A Follow-up Study of Systemic-Onset Juvenile Rheumatoid Arthritis in Children

我們回溯研究近十年來會於本院住院之全身性幼年類風濕性關節炎産現童。共有十一位男孩和十位女孩。平均年齡爲11.6±4.2歲，於確定診斷前症狀持續5.5±1.7月。平均追蹤時間爲45.7±9.5月。起始之臨床與實驗室表徵皆與先前報告相似，唯吾人發現60%之病童血液抹片檢查發現於白血球中有毒性顆粒(toxic granules)。起始之全身性及關節症狀可由抗發炎藥物或類固醇而得緩解。然有九位(43%)病童有持續性關節發炎超過六個月，而需使用其他緩解藥物。此九位罹患慢性關節炎患者中，病程屬單一週期系統型(monocyclic systermic)者佔六位，而七位其起始侵犯關節數大於四個，其中五例，雖經各種緩解藥物積極治療關節之破壞仍持續進行，導致嚴重功能障礙。此五例追蹤期間有持續性貧血，高血小板及血清C-反應蛋白上升，而ferritin值似與關節炎破壞程度無關。其中一例轉化爲全身性紅斑性狼瘡，併敗血症死亡。

關鍵字

全身性幼年型類風濕性關節炎；史迪爾氏症；單一週期性；多週期性

並列摘要

We analyzed the clinical and laboratory features, treatment, and course of twenty-one children with systemic-onset juvenile rheumatoid arthritis (S-JRA) encountered at our institution over the past ten years. There were eleven boys and ten girls. The mean age at onset was 11.6±4.2 years. The mean duration of symptoms prior to diagnosis was 5.5±1.7 months, and the mean follow-up period was 45.7±9.5 months. The clinical and laboratory features at presentation were similar to previous reports, except that peripheral blood smear revealed toxic granulation of neutrophils in 60% of our patients. Although systemic manifestation could be readily controlled by non-steroidal anti-inflammatory drugs (NSAIDs) with or without additional steroids, nine patients suffered from chronic arthritis (duration ＞6 months) requiring disease-modifying anti-rheumatic drugs (DMARDs). Of the nine children with chronic arthritis, six (67±) had a monocyclic systemic course, and seven (78%) had polyarticular disease (five or more joints affected) at the disease onset. Five patients developed severe destructive polyarthritis, with persistent anemia, thrombocytosis, elevated serum C-reactive protein (CRP) levels, and marked functional limitation during follow-up. One of the five patients with severe arthritis developed systemic lupus erythromatosis after 8-year follow-up, and died of sepsis. Our study indicated significant morbidity in children with S-JRA in Taiwan.

並列關鍵字

systemic onset juvenile rheumatoid arthritis ； Still's disease ； monocyclic ； polycyclic

國際替代計量

A Follow-up Study of Systemic-Onset Juvenile Rheumatoid Arthritis in Children

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