Article

Autoimmunity in isolated Addison's disease and in polyglandular autoimmune diseases type 1, 2 and 4.

Clinical Immunology and Allergy, Department of Medical and Surgical Sciences, University of Padova, Via Ospedale Civile 105, 35128 Padova, Italy.
Annales d Endocrinologie (impact factor: 0.74). 05/2001; 62(2):193-201. pp.193-201
Source: PubMed

ABSTRACT Sera from 300 Italian patients with Addison's disease were collected over a 30 year period. Among these patients, 82% had autoimmune disease, 13% had tuberculosis and 5% had another causal condition. In 59% of the cases, autoimmune disease was associated with the autoimmune manifestations contributing to the description of polyglandular autoimmune disease (PGAD). In PGAD type 1, the disease was associated with chronic candidiasis and/or chronic hypoparathyroidism. In PGAD type 2, the patients had autoimmune thyroid disease and/or diabetes mellitus type 1, and in PGAD type 4, they presented a combination with other autoimmune diseases excluding those previously mentioned. Finally, the autoimmune disease was apparently isolated in 41% of the cases. In addition, patients with these four forms of disease exhibited a different genetic pattern, sex distribution, and age at presentation in addition to minor frequency of autoimmune diseases. Adrenal cortex autoantibodies directed against 21-hydroxylase were common serological markers for these four main clinical forms, showing a very high frequency at clinical onset of adrenal insufficiency. In some patients, steroid-producing cell autoantibodies were also present and correlated with gonadal failure and they recognize of 17alpha-hydroxylase or P450 side chain cleavage enzymes as target antigens.

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Keywords

30 year period
 
300 Italian patients
 
Addison's disease
 
Adrenal cortex autoantibodies
 
autoimmune disease
 
autoimmune manifestations
 
chronic candidiasis
 
clinical onset
 
diabetes mellitus type 1
 
different genetic pattern
 
four forms
 
four main clinical forms
 
gonadal failure
 
minor frequency
 
PGAD type 1
 
PGAD type 2
 
PGAD type 4
 
polyglandular autoimmune disease
 
steroid-producing cell autoantibodies
 
target antigens