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The Expression of Autoimmune Polyglandular Disease Type I Appears Associated With Several HLA-A Antigens But Not With HLA-DR^*

Children's Hospital, University of Helsinki (P.A., J.P.), and the Finnish Red Cross Blood Transfusion Service (S.K., M.-L.L, A.T.) Helsinki, Finland

Address all correspondence and requests for reprints to: Pekka Ahonen, M.D., Children's Hospital, SF-00290 Helsinki, Finland.

We studied HLA-A, -B, -C, and -DR antigens in 45 patients (from among 34 families), aged 10.2-60 yr, with polyglandular autoimmune disease type I (APG I) and in other family members. HLA-A28 was more frequent in the patients (25%) than in unaffected siblings (16%; P < 0.05) or in normal Finnish subjects (8.8%; P < 0.005, corrected P < 0.2). Compared with the normal subjects, HLA-A28 was more frequent in the patients with hypoparathyroidism (31%; P < 0.001, corrected P < 0.04), adrenocortical failure (27%; P < 0.01), insulindependent diabetes mellitus (IDDM; 66%; P < 0.01), keratopathy (53%; P < 0.001, corrected P < 0.04), and alopecia (40%; P < 0.001, corrected P < 0.04), but not in the patients with ovarian failure (9%; P = NS). HLA-A28 was more frequent in the patients with hypoparathyroidism (31%) than in APG I patients without it (13%; P < 0.005, corrected P < 0.2). It was also more frequent in the patients with IDDM (66%) than in those without it (21%; P < 0.05). HLA-A3 was more frequent in the patients with ovarian failure (82%) than in APG I patients with normal ovarian function (22%; P < 0.025) and in normal subjects (45.5%; P < 0.05). HLA-A9 was less frequent in the patients with ovarian failure (0%) than in those with normal ovarian function (55%; P < 0.005, corrected P < 0.2), and it was less frequent (P < 0.025) in the patients with adrenocortical failure than in those with normal adrenal function. No association was found with any single DR antigen, but of 4 DR-typed IDDM patients, 3 were DR3 or DR4 positive (P = NS). The occurrence of adrenocortical failure, but not hypoparathyroidism, was familial and associated with HLA haploidentity among sets of affected siblings.

^* This work was supported by the Foundation for Paediatric Research, the Paulo Foundation, and the Finnish Cultural Foundation, Helsinki, Finland, and was partly carried out during a Research Fellowship (P.A.) of the National Medical Research Council (Academy of Finland).

Received August 17, 1987.

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