Disability, fatigue, pain and their associates in early diffuse cutaneous systemic sclerosis: The European Scleroderma Observational Study

Sébastien Peytrignet; Christopher P. Denton; Mark Lunt; Roger Hesselstrand; Luc Mouthon; Alan Silman; Xiaoyan Pan; Edith Brown; László Czirják; Jörg H.W. Distler; Oliver Distler; Kim Fligelstone; William J. Gregory; Rachel Ochiel; Madelon Vonk; Codrina Ancuţa; Voon H. Ong; Dominique Farge; Marie Hudson; Marco Matucci-Cerinic; Alexandra Balbir-Gurman; Øyvind Midtvedt; Alison C. Jordan; Wendy Stevens; Pia Moinzadeh; Frances C. Hall; Christian Agard; Marina E. Anderson; Elisabeth Diot; Rajan Madhok; Mohammed Akil; Maya H. Buch; Lorinda Chung; Nemanja Damjanov; Harsha Gunawardena; Peter Lanyon; Yasmeen Ahmad; Kuntal Chakravarty; Søren Jacobsen; Alexander J. MacGregor; Neil McHugh; Ulf Müller-Ladner; Gabriela Riemekasten; Michael Becker; Janet Roddy; Patricia E. Carreira; Anne Laure Fauchais; Eric Hachulla; Jennifer Hamilton; Murat Inanç; John S. McLaren; Jacob M. van Laar; Sanjay Pathare; Susanna Proudman; Anna Rudin; Joanne Sahhar; Brigitte Coppere; Christine Serratrice; Tom Sheeran; Douglas J. Veale; Claire Grange; Georges Selim Trad; Ariane L. Herrick

doi:10.1093/rheumatology/kex410

Disability, fatigue, pain and their associates in early diffuse cutaneous systemic sclerosis: The European Scleroderma Observational Study

Sébastien Peytrignet, Christopher P. Denton, Mark Lunt, Roger Hesselstrand, Luc Mouthon, Alan Silman, Xiaoyan Pan, Edith Brown, László Czirják, Jörg H.W. Distler, Oliver Distler, Kim Fligelstone, William J. Gregory, Rachel Ochiel, Madelon Vonk, Codrina Ancuţa, Voon H. Ong, Dominique Farge, Marie Hudson, Marco Matucci-CerinicAlexandra Balbir-Gurman, Øyvind Midtvedt, Alison C. Jordan, Wendy Stevens, Pia Moinzadeh, Frances C. Hall, Christian Agard, Marina E. Anderson, Elisabeth Diot, Rajan Madhok, Mohammed Akil, Maya H. Buch, Lorinda Chung, Nemanja Damjanov, Harsha Gunawardena, Peter Lanyon, Yasmeen Ahmad, Kuntal Chakravarty, Søren Jacobsen, Alexander J. MacGregor, Neil McHugh, Ulf Müller-Ladner, Gabriela Riemekasten, Michael Becker, Janet Roddy, Patricia E. Carreira, Anne Laure Fauchais, Eric Hachulla, Jennifer Hamilton, Murat Inanç, John S. McLaren, Jacob M. van Laar, Sanjay Pathare, Susanna Proudman, Anna Rudin, Joanne Sahhar, Brigitte Coppere, Christine Serratrice, Tom Sheeran, Douglas J. Veale, Claire Grange, Georges Selim Trad, Ariane L. Herrick^*

^*Corresponding author for this work

Clinic of Rheumatology

61 Citations (Scopus)

Abstract

Objectives. Our aim was to describe the burden of early dcSSc in terms of disability, fatigue and pain in the European Scleroderma Observational Study cohort, and to explore associated clinical features. Methods. Patients completed questionnaires at study entry, 12 and 24 months, including the HAQ disability index (HAQ-DI), the Cochin Hand Function Scale (CHFS), the Functional Assessment of Chronic Illness Therapy-fatigue and the Short Form 36 (SF36). Associates examined included the modified Rodnan skin score (mRSS), current digital ulcers and internal organ involvement. Correlations between 12-month changes were also examined. Results. The 326 patients recruited (median disease duration 11.9 months) displayed high levels of disability [mean (S.D.) HAQ-DI 1.1 (0.83)], with 'grip' and 'activity' being most affected. Of the 18 activities assessed in the CHFS, those involving fine finger movements were most affected. High HAQ-DI and CHFS scores were both associated with high mRSS (r = 0.34, P < 0.0001 and r = 0.35, P < 0.0001, respectively). HAQ-DI was higher in patients with digital ulcers (P = 0.004), pulmonary fibrosis (P = 0.005), cardiac (P = 0.005) and muscle involvement (P = 0.002). As anticipated, HAQ-DI, CHFS, the Functional Assessment of Chronic Illness Therapy and SF36 scores were all highly correlated, in particular the HAQ-DI with the CHFS (ρ = 0.84, P < 0.0001). Worsening HAQ-DI over 12 months was strongly associated with increasing mRSS (ρ = 0.40, P < 0.0001), decreasing hand function (ρ = 0.57, P < 0.0001) and increasing fatigue (ρ =-0.53, P < 0.0001). Conclusion. The European Scleroderma Observational Study highlights the burden of disability in early dcSSc, with high levels of disability and fatigue, associating with the degree of skin thickening (mRSS). Impaired hand function is a major contributor to overall disability.

