[2009] [THU0323] PROFILE OF PRE-SCLERODERMA EVOLUTION

E. Delgado, B. Rguez-Lozano, V. Hernandez, M. Gantes, S. Bustabad, J. Hernandez Rheumatology, Hospital Universitario de Canarias, La Laguna, Spain

Background: Factors that condition the evolution of pre-scleroderma to systemic sclerosis (SSc) are not well established.
Objectives: To describe the pattern of evolution and identify possible predictive risk factors in the pre-scleroderma phase.
Methods: Retrospective study of patients diagnosed with pre-scleroderma based on the presence of Raynaud's phenomenon and positive autoantibodies (nucleolar pattern ANA or anti-Scl-70 or anticentromere antibodies) and/or capillaroscopic findings of scleroderma pattern, during a period of 10 years. These patients were referred to our Rheumatolgy Department for capillaroscopy during the years 1999-2001. Clinical evaluation was performed (degree of cutaneous involvement using a modified Rodnan skin score, ANA and specificities, capillaroscopy performed by the same observer, ECG, chest radiography, respiratory function tests (spirometry/DLCO), echocardiogram, esophageal manometry and renal function) at the baseline and end of the study. At baseline, none of the patients met the criteria for the classification of SSc (ACR 1980).
Results: Of 290 patients undergoing capilloroscopy in the period 1999-2001, 17 patients (7%), all women, median age 52 years (19-62), were diagnosed with pre-scleroderma (7%) and 1 patient with diffuse SSc after the first clinical/immunological/capillaroscopic evaluation. Reasons for performing capillaroscopy in the selected population were: Raynaud's (65%), sclerodactyly (12%) and Raynaud's with positive Scl-70/anticentromere antibodies (18%). Baseline capillaroscopic patterns (Maricq) were: Slow escleroderma (5.28%), active scleroderma (3.17%), non-specific pattern of connective tissue disorders (6.34%) and normal (4. 23%). Clinical data included sclerodactyly (12. 62%), dysphagia (11. 62%), pathologic esophageal manometry (47%)( inferior esophageal sphincter hypotonia:7; esophageal aperistalsis: 1), chest radiography with interstitial pattern (4.24%) with DLCO/VA alteration in 2 patients. At baseline, no patients presented sclerodactyly-type skin involvement associated with lung involvement. Follow-up during 8-10 years was performed in 15 patients (one was lost to follow up and one died after 3 years). Evolution to defined SSc was found in 5 patients (30%): 4 diffuse (1 being the patient who died) and 1 with SSc sub-type CREST. All these patients at baseline had presented: Raynaud, positive Scl-70 or anticentromere antibodies, cutaneous involvement of the sclerodactyly or visceral (esophageal) type and slow capillaroscopic pattern of scleroderma.
Conclusion:
1.Of all patients referred for capillaroscopy in a 3-year period, 7% were diagnosed with pre-scleroderma.
2.In this exploratory study, evolution to defined SSc was observed in 30% of these patients after a 10-year follow up period.
3.Given the infrequency of this disease, multicenter studies are required to be able to draw conclusions with statistical power. However, all those patients who evolved to SSc presented the following at baseline: Raynaud's, positive anti-Scl-70 or anticentromere, sclerodactyly or visceral (esophageal) involvement and slow capillaroscopic pattern of scleroderma.
Disclosure of Interest: None declared

Ann Rheum Dis 2009;68(Suppl3):279

Scleroderma, myositis and related syndromes