Background. Autoimmune polyendocrine syndrome (APS) consists of multiple endocrine glands autoimmune disorders. In APS IIIA autoimmune thyroiditis occurs with immune-mediated diabetes without involving adrenal cortex. Although APS is an underdiagnosed disorder therefore more common than previously reported, APS with recurrent serositis is undoubtedly rare condition.
Material and methods. We presented a case of 16 year old girl with autoimmune thyroiditis, type I diabetes, recurrent serositis and 21st chromosome trisomy. Two years ago first episode of pericarditis was successfully treated with NSAID, diuretics and steroids. Second episode of pericarditis was unresponsive to pharmacological treatment therefore pericardial punction was performed. Third episode was remarkably difficult to treat as the patient presented with acute respiratory failure due to combined pericarditis and massive unilateral pleuritis. Pleural biopsy showed chronic non-specific fibrous pleuritis. Only one month and two surgeries later patient could return to her everyday life.
Conclusion. The patient was diagnosed with an undoubtedly rare APS with polyserositis and was sucessfully treated. Unfortunately there are no diagnostic tests for APS therefore diagnosis can only be suspected as a result of collection of clinical signs. Despite various attempts to create a definitive genetic test for APS typeIII, none were successful enough to proceed with daily clinical application so far. Constellation of several uncommon conditions may be indicative of rare underlying syndrome. One should always stay vigilant because up to a quarter of patients with some level of hypofunction of certain endocrine gland may have additional endocrinopathies.