Original language	English
Article number	kex410
Journal	Rheumatology (United Kingdom)
Volume	57
Issue number	2
Pages (from-to)	370-381
Number of pages	12
ISSN	1462-0324
DOIs	https://doi.org/10.1093/rheumatology/kex410
Publication status	Published - 01.02.2018

Funding

1Centre for Musculoskeletal Research, The University of Manchester, Manchester Academic Health Science Centre, Manchester, 2Centre for Rheumatology and Connective Tissue Diseases, UCL Division of Medicine, Royal Free Campus, London, UK,3Department of Rheumatology, Lund University, Lund, Sweden, 4Service de Médecine Interne, Hôpital Cochin, Centre de Référence pour les Vascularités Nécrosantes et la Sclérodermie Systémique, Université Paris Descartes, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France, 5Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford, UK, 6Department of Rheumatology and Immunology, Medical Center, University of Pécs, Pécs, Hungary, 7Department of Internal Medicine 3, University of Erlangen-Nuremberg, Erlangen, Germany, 8Department of Rheumatology, University of Zurich, Zurich, Switzerland, 9Rehabilitation Services, Salford Royal NHS Foundation Trust, Salford, UK, 10Department of the Rheumatic Diseases, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands, 11Rheumatology 2 Department, Clinical Rehabilitation Hospital, ‘Grigore T. Popa’ University of Medicine and Pharmacy, Ias¸i, Romania, 12Unité Clinique de Médecine Interne, Maladies Auto-immunes et Pathologie Vasculaire, Hôpital Saint-Louis, Paris, France, 13Lady Davis Institute, Jewish General Hospital, Montreal, 14Department of Medicine, McGill University, Montreal, Canada, 15Department Experimental and Clinical Medicine, Division Rheumatology AOUC, University of Florence, Florence, Italy, 16B. Shine Rheumatology Unit, Rambam Heath Care Campus, Rappaport Faculty of Medicine, Technion, Haifa, Israel, 17Rheumatology Unit, Oslo University Hospital Rikshospitalet, Oslo, Norway, 18Queen Elizabeth Hospital Birmingham, UHB Foundation Trust, Birmingham, UK, 19Rheumatology Unit, St Vincent’s Hospital, Melbourne, Australia, 20Department for Dermatology, University of Cologne, Köln, Germany, 21Department of Clinical Medicine, Cambridge University NHS Hospital Foundation Trust, Cambridge, UK, 22Department of Internal Medicine, Hôtel-Dieu Hospital, University of Nantes, Nantes, France, 23University of Liverpool, Aintree University Hospital, Liverpool, UK, 24Service de Médecine Interne, Hôpital Bretonneau Tours, Tours, France,25Centre for Rheumatic Diseases, Royal Infirmary, Glasgow, 26Department of Rheumatology, Sheffield Teaching Hospitals, Sheffield,27Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, 28NIHR Leeds Musculoskeletal Biomedical Research Unit, Leeds Teaching Hospitals NHS Trust, Leeds, UK, 29Division of Immunology and Rheumatology, Stanford University, Stanford, CA, USA, 30Institute of Rheumatology, University of Belgrade School of Medicine, Belgrade, Serbia, 31Clinical and Academic Rheumatology, North Bristol NHS Trust, Bristol, 32Rheumatology, Nottingham University Hospitals NHS Trust, Nottingham, UK, 33Rheumatology, Nottingham NHS Treatment Centre, Nottingham,34Peter Maddison Rheumatology Centre, Llandudno Hospital, Llandudno, 35Department of Rheumatology, Queen’s Hospital, Romford, UK,36Copenhagen Lupus and Vasculitis Clinic, Center for Rheumatology and Spine Diseases, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark, 37Department of Medicine, Norwich Medical School, University of East Anglia, Norwich, 38Department of Rheumatology, Royal National Hospital for Rheumatic Diseases, Bath, UK, 39Department of Rheumatology and Clinical Immunology, Justus-Liebig University Giessen, Kerckhoff Klinik, Bad Nauheim, Germany, 40Department of Rheumatology, University of Lübeck, Lübeck, 41Department of Rheumatology and Clinical Immunology, University Hospital Charité Berlin, Berlin, Germany, 42Department of Rheumatology, Royal Perth Hospital, Perth, Australia, 43Servicio de Reumatologia, Hospital Universitario 12 de Octubre, Madrid, Spain, 44Internal Medicine Unit, Limoges University Hospital, Limoges, 