Case report
Hepatitis and the polyglandular autoimmune syndrome, type 1
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Submission date: 2010-06-17
Final revision date: 2010-09-11
Acceptance date: 2010-10-14
Online publication date: 2011-07-11
Publication date: 2011-07-11
Arch Med Sci 2011;7(3):536–539
Autoimmune polyglandular syndrome, type 1 (APS-1) is a rare syndrome. Here we present a case report of a 24-year-old female patient who complained of progressive weakness. While autoimmune hepatitis was diagnosed, no improvement of biochemical parameters was obtained after immunosuppressive treatment. Hypoparathyroidism and adrenocortical failure were identified. Her health status clearly improved once proper control of the calcium-phosphate metabolism was obtained and after the administration of substitution hydrocortisone doses, leading to full normalization of biochemical liver tests. The reported case illustrates a rare form of APS-1 failure, in which the diagnosed autoimmune hepatitis was only the first symptom.