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Corticosteroid treatment of juvenile chronic polyarthritis over 22 years

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Summary

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.

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Nomenclature. Rheumatoid arthritis or chronic progredient polyarthritis “sensu strictiore” in children is similar to the adult, seronegative type of the disease. Oligo- and monarthritis are more often combined with iridocyclitis than in adult rheumatoid arthritis.

Complete Still's disease—acute onset with high, sometimes septic fever at the beginning and/or during the course of the disease. Extraarticular manifestations are predominant such as myopericarditis, rheumatic rash, hepato- and splenomegaly, lymphadenitis, leucocytosis, severe anaemia and rheumatic nodules.

Incomplete Still's disease—the initial acute and febrile course of the disease is present but less severe and systematic extraarticular, visceral manifestations are partly present.

The term “complete or incomplete Still's syndrome” should express the possibly near, but not warranted unity of the above classified three manifestations of the rheumatic disease in childhood. The term juvenile rheumatoid arthritis or juvenile chronic polyarthritis includes all three variations of the disease in childhood without the necessary differentiation, and is therefore not exact enough when comparing statistics, since the prognosis and therapy of the three types are different.

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Stoeber, E. Corticosteroid treatment of juvenile chronic polyarthritis over 22 years. Eur J Pediatr 121, 141–147 (1976). https://doi.org/10.1007/BF00443068

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