45Centre National de Référence Maladies Systémiques et Auto-immunes Rares, Département de Médecine Interne et Immunologie Clinique, Université de Lille, Lille, France, 46Department of Rheumatology, Gateshead Hospitals Foundation Trust, Gateshead, UK, 47Department of Internal Medicine, Division of Rheumatology, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey, 48Fife Rheumatic Diseases Unit, Whyteman’s Brae Hospital, Kirkcaldy, Scotland, UK, 49Department of Rheumatology and Clinical Immunology, UMC Utrecht, Utrecht, the Netherlands, 50Rheumatology, James Cook University Hospital, Middlesbrough, UK, 51Rheumatology Unit, Royal Adelaide Hospital, 52Discipline of Medicine, University of Adelaide, Adelaide, Australia, 53Department of Rheumatology and Inflammation Research, The Sahlgrenska Academy at Gothenburg University, Gothenburg, Sweden, 54Monash Centre for Inflammatory Diseases, Monash University, Melbourne, Australia, 55Department of Internal Medicine, Hôpital Edouard Herriot, Lyon, 56Department of Internal Medicine, Foundation Hospital Saint Joseph, Marseille, France, 57Department of Rheumatology, Cannock Chase Hospital, Cannock, UK, 58Rheumatology, St Vincent’s University Hospital, Dublin, Ireland, 59Department of Internal Medicine 69310, Centre Hospitalier Lyon Sud, Pierre-Bénite, Lyon, France, 60Internal Medecine, Ambroise Paré Hospital, Boulogne, Billancourt, 61Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester and 62NIHR Manchester Musculoskeletal Biomedical Research Unit, Central Manchester NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK Funding: This study was a part of ESOS, which was funded by a grant from the EULAR (European League Against Rheumatism) Orphan Disease Programme. Additional funding from Scleroderma and Raynaud’s UK allowed a one-year extension of the study. Disclosure statement: H.G. has done consultancy work and received honoraria from Actelion, UK. C.P.D. has done consultancy for GlaxoSmithKline (GSK), Actelion, Bayer, Inventiva and Merck-Serono, received research grant funding from GSK, Actelion, CSL Behring and Inventiva, received speaker’s fees from Bayer and given trial advice to Merck-Serono. A.R. receives funding from AstraZeneca. A.L.H. has done consultancy work for Actelion, served on a Data Safety Monitoring Board for Apricus, received research funding and speaker’s fees from Actelion, and speaker’s fees from GSK. S.P. has received research grants from Actelion Pharmaceuticals Australia, Bayer, CSL Biotherapies, GlaxoSmithKline Australia and Pfizer and speaker fees from Actelion. L.C. has done advisory board work for Gilead and Actelion and served on Data Safety Monitoring Boards for Cytori and Reata. O.D. had consultancy relationships and/or has received research funding from 4D Science, AbbVie, Actelion, Active Biotec, Bayer, BiogenIdec, Bristol-Myers Squibb (BMS), Boehringer Ingelheim, ChemomAb, EpiPharm, espeRare foundation, Genentech/Roche, GSK, Inventiva, iQone Healthcare, Lilly, medac, Mepha, MedImmune, Mitsubishi Tanabe Pharma, Pharmacyclics, Pfizer, Sanofi, Serodapharm and Sinoxa in the area of potential treatments of scleroderma and its complications and has a patent mir-29 for the treatment of systemic sclerosis licensed. U.M.-L. was funded in part by EUSTAR, EULAR and the European Community (Desscipher FP 7 Program). J.M.v.L. has received honoraria from Arthrogen, BMS, Eli Lilly, MSD, Pfizer, Roche, and research grants from AstraZeneca, Genentech and MSD. T.P.S. has received support from UCB, Roche, Pfizer and Novartis. W.G. has received teaching fees from Pfizer. M.E.A. has undertaken advisory board work and received honoraria from Actelion and received speaker’s fees from BMS. F.H. had her attendance at the Systemic Sclerois World Congress, Lisbon 18 20 February 2016 sponsored by Actelion Pharmaceutical and Actelion Pharmaceutical part-funded Specialist Nurse 2017 19 (£50 848). N.D. has done consultancy work for Abbvie, Pfizer, Roche and MSD, received speaker’s fees from AbbVie, Boehringer-Ingelheim, Pfizer, Richter Gedeon, Roche and MSD. All other authors have declared no conflicts of interest.

