Prognosis in juvenile chronic arthritis. Follow-up of 433 chronic rheumatic children.

A total of 433 children, hospitalized in the Rheumatic Children's Hospital Garmisch-Partenkirchen, were followed on average for 15 years (range 10-22 years) after the onset of definite juvenile chronic arthritis. This paper reveals clearly that the different subgroups of chronic rheumatic disease in childhood with their different complications have a definite relationship to prognosis. Severe limitation with inability to attend normal school or employment occurred in 13.4% of children with systemic polyarticular arthritis (Still's syndrome) and 11.4% of the non-systemic cases, but not in the pauci-articular group. In the latter group 82.5% of the children remained without disability or with only slight impairment: this is significantly better than in the systemic or non-systemic polyarticular groups. Although the high incidence of chronic rheumatic iridocyclitis is common in children with pauci-articular arthritis, none of this group had been handicapped by severe eye complications or blindness, in contrast to several early cases with systemic or non-systemic polyarthritis. This might be due to regular eye checks in the pauci-articular group. Of the children in the systemic polyarticular group 10% were dwarfed. Mortality in the systemic group was 13.8%, in contrast to 1% in the non-systemic polyarticular and 0% in the pauci-articular arthritis group. Secondary amyloidosis was the most important cause of death, mainly in systemic cases. Of the children whose amyloidosis had been verified, 44% died in their second or third decades, mostly with uraemia.

Hypohistidinemia in juvenile rheumatoid arthritis.

The mean level of plasma histidine in 86 children with rheumatoid arthritis was found to be significantly lower in comparison with that of controls. The possible influence of various drugs on the plasma histidine concentration is discussed.

Corticosteroid treatment of juvenile chronic polyarthritis over 22 years.

Between the years 1952 and 1974, 1293 children aged from 1-14 years with the adult type of rheumatoid arthritis and Still's disease were admitted, of whom 793 or 61.2% received oral corticosteroids, mostly in the form of continuous therapy. In all children with Still's syndrome (476 cases, of whom 84.4% received corticosteroid therapy), as well as in 75% of the children with rheumatoid arthritis (817 cases, 47.1% under corticosteroid treatment) the therapy had already been initiated by the referring institutions. The mortality and morbidity of corticosteroid treated children is compared with the results from the precortisosteroid era. The influence upon rheumatic activity as well as on joint pathology was compared in groups of patients with rheumatoid arthritis with and without steroid therapy. Corticosteroid therapy led to a significant reduction in the early mortality from myo- and pericarditis in Still's syndrome. On the other hand, the late mortality of 5% was encumbered with a high incidence of direct sequelae of longterm corticosteroid therapy and secondary diseases, especially amyloidosis. Furthermore, more than half of the children on longterm treatment had severe irreversible, and partially fatal lesions. The results of the present investigation strongly suggest that the indications for and the manner of using corticosteroids in treating rheumatoid arthritis and Still's syndrome ought to be revised.