Antibodies to adrenal steroidogenesis enzymes and side chain cleavage enzyme in chronic adrenal insufficiency

The significance of circulating antibodies to adrenal steroidogenesis enzymes P450c21, P450cl7, and P450scc in the etiological diagnosis of primary chronic adrenal insufficiency (1-CAI) and autoimmune polyglandular syndromes (APS) was evaluated by immunoblotting with recombinant homologous enzymes. Sera from 54 patients were tested: from 6 patients with tuberculous 1-CAI, 23 with isolated autoimmune 1-CAI, 15 with 1-CAI within the framework of type 2 APS (APS-1), 6 with 1-CAI within the framework of type 1 APS (APS-1, candido-polyendocrine syndrome), 3 with A PS-2 without 1-CAI, and from 1 patient with autoimmune oophoritis. The above antibodies were not detected in patients with tuberculous 1-CAI and with APS-2 without 1-CAI, or in the patient with autoimmune oophoritis. Antibodies to P450c21 were detected in 17 out of 23 (74%)) patients with isolated autoimmune 1-CAI, 12 out of 15 (80%o) patients with APS-2, and in 5 out of 6 (83%) patients with APS-1. No antibodies to P450cl7 were detected in patients with isolated 1-CAI; these antibodies were detected in only 1 out of 15 (6.6%) patient with APS-2 and in 4 out of 6 (67%)) patients with APS-2. Antibodies to P450scc were detected in 4 out of 23 (17.4%o) patients with isolated 1-CAI, 1 patient with APS-2, and in 4 out of 6 (67%o) patients with APS-1. Analysis of the results for groups with autoimmune 1-CAI and APS-2 (38 patients) showed that in the subgroup with 1-CAI lasting less than 15 years antibodies to P450c21 were detected in 86.2+6.4% cases, while in patients with 1-CAI duration of 15years or longer they were detected in 44.4±16.56%o cases (t=2.35; p<0.05). Hence, testing of antibodies to P450c21 is a valuable instrument for the etiological diagnosis of 1-CAI, specifically, for the differential diagnosis of autoimmune and tuberculous 1-CAI.

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