ADULT-ONSET STILLS DISEASE - CLINICAL AND LABORATORY FEATURES, TREATMENT AND PROGRESS OF 45 CASES

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  • 1 November 1986
    • journal article
    • research article
    • Vol. 61  (235) , 1055-1065
Abstract
The clinical and laboratory features, treatment, prognosis, complications and disability of 45 patients with adult-onset Still''s disease were studied. Sixty per cent of the patients were female. Median age at onset was 25 years and median observation period after onset was 41 months. For 28 patients detailed data were available of the first month of illness. In only 43 per cent of these did the disease present with the classical triad of fever, arthritis and rash, although in the whole group eventually, fever with temperatures of 40.degree. C or more occurred in 84 per cent, arthritis in 98 per cent, and the typical rash in 82 per cent. In 32 per cent of the patients with rash, this was pruritic. Other features seen frequently were: lymphadenopathy (71 per cent), splenomegaly (36 per cent), pleuritis and/or pneumonitis (31 per cent), pericarditis (22 per cent), leucocytosis (98 per cent) and hepatic abnormalities (84 per cent). The high spiking fever was reduced to normal in six of 21 cases (29 per cent) by aspirin, in 19 of 27 cases (70 per cent) by indomethacin and in six of seven cases (86 per cent) by naproxen. In 16 of 21 cases (76 per cent) glucocorticoids reduced the systemic and/or or joint symptoms. In three patients who remained febrile on glucocorticoid, indomethacin reduced temperature to normal. Eight patients whose joint disease improved on steroids later developed severe joint destructions. Thirteen patients received one or several slow-acting antirheumatic drugs. Only in eight of 18 trials (44 per cent) with one of these drugs did symptoms and signs improve. Fifty-one per cent of the 45 patients had self-limiting disease and 49 per cent had persistent disease with continuous activity for at least one year. At the time of evaluation 47 per cent of the 45 patients were in remission without medication, 33 per cent were in remission while on medication and 20 per cent had active disease. Three life-threatening complications occurred: two patients developed signs of cardiac tamponade and one almost died from diffuse intravascular coagulation. Disability was determined primarily by the course of the arthritis. At the time of review 43 per cent of the 45 patients had no joint destruction (Group 1), 24 per cent had destruction of at least one joint, but had no evidence of disease of the root joints (Group 2) and 33 per cent had destruction in at least one root joint (Group 3). Patients in Group 3 generally suffered from a more widespread polyarthritis than patients in Group 2. Sixty-seven per cent of the Group 3 patients required hip or knee arthroplasty, and only 13 per cent of the 15 Group 3 patients were in ARA functional class IV. This study shows that the course of the arthritis in adult-onset Still''s disease varies markedly, and that severe widespread polyarthritis is common.