Familial Association of Giant Cell Arteritis

Although the etiology of the polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) syndromes remains unknown, there is increasing evidence suggesting an important pathogenetic role of immunogenetic factors. Evidence suggestive of humoral immune mediated mechanisms is demonstrated by the presence of large molecular weight circulating immune complexes, elevated levels of IgG, IgA, IgM, total CH50, and complements C3 and C4 in the sera of patients with active PMR, GCA, or both (PMR-GCA) and by the presence of immunoglobulin and complement deposits in temporal arteries (1-5). The characteristic inflammatory changes, including multinucleated histiocytic and foreign body giant cells, lymphocytes, histiocytes, and some plasma cells and fibroblasts, and an increased number of circulating immunoblasts in active PMR, support the participation of cell-mediated immune mechanisms (6). Familial aggregation and the apparent predilection for whites suggest a genetic predisposition to the development of ---

Increased frequency of hla‐dr3 and dr4 in polymyalgia rheumatica‐giant cell arteritis

Lowenstein

Bridgeford

Vasey

et al. 1983

The epidemiology of giant cell arteritis. Report of a Ten‐Year Study in Shelby County, Tennessee

Smith

Fidler

Pinals

1983

154

We have studied the epidemiologv of giant cell arteritis (GCA) m an urban population in south central United States. The incidence of GCA in Shelby County, Tennessee for the years 1971 through 1980 was determined for the total population and for age-, sex-, and race-specific groups. Over this period 26 cases were identified. The average annual incidence was 0.35/100,000. This was increased to 1.58/100,000 for those over the age of 50. The incidence was 7 times greater in whites than in blacks and 7 times greater in females than in males. Clinical and laboratory features of the patients were reviewed and found to be similar to those in other populations. GCA is considerably less frequent in Shelby County, Tennessee than in other areas where similar epidemiologic studies have been performed. This difference can be accounted for only in part by racial distributions. Similar studies in southern geographic areas are needed to place our findings in perspective and point to specific factors which may have etiologic significance in GCA.From the Departments of Medicine and Pathology, University of Tennessee Center for the Health Sciences (UTCHS), Memphis.

Hla‐dr4 in giant cell arteritis: association with polymyalgia rheumatica syndrome

Richardson

Gladman

Fam

et al. 1987

Forty-three patients with well-documented giant cell arteritis (GCA) were studied clinically and with HLA typing. All patients were over age 40. Twenty-two of the patients had coexistent polymyalgia rheumatica (PMR). No association with class I HLA antigens was detected. When compared with HLA findings in 243 healthy controls, HLA-DR4 was increased in patients with GCA and PMR, but not in patients with GCA alone. Pooled analysis of data from 4 published papers confirmed the association of DR4 with GCA and PMR (P <