Abstract
Objectives. The multisystem manifestations of SSc can greatly impact patients' quality of life. The aim of this study was to identify factors associated with disability in SSc. Methods. SSc patients from the prospective DeSScipher cohort who had completed the scleroderma health assessment questionnaire (SHAQ), a disability score that combines the health assessment questionnaire and five visual analogue scales, were included in this analysis. The effect of factors possibly associated with disability was analysed with multiple linear regressions. Results. The mean SHAQ and HAQ scores of the 944 patients included were 0.87 (S.D. = 0.66) and 0.92 (S.D. = 0.78); 59% of the patients were in the mild to moderate difficulty SHAQ category (0≤SHAQ<1), 34% in the moderate to severe disability category (1≤SHAQ<2) and 7% in the severe to very severe disability category (2≤SHAQ≤3). The means of the visual analogue scales scores were in order of magnitude: overall disease severity (37 mm), RP (31 mm), pulmonary symptoms (24 mm), gastrointestinal symptoms (20mm) and digital ulcers (19 mm). In multiple regression, the main factors associated with high SHAQ scores were the presence of dyspnoea [modified New York Heart Association (NYHA) class IV (regression coefficient B= 0.62), modified NYHA class III (B = 0.53) and modified NYHA class II (B = 0.21; all vs modified NYHA class I)], FM (B = 0.37), muscle weakness (B = 0.27), digital ulcers (B = 0.20) and gastrointestinal symptoms (oesophageal symptoms, B= 0.16; stomach symptoms, B= 0.15; intestinal symptoms, B= 0.15). Conclusion. SSc patients perceive dyspnoea, pain, digital ulcers, muscle weakness and gastrointestinal symptoms as the main factors driving their level of disability, unlike physicians who emphasize objective measures of disability.
| Original language | English |
|---|---|
| Journal | Rheumatology (United Kingdom) |
| Volume | 57 |
| Issue number | 3 |
| Pages (from-to) | 441-450 |
| Number of pages | 10 |
| ISSN | 1462-0324 |
| DOIs | |
| Publication status | Published - 01.03.2018 |
Funding
Funding: This study, as part of the DeSScipher project, was supported by the European Community’s Framework Programme 7 [FP7-HEALTH-2012.2.4.4-2 Observational trials in rare diseases; grant agreement No. 305495]. Disclosure statement: D.H. received consultancies from Actelion, Switzerland. U.M.-L. is supported by the FP7 program of the European Commission. N.H. has received lecture fees and research support from Actelion and Bayer. Y.A. has received honoraria and/or research support/grants from Actelion, Bayer, Biogen, BMS, Genentech-Roche, Galapagos, Inventiva, Medac, Pfizer, Sanofi, Servier, Union Chimique Belge. O.D. had a consultancy relationship and/or has received research funding from 4 D Science, AbbVie, Actelion, Active Biotec, Bayer, BiogenIdec, Bristol-Myers Squibb, Boehringer Ingelheim, ChemomAb, EpiPharm, espeRare foundation, Genentech/Roche, GlaxoSmithKline, Inventiva, iQone Healthcare, Lilly, medac, Mepha, MedImmune, Pharmacyclics, Pfizer, Sanofi, Serodapharm and Sinoxa in the area of potential treatments of scleroderma and its complications. In addition, O.D. has a patent mir-29 for the treatment of systemic sclerosis licensed. V.L. received personal fees from the European Union Seventh Framework Program (FP7/2007-2013) during the conduct of the study. C.M. has received research support and/or honoraria and has/had consultancy relationship from Abbvie, Actelion, Genetech/Roche and Geneva Romfarm in the area of systemic sclerosis and its complications. B.M. had grant/research support from AbbVie, Protagen, EMDO and Novartis, congress support from Pfizer, Roche, Actelion and has a patent licensed for mir-29 for the treatment of systemic sclerosis. C.D. has received consultancy fees or honoraria from Actelion, Bayer, GSK, CSL Behring, Merck-Serono, Genentech-Roche, Inventiva, Sanofi-Aventis and Boehringer Ingelheim. All other authors have declared no conflicts of interest.
Research Areas and Centers
- Academic Focus: Center for Infection and Inflammation Research (ZIEL)
