(1) To review the diagnoses after 10 years in patients who were identified within 12 months of the onset of well established and undifferentiated connective tissue diseases (CTD). (2) To examine the death rates and disease remissions in these patients.
This inception cohort of 410 patients had less than one year of signs and/or symptoms of CTD.
Diagnoses of rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and poly/dermatomyositis (PM/DM) were made in 197 patients using accepted diagnostic and classification criteria.
Diagnoses of undifferentiated CTD were made in 213 patients.
These latter patients were placed in 3 categories : isolated Raynaud's phenomenon (RP), unexplained polyarthritis (UPA), and undifferentiated CTD (UCTD), defined as meeting at least 3 of 11 specific manifestations of CTD.
The diagnoses and remissions in all patients after 10 years were determined.
Patients with well established CTD tended to remain with the original diagnosis.
The 10 year survival was at least 87% in all diagnostic categories, with the exception of SSc, in which it was 56%. The progression of UPA to RA occurred infrequently.
The presence of antinuclear antibodies suggested that UPA may develop additional symptoms and/or a specific diagnosis, and RP in these patients increased the likelihood of progressing to UCTD or a specific well established CTD.
Ten percent of patients with RP progressed to SSc. (...)
Mots-clés Pascal : Tissu conjonctif pathologie, Atypique, Evolutivité, Epidémiologie, Diagnostic, Mortalité, Rémission, Etude longitudinale, Long terme, Diagnostic erreur, Homme, Peau pathologie, Système ostéoarticulaire pathologie, Maladie autoimmune, Maladie système, Immunopathologie, Muscle strié pathologie
Mots-clés Pascal anglais : Connective tissue disease, Atypical, Evolutivity, Epidemiology, Diagnosis, Mortality, Remission, Follow up study, Long term, Error diagnostic, Human, Skin disease, Diseases of the osteoarticular system, Autoimmune disease, Systemic disease, Immunopathology, Striated muscle disease
Notice produite par :
Inist-CNRS - Institut de l'Information Scientifique et Technique
Cote : 99-0264003
Code Inist : 002B07. Création : 16/11/1999.