A family study in Still's disease.

Familial studies in inflammatory polyarthritis indicate strong hereditary factors, particularly in relationship to the serological tests (Lawrence and Ball, 1958). In a single kindred of some 3,000 persons reported briefly by Whittinghill, Hendricks, Taylor, and Thorp (1958), rheumatoid arthritis was distributed independently of spondylitis and Still's disease. The ten spondylitics had higher than random frequencies of other cases of spondylitis and of Still's disease among their first-degree relatives, suggesting a genetic origin for this dual susceptibility. The five cases of Still's disease had, as their close relatives, spondylitics rather than persons with other forms of juvenile or adult rheumatoid arthritis. Precise details of the criteria used for the diagnosis of Still's disease in this study are not available, and the evaluation of this relationship is complicated by the fact that a transient attack of polyarthritis precedes the onset of spondylitis in about 25 per cent. of patients, often some years before the spondylitic phase (Hollister and Engleman, 1958). Conflicting results have been obtained with serological tests in Still's disease. We have found 45 per cent. of such patients to have a positive Rose-Waaler test on at least one occasion during a 4to 10-year follow-up, although only 13 per cent. were positive on the first test (Bywaters, Carter, and Scott, 1959a). McEwen, Ziff, Carmel, Ditata, and Tanner (1958), using the euglobulin fraction for the S.C.A.T., found 58 per cent. positive, but with their inhibition test 94 per cent. were positive. With a view to elucidating this problem further, a group of patients with Still's disease admitted to the Canadian Red Cross Hospital, Taplow, have been used as probands for a family study.