Juvenile-onset mixed connective tissue disease: longitudinal follow-up.
To establish the symptoms and clinical course of juvenile-onset mixed connective tissue disease, we studied 14 patients, classified according the criteria of Kasukawa et al. The patient records were studied retrospectively and all patients were examined in a 1-day follow-up program. Systemic lupus erythematosus and polymyositis/dermatomyositis-like symptoms disappeared in time, whereas scleroderma-like symptoms (such as in the Raynaud phenomenon) and joint abnormalities persisted. Extensive limitation of joint function was found in four patients. At the time of follow-up, no active renal disease was found. Thrombocytopenia was still present in one of the three patients who had had this feature. All patients had abnormalities of esophageal motility. Long-term corticosteroid treatment was associated with aseptic bone necrosis in three patients and growth retardation in one. We conclude that the Kasukawa criteria are easy to apply to children, and that juvenile-onset mixed connective tissue disease has many similarities to the adult form of the disease.
Tiddens
H A
HA
Department of Immunology, University Hospital for Children and Youth Het Wilhelmina Kinderziekenhuis, Utrecht, The Netherlands.
van der Net
J J
JJ
de Graeff-Meeder
E R
ER
Fiselier
T J
TJ
de Rooij
D J
DJ
van Luijk
W H
WH
Herzberger
R
R
van Suijlekom
L W
LW
van Venrooij
W J
WJ
Zegers
B J
BJ
D000293:Adolescent; D000974:Antibodies, Antinuclear / Q000032:analysis; D001168:Arthritis / Q000503:physiopathology; D001324:Autoantigens / Q000032:analysis; D002648:Child; D003430:Cross-Sectional Studies; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007596:Joints / Q000503:physiopathology; D008137:Longitudinal Studies; D008168:Lung / Q000503:physiopathology; D008297:Male; D008947:Mixed Connective Tissue Disease / Q000097:blood / Q000276:immunology / Q000503:physiopathology*; D009132:Muscles / Q000503:physiopathology; D020733:RNA, Small Cytoplasmic; D016059:Range of Motion, Articular / Q000502:physiology; D011928:Raynaud Disease / Q000503:physiopathology; D012189:Retrospective Studies; D012261:Ribonucleoproteins / Q000032:analysis / Q000276:immunology; D012594:Scleroderma, Localized / Q000503:physiopathology; D055517:snRNP Core Proteins; D000097585:SS-B Antigen; D000099207:SS-A Antigen
D000974:Antibodies, Antinuclear; D001324:Autoantigens; D020733:RNA, Small Cytoplasmic; D012261:Ribonucleoproteins; D055517:snRNP Core Proteins; D000097585:SS-B Antigen; D000099207:SS-A Antigen; C116006:RO60 protein, human