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Case Report
Familial Giant Cell Arteritis and Polymyalgia Rheumatica: Aggregation in 2 Families
PIERANNA FIETTA, PAOLO MANGANELLI, ADELE ZANETTI, and TAURO MARIA NERI
ABSTRACT.
The ethnic and geographic prevalence, the familial aggregation, and the reported association with some HLA class II antigens of both giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) strongly suggest the role of genetic factors in the pathogenesis of these diseases. We describe the familial aggregation of GCA and PMR in 2 unrelated families from Northern Italy. In the first family, 2 sisters developed GCA a few months apart. In the second, one sister had GCA, and 2 years later her siblings developed PMR nearly simultaneously. Patients with GCA in the first family shared the whole HLA genotype (A*24.*26, B*38.*55, DRB1*11.*14, DQB1*05.*07, DRB3*). In the second family, both PMR siblings carried the A*68, B*44, DRB1*11, DQB1*07, DRB3* alleles. Thus all patients of both families shared DRB1*11, DQB1*07, and DRB3*. Predisposing immunogenetic factors of both GCA and PMR are discussed. (J Rheumatol 2002;29:1551-5)
Key Indexing Terms:
GIANT CELL ARTERITIS
POLYMYALGIA RHEUMATICA
FAMILIAL AGGREGATION
HLA TYPING
From the Dipartimento Osteo-Articolare, and Cattedra e U.O.C. di Genetica Medica, Dipartimento di Clinica Medica, Nefrologia e Scienze della Prevenzione, Università degli Studi di Parma, Parma, Italy.
P. Fietta, MD; P. Manganelli, MD, Unità Operativa di Reumatologia e Medicina Interna, Azienda Ospedaliera di Parma; A. Zanetti, MD; T.M. Neri, MD, Dipartimento di Clinica Medica, Nefrologia e Scienze della Prevenzione, Università degli Studi di Parma.
Address reprint requests to Dr. P. Manganelli, Dipartimento Osteo-Articolare, Unità Operativa di Reumatologia e Medicina Interna, Azienda Ospedaliera di Parma, Via Gramsci 14, 43100 Parma, Italy. E-mail: pmanganelli@ao.pr.it
Submitted October 4, 2001; revision accepted January 24, 2002.
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