Adrenal-Cortex Autoantibodies and Steroid-Producing Cells Autoantibodies in Patients with Addisons Disease: Comparison of Immunofluorescence and Immunoprecipitation Assays
C. Betterle,
M. Volpato,
B. Pedini,
S. Chen,
B. Rees Smith and
J. Furmaniak
Department of Clinical Immunology and Allergy, Istituto di
Semeiotica Medica (C.B., M.V., B.P.), University of Padova, Padova,
Italy; FIRS Laboratories (S.C., B.R-S., J.F.), RSR Ltd., Cardiff
CF4 5DU, United Kingdom; and Department of Medicine University of Wales
College of Medicine (B.R-S., J.F.), Cardiff GF4 4XN, United
Kingdom
Address all correspondence and requests for reprints to: Corrado Betterle, Istituto di Semeiotica Medica, University of Padova, via Ospedale Civile 105, Padova, Italy 35128. E-mail:
betterle{at}ux1.unipd.it
Autoimmune Addisons disease and premature ovarian failureare
characterized by the presence of organ-specific autoantibodies.The
main adrenal and gonadal autoantigens have been identifiedand cloned,
and the relationship between the autoantibodiesdetected by
immunofluorescence techniques and those detectedby the new assays
using recombinant autoantigens needed to beinvestigated. We studied
165 patients with Addisons disease:143 patients had different forms
of autoimmune Addisonsdisease (13 with idiopathic premature ovarian
failure) and 22had nonautoimmune Addisons disease. Adrenal-cortex
autoantibodiesand steroid-producing cell autoantibodies were measured
by theimmunofluorescence techniques. Autoantibodies to steroid
21-hydroxylase,17-hydroxylase, and P450 side chain cleavage enzyme
were measuredby immunoprecipitation assay using
35S-labeled recombinant proteins.
Adrenal-cortex autoantibodies and autoantibodies to 21-hydroxylasewere
found in 81% of the patients with autoimmune Addisonsdisease. None
of the patients with nonautoimmune Addisonsdisease had
adrenal-cortex autoantibodies or autoantibodiesto 21-hydroxylase. A
high association between these two markersin patients with different
forms of autoimmune Addisonsdisease and in those with short- or
long-standing disease wasfound. Steroid-producing cells autoantibodies
were found in26% of the patients with autoimmune Addisons disease,
andautoantibodies to 17-hydroxylase and/or P450 side chain cleavage
enzymein 36% of the patients. Steroid-producing cells autoantibodies
werefound in 11/13 (85%) of patients with idiopathic premature
ovarianfailure associated with autoimmune Addisons disease,and
autoantibodies to 17-hydroxylase and/or P450 side chaincleavage
were found 12/13 (92%) of patients; the only case negativefor all
these three markers suffered from Turners syndrome.
Provided that a high standard of immunofluorescence techniqueis
maintained, measurement of adrenal cortex autoantibodiesor
steroid-producing cells autoantibodies by either immunofluorescenceor
immunoprecipitation assay is essentially equivalent.
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