Juvenile-onset mixed connective tissue disease: longitudinal follow-up.
| Title: | Juvenile-onset mixed connective tissue disease: longitudinal follow-up. |
|---|---|
| Authors: | Tiddens HA; Department of Immunology, University Hospital for Children and Youth Het Wilhelmina Kinderziekenhuis, Utrecht, The Netherlands.; van der Net JJ; de Graeff-Meeder ER; Fiselier TJ; de Rooij DJ; van Luijk WH; Herzberger R; van Suijlekom LW; van Venrooij WJ; Zegers BJ; et. al. |
| Source: | The Journal of pediatrics [J Pediatr] 1993 Feb; Vol. 122 (2), pp. 191-7. |
| Publication Type: | Journal Article |
| Language: | English |
| Journal Info: | Publisher: Mosby Country of Publication: United States NLM ID: 0375410 Publication Model: Print Cited Medium: Print ISSN: 0022-3476 (Print) Linking ISSN: 00223476 NLM ISO Abbreviation: J Pediatr Subsets: MEDLINE |
| Imprint Name(s): | Original Publication: St. Louis, MO : Mosby |
| MeSH Terms: | RNA, Small Cytoplasmic*; Mixed Connective Tissue Disease/*physiopathology; Antibodies, Antinuclear/analysis ; Arthritis/physiopathology ; Autoantigens/analysis ; Joints/physiopathology ; Lung/physiopathology ; Mixed Connective Tissue Disease/blood ; Mixed Connective Tissue Disease/immunology ; Muscles/physiopathology ; Range of Motion, Articular/physiology ; Raynaud Disease/physiopathology ; Ribonucleoproteins/analysis ; Ribonucleoproteins/immunology ; Scleroderma, Localized/physiopathology ; Adolescent ; Child ; Cross-Sectional Studies ; Female ; Follow-Up Studies ; Humans ; Longitudinal Studies ; Male ; Retrospective Studies ; snRNP Core Proteins ; SS-B Antigen ; SS-A Antigen |
| Abstract: | 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. |
| Substance Nomenclature: | 0 (Antibodies, Antinuclear); 0 (Autoantigens); 0 (RNA, Small Cytoplasmic); 0 (Ribonucleoproteins); 0 (snRNP Core Proteins); 0 (SS-B Antigen); 0 (SS-A Antigen); 0 (RO60 protein, human) |
| Entry Date(s): | Date Created: 19930201 Date Completed: 19930305 Latest Revision: 20260128 |
| Update Code: | 20260130 |
| DOI: | 10.1016/s0022-3476(06)80112-5 |
| PMID: | 8429431 |
| Database: | MEDLINE |
Journal Article