ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 2711

Joint and Tendon Involvement Predict Severe Disease Progression in Systemic Sclerosis: A Prospective Study

Jerome Avouac1, Ulrich Walker2, Eric Hachulla3, Gabriela Riemekasten4, Giovanna Cuomo5, Patricia E. Carreira6, Paola Caramaschi7, Lidiya P. Ananieva8, Marco Matucci-Cerinic9, Laszlo Czirjak10, Christopher P. Denton11, Ulf Muller-Ladner12 and Yannick Allanore13, 1Paris Descartes University and INSERM U1016, Rheumatology A department, Cochin Hospital, Paris, France, 2Department of Rheumatology, Basel University, basel, Switzerland, 3Internal Medicine, National Scleroderma Centre, Lille CEDEX, France, 4Rheumatology and Clinical Immunology, Charité – University Hospital, Berlin, Germany, 5Department of Internal and Experimental Medicine, Second University of Naples, Naples, Italy, 6Rheumatology Department. Hospital Universitario 12 de Octubre, Madrid, Spain, 7Rheumatology Unit, Azienda Ospedaliera Universitaria Integrata, Verona, Italy, 8Microcirculation and Inflammation, Research Institute of Rheumatology RAMS, Moscow, Russia, 9Dept of Medicine/Div of Rheum, University of Florence, Florence, Italy, 10Rheumatology and Immunology Medical School, University of Pécs, Pécs, Hungary, 11Centre for Rheumatology, Royal Free and University College Medical School, London, United Kingdom, 12Internal Medicine/Rheumatology, Univ Giessen/Kerckhoff-Clinic, Bad Nauheim, Germany, 13Paris Descartes University, Rheumatology A Department and INSERM U1016, Cochin Hospital, Paris, France

Meeting: 2014 ACR/ARHP Annual Meeting

Keywords:

Session Information

Title: Systemic Sclerosis, Fibrosing Syndromes and Raynaud's - Clinical Aspects and Therapeutics: Determinants of Disease, Classification and Response

Session Type: Abstract Submissions (ACR)

Background/Purpose

Joint involvement is frequent and strongly contributes to impaired quality of life in systemic sclerosis (SSc). In a previous cross-sectional study, synovitis and tendon friction rubs (TFRs) were associated with systemic inflammation and disease activity. Therefore, the aim of the present study was to determine whether joint synovitis and TFRs can predict the progression of SSc over time. 

Methods

We included patients from the EUSTAR database (MEDS online) with early disease duration (first non Raynaud’s symptom equal or less than 3 years) and with a follow-up of at least two years. We extracted data regarding the presence or not of synovitis and TFRs and data related to disease progression. Skin progression was defined by a ≥30% worsening of the modified Rodnan skin score (mRSS). Lung progression was defined by the new onset of pulmonary fibrosis on high resolution CT scan, or the deterioration of lung volume (≥10% of forced vital capacity, FVC). Cardiovascular worsening was defined for skin by new ischemic digital ulcers (DU), for lung by pre-capillary pulmonary arterial hypertension (PAH) on right heart catheterization, and for heart by the reduction of the left ventricular ejection fraction below 50% on echocardiography. Renal progression was defined by the occurrence of scleroderma renal crisis. Overall disease progression was defined according to the occurrence of at least one organ progression.

Results

From the 9165 patients included in the database, 1301 patients (1079 females) met our inclusion criteria (mean ± SD age of 55±15 years, mean ± SD follow-up: 4.5±2.2 years). During the follow-up period, 579 patients (45%) experienced skin and/or lung and/or cardiovascular progression with a mean time to development of 3.2±1.9 years.

Joint synovitis (Hazard Ratio, HR: 1.26, 95% confidence interval, CI, 1.01-1.59) and TFRs (HR: 1.32, 95%CI 1.03-1.70) were both independently predictive of overall disease progression, as were also the diffuse cutaneous subset (HR: 1.30, 95%CI 1.05-1.61) and positive antitopoisomerase-I antibodies (HR: 1.25, 95%CI 1.02-1.53).

The mean change of mRSS over the follow-up period was 9.4±4.11, and 99/123 patients (80%) had a progression of at least 5 points. Joint synovitis (HR: 1.63, 95%CI 1.05-2.55) and TFRs (HR: 1.67, 95%CI 1.01-2.75) were independently predictive of skin progression.

Joint synovitis was predictive of the occurrence of new digital ulcer(s) (HR: 1.45, 95%CI 1.08-1.96) and decreased left ventricular ejection fraction (HR: 2.20, 95%CI 1.06-4.57); TFRs were confirmed to be an independent predictor of scleroderma renal crisis (HR: 2.33, 95% CI 1.03-6.19). 

Conclusion

This first report of the prospective follow-up of EUSTAR patients identified for the first time the merit of baseline synovitis and extended previous data for tendon friction rubs in early SSc patients. These results obtained through the largest worldwide database support the use of these easily detected clinical findings for the risk stratification of SSc patients. These parameters might be used in the future to select high-risk patients, guide therapies and might be regarded as potential surrogate markers for severity.

Disclosure:

J. Avouac,
None;

U. Walker,
None;

E. Hachulla,
None;

G. Riemekasten,
None;

G. Cuomo,
None;

P. E. Carreira,
None;

P. Caramaschi,
None;

L. P. Ananieva,
None;

M. Matucci-Cerinic,
None;

L. Czirjak,
None;

C. P. Denton,
None;

U. Muller-Ladner,
None;

Y. Allanore,
None.

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