Fifteen-year experience of pediatric-onset mixed connective tissue disease.
The aim of this study was to investigate the initial clinical manifestations, laboratory data, complications, and outcomes of patients with pediatric-onset mixed connective tissue disease (MCTD) in Taiwan. We reviewed medical charts of patients younger than 18 years with a diagnosis of mixed connective tissue disease based on the criteria of Kasukawa (1) at the pediatric department of National Taiwan University Hospital from 1993 to 2008. A total of 12 patients were included. All of the patients were female. The mean age at disease onset was 10.7 years (range 6.5 to 14 years). The most common symptoms at disease onset were polyarthritis (7/12 patients) and Raynaud's phenomenon (7/12 patients). The clinical symptoms changed with time, and other symptoms encompassing the criteria for MCTD developed sequentially. Inflammatory manifestations (arthritis, fever, and skin rash) improved following treatment, whereas sclerodermatous features (sclerodactyly, esophageal disease, and vasculopathy) persisted and were often unresponsive to therapy. The organ involvement-free rates at 2 years, 5 years, and 10 years were 91.7%, 78.6%, and 52.4%, respectively. In this retrospective study, sclerodermatous changes of internal organs were a poor prognostic factor in our population, and we emphasize that long-term follow-up is necessary, and appropriate treatment should be applied to improve the outcomes.
Tsai
Yi-Ying
YY
Department of Pediatrics, National Taiwan University Hospital, 7 Chung-Shan South Road, Taipei, 10002, Taiwan, Republic of China.
Yang
Yao-Hsu
YH
Yu
Hsin-Hui
HH
Wang
Li-Chieh
LC
Lee
Jyh-Hong
JH
Chiang
Bor-Luen
BL
D000293:Adolescent; D017668:Age of Onset; D001168:Arthritis / Q000150:complications* / Q000473:pathology; D002648:Child; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008947:Mixed Connective Tissue Disease / Q000150:complications / Q000473:pathology*; D011379:Prognosis; D011928:Raynaud Disease / Q000150:complications* / Q000473:pathology; D012189:Retrospective Studies; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000150:complications* / Q000473:pathology; D012720:Severity of Illness Index; D013624:Taiwan