, requiring a pericardial window. An exhaustive study regarding autoimmunity and serologic causes were performed, but no etiology was identified. The patient was discharged under methylprednisolone 32 mg in slow and gradual discontinuation scheme. After four months, when the patient was on methylprednisolone 8 mg, new episode of pericarditis, establishing the diagnosis of recurrent pericarditis. Because the patient had hyponatraemia (126 mmol/l) and hyperkalaemia (6.4 mmol/l), an endocrinological assessment was required. Due to suspected adrenal insufficiency, an analytical and hormonal study was requested, showing the following results: ACTH 172 pg/ml (9–52); cortisol<1 µg/dl (5–25); renin 5306 µU/ml (7–76) and anti-21 hydroxylase antibody 25.1 U/ml (<1). These findings established the diagnosis of autoimmune primary adrenal insufficiency. Moreover, additional autoimmunity assessments requested showed the following results: anti-transglutaminase IgA, anti-TPO, anti-thyroglobulin and anti-intrinsic factor antibodies negative; anti-parietal cell antibody strong positive. The study was compatible with polyglandular autoimmune syndrome. The patient started therapy with prednisolone and fludrocortisone, with clinical improvement without further pericarditis’ episodes.

Conclusion: Pericarditis with tamponade was the first manifestation of Addison Disease in this patient. This association corresponds to a rare case, with few cases described in the literature. The early identification of this condition can be life-saving. This patient, after 2 years of follow-up under treatment directed to Addison Disease, did not relapse pericarditis and shock. The presence of unexplained hyponatremia and hyperkalaemia should raise the suspicious of Addison Disease, even in the existence of atypical presentations.