Clinical outcome of pediatric-onset collagen disease patients

DOI Web Site

Bibliographic Information

Other Title
  • 当科を受診した小児期発症膠原病患者の臨床経過
  • 症例報告 当科を受診した小児期発症膠原病患者の臨床経過
  • ショウレイ ホウコク トウ カ オ ジュシン シタ ショウニキ ハッショウコウゲンビョウ カンジャ ノ リンショウ ケイカ

Search this article

Abstract

Clinical features and outcome of 44 （5 men and 39 women） pediatric-onset collagen diseases were examined. Age at onset and age at final observation were 12.4±2.5 and 36.0±10.6 years old, respectively. Duration from onset to final observation was 11.9±8.9 years. Diagnoses（ there is some overlapping） were 26 of systemic lupus erythematosus（ SLE）, 8 of juvenile idiopathic arthritis, 6 of Sjögren’s syndrome, 5 of mixed connective tissue disease, 3 of polymyositis/dermatomyositis, 2 of Behçet disease, and 1 of systemic sclerosis. Most patients with SLE showed nonspecific manifestations compared with other diseases. SLE patients with facial erythema or proteinuria at disease onset tended to be diagnosed earlier than those with nonspecific symptoms and signs. Steroid pulse and immunosuppressant were frequently used in patients with SLE compared with other diseases. At final observation, 16 patients （37.7±11.1 years old） were visiting our hospital regularly, 17（ 32.5±8.5） were referred to other doctors, 6（ 42.5±10.0） were dead, and 5（ 35.4±9.6） were missing. All 6 fatal cases were SLE. Since pediatric-onset SLE patients are refractory and their outcome is poor, pediatricians and internists should cooperate to improve prognosis of SLE.

Journal

Keywords

Details 詳細情報について

Report a problem

Back to top