X-linked Agammaglobulinemia Presenting as Juvenile Chronic Arthritis: Report of One Case

以幼年型慢性關節炎為臨床表現之性聯丙種免疫球蛋白缺乏血症：一病例報告

傅仁良(Jen-Liang Fu) ； 徐世達(Shyh-Dar Shyur) ； 林鴻瀛(Horng-Ying Lin) ； 賴永清(Yung-Ching Lai)

《Acta Paediatrica Taiwanica》 40卷4期 (1999/08) Pp. 280-283

https://doi.org/10.7097/APT.199908.0280

摘要

Bruton氏病（性聯丙種免疫球蛋白缺乏血症）是一種性染色體隱性遺傳疾病，只侵兒犯男性。20%的Bruton氏病患者會合并有關節炎。有的關節炎是屬於敗血性關節炎，但有的則表現的像類風濕性關節炎或幼年型慢性關節炎。本文報告一例罹患Bruton氏病的男孩同時也得到右側膝關節的慢性關節炎。其關節炎符合幼年型慢性關節炎的診斷。其關節炎在定期給與靜脈注射免疫球蛋白及口服naproxen之治療之後很快改善。因此，吾人建議，看到一位幼年型慢性關節炎的男性患者時，必須想到Bruton氏病可能為其潛在的原因之一。

關鍵字

性聯丙種免疫球蛋白缺乏血症 ； 幼年型慢性關節炎

並列摘要

Bruton agammaglobulinemia (X-linked agammaglobulinemia, XLA), transmitted by X-linked recessive inheritance, affects only males. Twenty percent of patients with XLA may have arthritis. Septic arthritis may occur, but there is also a form of arthritis that is similar to rheumatoid arthritis or juvenile chronic arthritis. Here we report one case of XLA in a boy with non-erosive chronic right knee arthritis. There was no evidence of septic arthritis. Regular intravenous gammaglobulin replacement therapy and oral naproxen resulted in dramatic improvement in the arthritis. This case illustrates that XLA should be considered as a possible underlying cause of juvenile chronic arthritis in males.

並列關鍵字

X-linked agammaglobulinemia ； juvenile chronic arthritis