To analyze the clinical features and outcome of juvenile-onset mixed connective tissue disease (MCTD).
Clinical and laboratory findings were compared in 2 groups of MCTD patients divided according to age at onset (juvenile onset : under 16 yrs ; adult onset : 16 yrs or older).
Systemic lupus erythematosus-like symptoms, such as facial erythema, photosensitivity, LE cells, lymphadenopathy, and cellular casts, were more frequent in juvenile onset MCTD than in the adult form of the disease.
On the other hand, scleroderma-like symptoms, such as esophageal hypomotility, scleroderma-like lesions evident on skin biopsy, pulmonary involvement, proximal scleroderma, and pitting scars, were less frequent in juvenile onset MCTD than in the adult form.
Patients with juvenile onset MCTD more frequently met the classification criteria for systemic lupus erythematosus (SLE) and less frequently met those for progressive systemic sclerosis (SSc), compared to patients with adult onset MCTD.
At disease onset, hand edema and stiffness were observed less frequently in juvenile onset MCTD than in the adult form.
Furthermore, the mortality rate was lower in the former than in the latter (2.8% vs 8.4%). Conclusion. (...)
Mots-clés Pascal : Tissu conjonctif maladie mixte, Maladie autoimmune, Age apparition, Homme, Symptomatologie, Pronostic, Epidémiologie, Echelon national, Japon, Asie, Peau pathologie, Appareil circulatoire pathologie, Appareil respiratoire pathologie, Système ostéoarticulaire pathologie, Muscle strié pathologie, Maladie système, Immunopathologie
Mots-clés Pascal anglais : Sharp syndrome, Autoimmune disease, Age of onset, Human, Symptomatology, Prognosis, Epidemiology, National scope, Japan, Asia, Skin disease, Cardiovascular disease, Respiratory disease, Diseases of the osteoarticular system, Striated muscle disease, Systemic disease, Immunopathology
Notice produite par :
Inist-CNRS - Institut de l'Information Scientifique et Technique
Cote : 96-0303716
Code Inist : 002B07. Création : 10/04/1997.