Autoimmune polyglandular syndrome

Autoimmune polyglandular syndrome-Chron c autoimmune thyroiditis may occur in association with other autoimmune disorders. Treat patients with concomitant adrenal insufficiency with replacement glucocorticoids prior to initiation of treatment. Failure to do so may precipitate an acute adrenal crisis when thyroid hormone therapy is initiated. Patients with diabetes mellitus may require upward adjustments of their antidiabetic therapeutic regimens. Nontoxic diffuse goiter or nodular thyroid disease Use caution when administering levothyroxine to patients with nontoxic diffuse goiter or nodular thyroid disease in order to prevent precipitation of thyrotoxicosis. If the serum TSH is already suppressed, do not administer levothyroxine. 

Autoimmune polyglandular syndrome with progressive thyroid autoimmunity, type 1 diabetes mellitus, amenorrhea, and adrenal insufficiency has been reported in a 51-year-old woman treated with interferon alfa for chronic hepatitis C (545). Pancreas and pituitary gland autoantibodies, which were undetectable before interferon alfa treatment, were present at the time of diagnosis. After withdrawal, she recovered normal thyroid function, but was still insulin dependent with amenorrhea and adrenal insufficiency. 

Sasso FC, Carbonara O, Di Micco P, Coppola L, Torella R, Niglio A. A case of autoimmune polyglandular syndrome developed after interferon-alfa therapy. Br J Clin Pharmacol 2003 56 238-9. 

Autoimmunity (a) isolated autoimmune premature ovarian failure or (b) as a component of an autoimmune polyglandular syndrome in association with Addison s disease, hypothyroidism, hypoparathyroidism, or mucocutaneous candidiasis Iatrogenic (chemotherapy, radiation, extensive ovarian surgery) X-chromosome abnormalities... 

The first type of autoimmune polyglandular syndrome, also referred to as APECED, is characterized by the presence of three major component diseases chronic candidiasis, hypoparathyroidism, and autoimmune Addison disease. It has primarily been seen, to... 

The least well characterized autoimmune polyglandular syndrome is the type 3 syndrome. This syndrome is defined by the presence of autoimmune thyroid disease with another autoimmune disease, such as diabetes mellitus type l, autoimmune gastritis, or myasthenia gravis, but in the absence of Addison disease. APGS type 3 primarily involves females (the female to male ratio is 7 1) who have HLA-DR3-associated autoimmune disease and is probably the most common of the autoimmune polyglandular syndrome disorders. Since different and multiple clinical combinations can be found, the classification of this type of autoimmune polyglandular syndrome is probably more complicated than originally anticipated. 

Polyendocrinopathies, autoimmune. Autoimmune diseases affecting multiple endocrine organs, (i) The autoimmune polyglandular syndrome type 1 is characterized by mucocutaneous candidiasis in association with endocrine manifestation (also called APECED syndrome autoimmune polyendocrinopathy-candidiasis-ectodermal-dystrophy), while (ii) the autoimmune polyglandular... 

L. Prentice, M. Volpato et al. (1996). Autoantibodies to steroidogenic enzymes in autoimmune polyglandular syndrome, Addison s disease, and premature ovarian failure. J. Clin. Endocrinol. Metab. 81, 1871-1876. 

Another issue is autoantibodies to P450 llAl (and also P45017A1) inpatients with autoimmune polyglandular syndrome types I and II and Addison s disease [1969-1971]. As with other P450s recognized by antoantibodies, causal relationships between immnnity and disease are unclear. 

As with some other P450s, circulating antibodies to P450 17A1 are seen in some autoimmune diseases, e.g., autoimmune polyglandular syndrome and Addison s disease [1969, 2254], but no causal relationship has been demonstrated. 

Type I diabetes mellitus and thyroid disease reportedly develop in 0.08-2.6% and 10-15% of patients treated with combined interferon alfa- -ribavirin for chronic hepatitis C but rarely coexist however, both conditions have been reported in a 33-year-old woman [50 ]. In another case, a 55-year-old woman developed type 1 diabetes and had a recurrence of Graves disease during treatment with peginterferon alfa -I- ribavirin for chronic hepatitis C [51 ]. There were serum anti-glutamic acid decarboxylase antibodies and the authors suggested that she had autoimmune polyglandular syndrome type III. 

Compared with the autoimmune polyglandular and autoimmune lymphoproliferative syndromes described above, all other known autoimmune diseases and syndromes may not be inherited nonetheless, genes are responsible for differences in the susceptibility for disease development. Multiple genes, acting in concert with various environmental factors, seem to be involved in the autoimmune pathogenesis of most autoimmune diseases (see chapter 9). 

Polyglandular autoimmune syndrome type I (Addison s disease, candidiasis, hypoparathyroidism, and primary gonadal failure)... 

Endocrine Hypocalcemia alone may impair insulin release. In addition, idiopathic hypoparathyroidism may be associated with polyglandular autoimmune syndromes. 

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