Atypical and severe presentation of autoimmune polyglandular syndrome type 2

Abstract

Background: Recurrent pericarditis has been described as an unusual manifestation of autoimmune polyglandular syndrome type 2 (APS 2). Case report: We describe a case of a 44-year-old woman who was admitted to hospital due to 5 pericarditis, 3 of them with cardiac tamponade, and in the etiological study of this pathology she was diagnosed with an APS 2. Conclusion: The association of serositis with APS 2 is exceptional with less than 10 cases reported in the literature. The presence of recurrent pericarditis of unknown cause should make us consider APS 2 in the differential diagnosis.