Abstract | OBJECTIVE: Autoimmune destruction of the adrenal gland is the major cause of Idiopathic Addison's disease, but the significance of 21-hydroxylase autoantibodies and their correlation with the presence of other autoantibodies have not so far been investigated in a larger population of patients with Addison's disease. We have now characterized a cohort of patients with idiopathic Addison's disease (n = 97) regarding the specificity of autoantibodies against the adrenal cortex and, as Addison's disease can be either an isolated condition or part of a polyendocrine disorder, we investigated the presence of organ-specific polyendocrine autoimmunity in this patient population. DESIGN: Cross-sectional study. MEASUREMENTS:
Autoantibodies were analysed with indirect immunofluorescence (IF) on tissue preparations, ELISA and in Western blots using bacterially expressed proteins. RESULTS: Eighty-four per cent (81/97) of the patient sera recognized the steroid-producing cells of the adrenal cortex in indirect IF. The antigen was identified as 21-hydroxylase by 72% (70/97) of the patient sera in Western blots. Seven sera that were negative on adrenocortical IF identified 21-hydroxylase on Western blot, while eight IF-positive sera were 21-hydroxylase-negative. Five sera weakly recognized 17 alpha-hydroxylase in Western blots, but all of these were also positive for 21-hydroxylase. In 13 cases (12 women), the sera also reacted with testicular Leydig cells, and nine of these identified the side-chain cleavage (SCC) enzyme. Other clinically evident organ-specific autoimmune disorders were present in 40% of the 97 patients and abnormal titres of organ-specific antibodies were found in 60% of the patients. CONCLUSIONS:
|
Authors | A Söderbergh, O Winqvist, I Norheim, F Rorsman, E S Husebye, O Dolva, F A Karlsson, O Kämpe |
Journal | Clinical endocrinology
(Clin Endocrinol (Oxf))
Vol. 45
Issue 4
Pg. 453-60
(Oct 1996)
ISSN: 0300-0664 [Print] England |
PMID | 8959085
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
|
Chemical References |
|
Topics |
- Addison Disease
(immunology)
- Adrenal Cortex
(immunology)
- Adult
- Autoantibodies
(blood)
- Autoimmunity
- Blotting, Western
- Cohort Studies
- Cross-Sectional Studies
- Diabetes Mellitus, Type 1
(immunology)
- Enzyme-Linked Immunosorbent Assay
- Female
- Fluorescent Antibody Technique, Indirect
- Humans
- Islets of Langerhans
(immunology)
- Leydig Cells
(immunology)
- Liver
(immunology)
- Male
- Middle Aged
- Ovary
(immunology)
- Parathyroid Glands
(immunology)
- Parietal Cells, Gastric
(immunology)
- Polyendocrinopathies, Autoimmune
(immunology)
- Thyroid Gland
(immunology)
|