title: Follow-up of 151 patients with high-titer U1RNP antibodies.
abstract: We performed a longitudinal follow-up study of clinical findings in 151 patients with high-titer antibodies against U1 ribonucleoprotein (U1RNP) as measured by haemagglutination. Formal connective tissue disease (CTD) diagnoses were assigned and diagnostic transitions analysed. One-hundred eighteen females and 33 males entered the study; the mean duration of follow-up was 7.1 years. Mean age at entry was 34.7 years; 73% of the patients had early disease (duration < 2 years). Fifty-six patients (37%) presented with a definite diagnosis, most often mixed connective tissue disease (MCTD, n = 40), followed by systemic lupus erythematosus (SLE, n = 11) and systemic sclerosis (SSc, n = 5). Of 84 patients (56%) presenting with nonspecific symptoms of possible, "undifferentiated" CTD, 58 developed MCTD, 4 SSc and 2 SLE. By the end of the follow-up period. 127 patients had developed a well-defined CTD; final diagnoses were: MCTD (n = 97), SLE (n = 18), SSc (n = 12). We conclude that CTD in the context of high-titer anti-U1RNP antibodies may be transitive and sequential in nature, although the diagnostic criteria for MCTD previously proposed by our group seem to delimit a clinically stable condition in most patients in this subgroup.
authors.affiliation: Department of Nephrology, Hvidovre University Hospital, Denmark.
authors.forename: P B
authors.initials: PB
authors.lastname: Frandsen
authors.forename: N J
authors.initials: NJ
authors.lastname: Kriegbaum
authors.forename: S
authors.initials: S
authors.lastname: Ullman
authors.forename: M
authors.initials: M
authors.lastname: Høier-Madsen
authors.forename: A
authors.initials: A
authors.lastname: Wiik
authors.forename: P
authors.initials: P
authors.lastname: Halberg
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000974:Antibodies, Antinuclear / Q000032:analysis*; D002648:Child; D002675:Child, Preschool; D003240:Connective Tissue Diseases / Q000175:diagnosis / Q000276:immunology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000175:diagnosis / Q000276:immunology; D008297:Male; D008875:Middle Aged; D008947:Mixed Connective Tissue Disease / Q000175:diagnosis / Q000276:immunology; D017412:Ribonucleoprotein, U1 Small Nuclear / Q000276:immunology*; D012595:Scleroderma, Systemic / Q000175:diagnosis / Q000276:immunology
chemical_list: D000974:Antibodies, Antinuclear; D017412:Ribonucleoprotein, U1 Small Nuclear