Early Versus Late Onset Systemic Sclerosis: Analysis of 1037 Patients From Rescle Registry

Marco A. Alba¹, Juan Carlos Mejia², Gerard Espinosa³, María-Victoria Egurbide⁴, Carles Tolosa⁵, Luis Trapiella⁶, Carmen Pilar Simeon⁷, Vicent Fonollosa⁸ and And RESCLE investigators⁹, ¹Systemic Autoimmune Diseases, Vasculitis Research Unit. Hospital Clínic. University of Barcelona. IDIBAPS, Barcelona, Spain, ²Department of Systemic Autoimmune Diseases, Hospital Clinic University Barcelona, Barcelona, Spain, ³Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Barcelona, Spain, ⁴Autoimmune Disease Research Unit, Service of Internal Medicine,, Hospital de Cruces, UPV/EHU, Barakaldo, Spain, Barakaldo, Spain, ⁵Department of Internal Medicine, Corporación Sanitaria Universitaria Parc Taulí, Barcelona, Spain, ⁶Department of Internal Medicine,, Hospital Universitario Central de Asturias, Asturias, Spain, ⁷Servicio de Medicina Interna, Hospital Valle de Hebron, Barcelona, Spain, ⁸Department of Internal Medicine, Hospital Vall dxHebron, Barcelona, Spain, ⁹RESCLE, Barcelona, Spain

Meeting: 2012 ACR/ARHP Annual Meeting

Keywords: Morbidity and mortality and systemic sclerosis

Session Information

Title: Systemic Sclerosis, Fibrosing Syndromes, and Raynaud’s – Clinical Aspects and Therapeutics

Session Type: Abstract Submissions (ACR)

Background/Purpose: Systemic sclerosis (SSc) is characterized by extensive fibrosis, vascular dysfunction and the presence of several auto-antibodies. As in other autoimmune diseases, age at disease onset seems to modify initial and cumulative clinical manifestations. The aim of this study was to determine if the age at disease onset may modulate the clinical characteristics and evolution of patients with SSc.

Methods: The Spanish Network for Systemic Sclerosis recruited 1037 patients with a mean follow-up of 5.2±6.8 years. Based on the mean ± 1SD of age at disease onset (45±16 years), patients were classified in 3 groups; Group 1: age equal or below 30 years (early onset); Group 2: age between 31 and 58 years, and Group 3: age equal or older than 59 years (late onset). We compared the initial clinical presentation, capillaroscopy pattern, immunological features, cumulative clinical manifestations and death rates between the three groups.

Results: One hundred and ninety five patients belonged to group 1, 651 to group 2 and 191 to group 3. Female distribution was similar between the three groups (91%, 86%, and 88%). Interestingly, time from disease onset to diagnosis was significantly higher in patients with early onset (group 1) (12±13, 5.8±6.7, and 2.4±3.6 years; p<0.001). Raynaud’s phenomenon was the most frequent initial manifestation without differences between the three groups (88%, 84%, and 78%; p=0.134). Patients with early onset SSc had higher prevalence of myositis (11%, 7.2%, and 2.9%; p=0.009), esophageal involvement (72%, 67%, and 56%; p=0.004) and lower prevalence of centromeric antibodies (33%, 46%, and 47%; p=0.007). In contrast, patients with late onset SSc was characterized by lower prevalence of digital ulcers (54%, 41%, and 34%; p<0.001) but higher rates of heart conduction system alterations (8.7%, 13%, and 21%; p=0.004), and pulmonary hypertension (12%, 19%, and 25%; p=0.048). Mortality tended to be higher in late onset patients (9.7%, 15%, and 18%; p=0.053) and the Kaplan–Meier survival curves were significantly different (p<0.0001) for the three groups of patients.

Conclusion: Age at disease onset is associated with differences in clinical presentation and outcome in patients with SSc.

Disclosure:

M. A. Alba,
None;

J. C. Mejia,
None;

G. Espinosa,
None;

M. V. Egurbide,
None;

C. Tolosa,
None;

L. Trapiella,
None;

C. P. Simeon,
None;

V. Fonollosa,
None;

A. RESCLE investigators,
None.

« Back to 2012 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/early-versus-late-onset-systemic-sclerosis-analysis-of-1037-patients-from-rescle-registry/