[2008] [FRI0256] SEROLOGIC PROFILE AND MORTALITY RATES OF SCLERODERMA RENAL CRISIS IN ITALY
V. Codullo1, I. Cavazzana2, C. Bonino1, F. Cozzi3, N. Del Papa4, C. Ferri5, F. Franceschini2, R. Giacomelli6, M. Matucci-Cerinic7, G. Morozzi8, A. Ruffatti3, G. Valentini9, C. Montecucco1 1Chair of Rheumatology, University of Pavia, Policlinico San Matteo, Pavia; 2Department of Allergology and Clinical Immunology, Spedali Civili, Brescia; 3Chair of Rheumatology, University of Padua, Padua; 4Rheumatology, G. Pini Hospital, Milan; 5Rheumatology, University of Modena-Reggio Emilia, Modena; 6Department of Internal Medicine and Public Health, University of L'Aquila, L'Aquila; 7Department of Medicine and Rheumatology, University of Florence, Florence; 8Department of Clinical Medicine and Immunological Science, University of Siena, Siena; 9Department of Clinical and Experimental Medicine, Second University of Naples, Naples, Italy
Background: The prevalence of scleroderma renal crisis (SRC) has been reported to differ among various ethnic groups, having been described higher figures in both Caucasians and Afro-Americans from USA and Northern Europe with respect to systemic sclerosis (SSc) patients of the Mediterranean area. A similar trend has been reported for antibodies anti-RNA polymerase (RNAP) I-III, which are a serologic marker of SSc with diffuse cutaneous (dc) involvement and SRC.
Objectives: To analyse the serological characteristics and survival of Italian patients with SSc and SRC.
Methods: A retrospective analysis was performed on the medical records from 9 tertiary rheumatologic referral centres in Italy from 1984 to 2006. Patients with a diagnosis of SRC and an available serum sample for laboratory tests were included. The following serologic tests were performed: anti-nuclear antibodies (ANA) in indirect immunofluorescence, anti-extractable nuclear antigens (ENA) by ELISA, anti-RNAP by ELISA for the subunit III and by immunoprecipitation.
Results: A total of 41 cases were identified and evaluated. Mean age at SSc onset was 52.8±13.2 years and at SRC 55.4±11.8 years. Female:male ratio was 5:1. Ninety percent of patients had a dc-SSc. Cumulative survival rates were 55%, 47.5%, 38% and 33% at 1,2,5 and 10 years of follow-up respectively. Thirty-nine patients had a positive ANA test (95%) and 34 (83%) had an anti-ENA or anti-RNAP I-III reactivity. ELISA and IP for anti-RNAP gave concordant results in all cases and positive sera in ELISA showed a reactivity to both subunits I and III. Anti-topoisomerase (topo) I antibodies were detected in 27 (66%) patients, whereas anti-RNAP I-III in 5 (12%). Anti-topo I positive patients (A) had a mean age of 52.3±11.1 years at SSc onset, 92% of them showed a dc-SSc. In the anti-RNAP I-III positive group (B), mean age at SSc onset was 48±8.15 years (A vs B NS) and all patients had a dcSSc. There was a statistically significant difference in survival rates of dcSSc patients in these two groups: anti-topo I patients had a cumulative survival rate of 55%, 35% and 15% at 1,2 and 10 years of follow-up respectively; in the anti-RNAP I-III group cumulative survival was of 80% after 8 years of follow-up (A vs B p=0.028 log-rank test).
Conclusion: SRC is a rare manifestation of SSc in Italy but it is still associated with a severe prognosis. The autoimmune profile in SRC does not seem to be specifically associated with anti-RNAP I-III in our series, while there is a relative increase of anti-topo I positive patients. This feature might account for the higher mortality rates that we observed since anti-topo I positive SRC is associated with a poorer survival compared to anti-RNAP I-III positive SRC. Larger and prospective studies are needed to clarify these clinical and serological associations.
Ann Rheum Dis 2008;67(Suppl II):366
Scleroderma, Myositis and related syndromes
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