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Autoimmune polyendocrine syndrome (APS) is an autoimmune disorder defined by multiple endocrinopathies and the presence of other systemic or organ-specific autoimmunities. This case study, reports on a 46-year-old woman with confirmed idiopathic hyperparathyroidism, who was referred for inflammatory back pain. HLA-B27 positivity, recurrent anterior uveitis and radiologic findings led to ankylosing spondylitis (AS) diagnosis. By further investigations, a subclinical atrophic autoimmune thyroid disease (AITD) was also diagnosed for her in addition to a history of premature ovarian failure. According to the absence of adrenal insufficiency in addition to an AITD and systemic autoimmune disorder, APS type IIIC was the most probable diagnosis. To the best of our knowledge, this is the first case report of APS type III associated with AS.