Functional Disability and Its Predictors in Systemic Sclerosis: A Study from the Desscipher Project within the European Scleroderma Trials and Research Group

Veronika K. Jaeger¹, Lazlo Czirjak², Veronika Lóránd², Gabriele Valentini³, Serena Vettori⁴, Francesco Del Galdo⁵, Giuseppina Abignano⁶, Oliver Distler⁷, Britta Maurer⁷, Christopher Denton⁸, Svetlana Nihtyanova⁹, Yannick Allanore¹⁰, Jerome Avouac¹¹, Gabriela Riemekasten¹², Elise Siegert¹³, Dörte Huscher¹⁴, Marco Matucci-Cerinic¹⁵, Serena Guiducci¹⁶, Marc Frerix¹⁷, Ingo H. Tarner¹⁸, Beata Garay-Toth¹⁹, Lidiya P. Ananieva²⁰, Franco Cozzi²¹, Sule Yavuz²², Nicolas Hunzelmann²³, Alessandra Vacca²⁴, Tim Schmeiser²⁵, Simona Rednic²⁶, Valeria Riccieri²⁷, Brigitte Krummel-Lorenz²⁸, Armando Gabrielli²⁹, Paloma Garcia De La Peña³⁰, Codrina Ancuta³¹, Ulf Müller-Ladner³², Ulrich A. Walker¹ and on behalf of the DeSScipher Consortium and contributing EUSTAR centres, ¹Department of Rheumatology, University Hospital Basel, Basel, Switzerland, ²Department of Rheumatology and Immunology, University of Pécs, Pécs, Hungary, ³Internal and Experimental Medicine, Rheumatology Unit, Second University of Naples, Naples, Italy, ⁴Department of Internal and Experimental Medicine, Rheumatology Unit, Second University of Naples, Naples, Italy, ⁵Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds and NIHR Leeds Musculoskeletal Biomedical Research Unit, Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom, ⁶Clinical and Experimental Medicine, Second University of Naples, Napoli, Italy, ⁷Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland, ⁸Division of Medicine, Centre for Rheumatology and Connective Tissue Disease, University College London, London, United Kingdom, ⁹Centre for Rheumatology and Connective Tissue Diseases, University College London Medical School, Royal Free Hospital, London, United Kingdom, ¹⁰Rheumatology, Paris Descartes University, Paris, France, ¹¹Department of Rheumatology, University of Paris Descartes, Paris, France, ¹²Department of Rheumatology, Universitatsklinikum Schleswig-Holstein, Lubeck, Germany, ¹³Rheumatology and Clinical Immunology, Charité – University Medicine Berlin, Berlin, Germany, ¹⁴Epidemiology, German Rheumatism Research Centre, Berlin, Germany, ¹⁵Department of Medicine, Division of Rheumatology, University of Florence, Florence, Italy, ¹⁶Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence, Florence, Italy, ¹⁷Department of Rheumatology and Clinical Immunology, Justus-Liebig-University Giessen, Kerckhoff-Klinik, Bad Nauheim, Germany, ¹⁸Rheumatology and Clinical Immunology, Justus-Liebig-University of Giessen, Kerckhoff-Klinik, Bad Nauheim, Germany, ¹⁹FESCA, Budapest, Hungary, ²⁰Microcirculation and Inflammation, Research Institute of Rheumatology RAMS, Moscow, Russia, ²¹Division of Rheumatology, University Hospital of Padova, Padova, Italy, ²²Marmara University Faculty of Medicine, Rheumatology, Istanbul, Turkey, ²³Department of Dermatology, University of Cologne, Cologne, Germany, ²⁴University Hospital of Cagliari, Rheumatology Unit, Monserrato, Italy, ²⁵Krankenhaus St. Josef, Wuppertal, Germany, ²⁶Rheumatology, Emergency County Clinical Hospital Cluj Napoca, Cluj-Napoca, Romania, ²⁷Cattedra di Reumatologia, Dip Clinica e Terapia Medica, Sapienza Università di Roma, Roma, Italy, ²⁸Rheumatologist, Frankfurt, Germany, ²⁹Istituto di Clinica Medica Generale, Ematologia ed Immunologia Clinica, Università Politecnica delle Marche, Ancona, Italy, ³⁰Rheumatology, Hospital Madrid Norte Sanchinarro, Madrid, Spain, ³¹G.T.Popa Center for Biomedical Research, Iasi, Romania, ³²Justus-Liebig-University Giessen, Department of Internal Medicine and Rheumatology, Kerckhoff-Klinik, Bad Nauheim, Germany, Bad-Nauheim, Germany

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords: Systemic sclerosis

Session Information

Date: Monday, November 14, 2016

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

Session Type: ACR Poster Session B

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

Background/Purpose: Systemic sclerosis (SSc) can greatly impact the patients’ quality of life due to its multisystem manifestations. The health assessment questionnaire (HAQ) is one of the most commonly used measures of disability in musculoskeletal disorders and was extended to form the scleroderma HAQ (SHAQ), a more disease-specific disability scale that incorporates the HAQ and 5 visual analogue scales (VAS) into one score. This cross-sectional study aims to identify contributors of disability in SSc by means of the SHAQ.

