Undifferentiated connective tissue disease with antibodies to Ro/SSA: Clinical features and follow-up of 148 patients

I. Cavazzana, F. Franceschini, N. Belfiore1, M. Quinzanini, R. Caporali1, P. Calzavara-Pinton2, L. Bettoni, A. Brucato3, R. Cattaneo, C. Montecucco1

Servizio di Allergologia e Immunologia Clinica, Cattedra di Immunologia Clinica, Spedali Civili, Universitá degli Studi di Brescia, Brescia; 1Servizio di Reumatologia, IRCCS Policlinico S. Matteo, Pavia; 2Divisione di Dermatologia, Spedali Civili, Brescia; 3Divisione Medica "Brera" e Divisione di Reumatologia, Ospedale Niguarda Ca' Granda, Milano, Italy.

ABSTRACT
Objective
To evaluate the clinical and serologic profile, the rate of progression to well defined CTD and the possible predictors of disease evolution in patients affected by UCTD with antibodies anti-Ro/SSA. 

Methods
148 patients diagnosed as UCTD were retrospectively evaluated. Antibodies to SSA/Ro were determined by counterimmunoelectrophoresis and ELISA. 

Results
Thirty-six patients (24.3%) developed a well-defined CTD after a mean follow-up of 4.5 years. Most patients developed primary Sjögren's syndrome (SS) (50%) or systemic lupus erythematosus (SLE) (30.5%). Leukopenia and xerophthalmia developed more frequently in the group of patients evolving to defined CTDs (p < 0.0032 and p < 0.0063). Leukopenia independently predicted the evolution in CTD by multivariate regression analysis (p < 0.019). Anti-dsDNA predicted the evolution in SLE (p < 0.0207), while the presence of additional anti-ENA specificity to  anti-Ro/SSA was not associated with the outcome. 

Conclusion
24.3% of patients with UCTD and antibodies to Ro/SSA can progress in a relatively short period of time to well-defined CTDs. The development of primary SS could be predicted by xerophthalmia and SLE by the appearance of anti-dsDNA antibodies. 

Key words
UCTD, anti-Ro/SSA antibodies, Sjögren's syndrome, systemic vasculitis, anti-ENA antibodies.


This study was supported in part by grant no. 96.00963.CT 04 from the Consiglio Nazionale delle Ricerche, Italy.

Please address correspondence to: Dr. Ilaria Cavazzana, MD, Servizio di Immunologia Clinica, Spedali Civili, Piazzale Spedali Civili no. 1, 25100 Brescia, Italy. 
E-mail: cattaneo@master.cci.unibs.it 

Clin Exp Rheumatol 2001; 19: 403-409.
© Copyright Clinical and Experimental Rheumatology 2001.