Impaired Quality of Life in Systemic Sclerosis and Patient Perception of the Disease: A Large International Survey

Camelia Frantz¹, Jerome Avouac¹, Oliver Distler², Fazia Amrouche¹, Dominique Godard³, Ann Kennedy⁴, John Varga⁵, Marco Matucci-Cerinic⁶ and Yannick Allanore⁷, ¹Paris Descartes University, Rheumatology A department, Cochin Hospital, Paris, France, ²Division of Rheumatology, University Hospital And Eular Scleroderma Trials And Research (EUSTAR) Board, Zurich, Switzerland, ³Association des Sclérodermie de France, Auxerre, France, ⁴Federation of European Scleroderma Associations (FESCA), Tournai, Belgium, ⁵Division of Rheumatology, Northwestern University, Feinberg School of Medicine, Chicago, IL, ⁶Rheumatology Department, University of Florence And World Scleroderma Foundation, Florence, Italy, ⁷Paris Descartes University, Rheumatology A department, Cochin Hospital, And Eular Scleroderma Trials And Research (EUSTAR) Board, Paris, France

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: Quality of life and systemic sclerosis

Session Information

Date: Sunday, November 8, 2015

Title: Systemic Sclerosis, Fibrosing Syndromes and Raynaud's - Clinical Aspects and Therapeutics Poster I

Session Type: ACR Poster Session A

Session Time: 9:00AM-11:00AM

Background/Purpose: Systemic sclerosis (SSc) is recognized as one of the most fatal rheumatic diseases, but it also promotes many detrimental effects on health-related quality of life (HRQOL). However, previous studies on HRQOL in SSc have lacked power, due to the difficulties involved in recruiting large samples for such a rare condition. Indeed, most previous studies focused on the outcome of patients in the early stages of disease or with DcSSc. The purpose of this study was to assess HRQOL and disease perception in a large, international group of SSc patients using validated questionnaires.

Methods: We conducted an international cross-sectional survey supported by EUSTAR, FESCA and Scleroderma US Foundation, from December 2013 to April 2014. We designed a standardized questionnaire translated and available on a website, including: Socio-demographic information, disease characteristics and self-assessment questionnaires namely Short Form 36 (SF-36) and Revised Illness Perception Questionnaire (IPQ-R).

Results: 1902 SSc patients from 60 countries were included with a mean±standard deviation (SD) age of 54±16 years and a mean±SD disease duration of 13±12 years. 712 (34.4 %) patients had DcSSc, 853 (44.8 %) had limited cutaneous SSc (LcSSc) and 122 (6.4 %) had SSc sine scleroderma.

HRQOL was found to be strongly impaired in SSc; in physical health (PCS, mean±SD 43.4±23.4; 100=best health) but also in mental health (MCS, mean±SD 52.3±23.1; 100=best health). SSc patients also had strongly perceptions about the chronic nature and negative consequence of the disease, and experienced negative emotions due to SSc.

Patients with DcSSc had a poorer HRQOL than those with LcSSc, for both physical (PCS, mean±SD 46.6±23.7 vs. 39.8±22.3; p<0.0001) and mental components (MCS, mean ± SD 53.8±23.0 vs. 50.3±23.2; p=0.003). They also had a more negative perception of their disease. Late-stage SSc patients were more likely to perceive their disease chronic (p<0.0001), less controllable (p=0.03) and with more consequences (p=0.008), but they also had a better understanding of their disease and experienced fewer negative emotions due to SSc.

Raynaud’s phenomenon was the organ involvement with the most severe impact on daily life, associated with the most negative perception of illness severity followed by gastrointestinal complications, musculoskeletal and skin lesions.

Non-European SSc patients reported greater impacts on QOL, particularly for the physical component (PCS, mean±SD 39.9±22.6 vs. 46.0±23.7; p<0.0001), and they had a more negative perception of their illness than European SSc patients, but they had a better understanding of their disease and experienced fewer negative emotions.

Conclusion: This study provides unique information about the patients’ perception of the disease and its impact on QOL, according to disease subset, disease duration and various geographical origins. The key message overall is the major impact on QOL and the negative perceptions of their disease expressed by SSc patients. Nevertheless, perceptions of the illness tended to improve with disease duration, suggesting the development of effective coping strategies. These results should be taken into account in patient management and future trials.

Disclosure: C. Frantz, None; J. Avouac, None; O. Distler, None; F. Amrouche, None; D. Godard, None; A. Kennedy, None; J. Varga, None; M. Matucci-Cerinic, None; Y. Allanore, None.

To cite this abstract in AMA style:

Frantz C, Avouac J, Distler O, Amrouche F, Godard D, Kennedy A, Varga J, Matucci-Cerinic M, Allanore Y. Impaired Quality of Life in Systemic Sclerosis and Patient Perception of the Disease: A Large International Survey [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/impaired-quality-of-life-in-systemic-sclerosis-and-patient-perception-of-the-disease-a-large-international-survey/. Accessed .

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