Methods: Adult patients from the prospective DeSScipher cohort were included in the analysis if they had one complete SHAQ recorded (range 0-3) and fulfilled either the 1980 ACR or the 2013 ACR/EULAR criteria for SSc. Multiple linear regression analysis was used to assess the combined effect of factors defined a priori and possibly associated with a lower SHAQ.

Results: Between June 2013 and January 2016, 813 patients had one complete SHAQ recorded (34% of all patients followed in the DeSScipher cohort). The mean SHAQ score was 0.86 (standard deviation [SD] 0.65) and the mean HAQ score was 0.92 (SD 0.77). 60% of patients were in the “mild to moderate difficulty” SHAQ category (score of 0-1), 34% in the “moderate to severe disability” category (score of 1-2) and 6% in the “severe to very severe disability” category (score of 2-3). In order of magnitude, the means of the five VASs included in the SHAQ were: Overall disease severity (30, IQR 10-51), Raynaud’s phenomenon (21, IQR 3-50), pulmonary symptoms (10, IQR 1-40), gastrointestinal symptoms (6, IQR 1-31) and digital ulcers (2, IQR 1-30). In multiple linear regression, the main contributor to functional disability was dyspnea. The SHAQ scores reported by patients with NYHA class 4, 3 or 2 were on average 0.65 units (95% confidence interval [CI] 0.30-1.01), 0.58 units (95%CI 0.41-0.75) and 0.19 units (95%CI 0.10-0.27) higher than that of patients with NYHA class 1. The presence of fibromyalgia (0.41 units, 95%CI 0.21-0.62) as well as muscle weakness (0.25 units, 95%CI 0.14-0.36) were also associated with higher levels of disability (Figure). Patients reporting esophageal, gastric and intestinal symptoms simultaneously had, on average, a SHAQ score of 0.45 units (95% CI 0.33-0.58) higher than patients reporting no gastrointestinal symptoms. Patients with symptoms in two gastrointestinal regions had a SHAQ score of 0.28 units (95%CI 0.17-0.39) and in one region of 0.13 units (95% CI 0.04-0.23) higher than patients with no symptoms. The contributing factors to an impaired functional ability were similar in patients with diffuse SSc and patients with limited SSc.

Conclusion: Patients perceive dyspnea, pain, muscle weakness and gastrointestinal symptoms as the main factors driving their level of disability. Acknowledgement: The DeSScipher project was funded by the European Community’s Framework Programme 7 under grant agreement N° 305495.

Disclosure: V. K. Jaeger, None; L. Czirjak, None; V. Lóránd, None; G. Valentini, None; S. Vettori, None; F. Del Galdo, None; G. Abignano, None; O. Distler, None; B. Maurer, None; C. Denton, None; S. Nihtyanova, None; Y. Allanore, None; J. Avouac, None; G. Riemekasten, None; E. Siegert, None; D. Huscher, None; M. Matucci-Cerinic, None; S. Guiducci, None; M. Frerix, None; I. H. Tarner, None; B. Garay-Toth, None; L. P. Ananieva, None; F. Cozzi, None; S. Yavuz, None; N. Hunzelmann, None; A. Vacca, None; T. Schmeiser, None; S. Rednic, None; V. Riccieri, None; B. Krummel-Lorenz, None; A. Gabrielli, None; P. Garcia De La Peña, None; C. Ancuta, None; U. Müller-Ladner, None; U. A. Walker, None.

To cite this abstract in AMA style:

Jaeger VK, Czirjak L, Lóránd V, Valentini G, Vettori S, Del Galdo F, Abignano G, Distler O, Maurer B, Denton C, Nihtyanova S, Allanore Y, Avouac J, Riemekasten G, Siegert E, Huscher D, Matucci-Cerinic M, Guiducci S, Frerix M, Tarner IH, Garay-Toth B, Ananieva LP, Cozzi F, Yavuz S, Hunzelmann N, Vacca A, Schmeiser T, Rednic S, Riccieri V, Krummel-Lorenz B, Gabrielli A, Garcia De La Peña P, Ancuta C, Müller-Ladner U, Walker UA. Functional Disability and Its Predictors in Systemic Sclerosis: A Study from the Desscipher Project within the European Scleroderma Trials and Research Group [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/functional-disability-and-its-predictors-in-systemic-sclerosis-a-study-from-the-desscipher-project-within-the-european-scleroderma-trials-and-research-group/. Accessed .

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