Abstract

BOTTA, Silvia; ROVETO, Silvana  and  RIMOLDI, Daniel. Seric 21- hydroxilase antibodies in patients with anti-microsomal fraction antibodies: Autoimmune polyendocrine syndrome. Medicina (B. Aires) [online]. 2007, vol.67, n.2, pp.143-146. ISSN 0025-7680.

Autoimmune polyendocrine syndrome (APS) is the association of  autoimmune endocrine diseases, with other autoimmune nonendocrine disorders. APS types 1, 2 and 4  include autoimmune adrenalitis; this suggests  the presence of  autoantibodies. A specific serological marker for these is the anti 21- hydroxilase autoantibody (a21-OH). APS type 2 is the association of autoimmune adrenalitis, to autoimmune thyroid disease and/or diabetes mellitus, all these are induced by autoantibodies. Alopecia, vitiligo, myasthenia and other manifestations can be minor components. We sought to establish the prevalence of seric a21-OH in patients with positive anti-microsomal fraction autoantibodies, autoimmune thyroid disease and/or non-endocrine autoimmune diseases. We also aimed to diagnose incomplete forms of APS and to  follow up  patients at risk of progression  to complete forms of APS. A population of 72 patients and another of 60 controls with negative anti-microsomal fraction autoantibodies were studied. Elevated seric a21-OH were found in two patients. Patient A with 47 U/ml had autoimmune hypothyroidism and myasthenia; and patient B with 8.75 U/ml had autoimmune hypothyrodism and vitiligo; they both  lacked adrenal insufficiency. Seric a21-OH had a prevalence of 2.8%. Regarding the adrenal component, patients A and B had an incomplete and latent APS type 2. Considering a21-OH as markers of latent endocrine autoimmune diseases and taking into account the eventual risk of developing clinical manifestations, periodic biochemical and clinical follow-ups are recommended.

Keywords : Autoantibodies; Autoimmune polyendocrine syndrome; Adrenal insufficiency.

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