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

SDG 3 Good Health and Well-being

Research Areas and Centers

Academic Focus: Center for Brain, Behavior and Metabolism (CBBM)

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10.1093/rheumatology/kex410

Cite this

Peytrignet, S., Denton, C. P., Lunt, M., Hesselstrand, R., Mouthon, L., Silman, A., Pan, X., Brown, E., Czirják, L., Distler, J. H. W., Distler, O., Fligelstone, K., Gregory, W. J., Ochiel, R., Vonk, M., Ancuţa, C., Ong, V. H., Farge, D., Hudson, M., ... Herrick, A. L. (2018). Disability, fatigue, pain and their associates in early diffuse cutaneous systemic sclerosis: The European Scleroderma Observational Study. Rheumatology (United Kingdom), 57(2), 370-381. Article kex410. https://doi.org/10.1093/rheumatology/kex410

@article{5165bd07e4944dcd970bd113c2bce85b,

title = "Disability, fatigue, pain and their associates in early diffuse cutaneous systemic sclerosis: The European Scleroderma Observational Study",

abstract = "Objectives. Our aim was to describe the burden of early dcSSc in terms of disability, fatigue and pain in the European Scleroderma Observational Study cohort, and to explore associated clinical features. Methods. Patients completed questionnaires at study entry, 12 and 24 months, including the HAQ disability index (HAQ-DI), the Cochin Hand Function Scale (CHFS), the Functional Assessment of Chronic Illness Therapy-fatigue and the Short Form 36 (SF36). Associates examined included the modified Rodnan skin score (mRSS), current digital ulcers and internal organ involvement. Correlations between 12-month changes were also examined. Results. The 326 patients recruited (median disease duration 11.9 months) displayed high levels of disability [mean (S.D.) HAQ-DI 1.1 (0.83)], with 'grip' and 'activity' being most affected. Of the 18 activities assessed in the CHFS, those involving fine finger movements were most affected. High HAQ-DI and CHFS scores were both associated with high mRSS (r = 0.34, P < 0.0001 and r = 0.35, P < 0.0001, respectively). HAQ-DI was higher in patients with digital ulcers (P = 0.004), pulmonary fibrosis (P = 0.005), cardiac (P = 0.005) and muscle involvement (P = 0.002). As anticipated, HAQ-DI, CHFS, the Functional Assessment of Chronic Illness Therapy and SF36 scores were all highly correlated, in particular the HAQ-DI with the CHFS (ρ = 0.84, P < 0.0001). Worsening HAQ-DI over 12 months was strongly associated with increasing mRSS (ρ = 0.40, P < 0.0001), decreasing hand function (ρ = 0.57, P < 0.0001) and increasing fatigue (ρ =-0.53, P < 0.0001). Conclusion. The European Scleroderma Observational Study highlights the burden of disability in early dcSSc, with high levels of disability and fatigue, associating with the degree of skin thickening (mRSS). Impaired hand function is a major contributor to overall disability.",

author = "S{\'e}bastien Peytrignet and Denton, \{Christopher P.\} and Mark Lunt and Roger Hesselstrand and Luc Mouthon and Alan Silman and Xiaoyan Pan and Edith Brown and L{\'a}szl{\'o} Czirj{\'a}k and Distler, \{J{\"o}rg H.W.\} and Oliver Distler and Kim Fligelstone and Gregory, \{William J.\} and Rachel Ochiel and Madelon Vonk and Codrina Ancu{\c t}a and Ong, \{Voon H.\} and Dominique Farge and Marie Hudson and Marco Matucci-Cerinic and Alexandra Balbir-Gurman and {\O}yvind Midtvedt and Jordan, \{Alison C.\} and Wendy Stevens and Pia Moinzadeh and Hall, \{Frances C.\} and Christian Agard and Anderson, \{Marina E.\} and Elisabeth Diot and Rajan Madhok and Mohammed Akil and Buch, \{Maya H.\} and Lorinda Chung and Nemanja Damjanov and Harsha Gunawardena and Peter Lanyon and Yasmeen Ahmad and Kuntal Chakravarty and S{\o}ren Jacobsen and MacGregor, \{Alexander J.\} and Neil McHugh and Ulf M{\"u}ller-Ladner and Gabriela Riemekasten and Michael Becker and Janet Roddy and Carreira, \{Patricia E.\} and Fauchais, \{Anne Laure\} and Eric Hachulla and Jennifer Hamilton and Murat Inan{\c c} and McLaren, \{John S.\} and \{van Laar\}, \{Jacob M.\} and Sanjay Pathare and Susanna Proudman and Anna Rudin and Joanne Sahhar and Brigitte Coppere and Christine Serratrice and Tom Sheeran and Veale, \{Douglas J.\} and Claire Grange and Trad, \{Georges Selim\} and Herrick, \{Ariane L.\}",

year = "2018",

month = feb,

day = "1",

doi = "10.1093/rheumatology/kex410",

language = "English",

volume = "57",

pages = "370--381",

journal = "Rheumatology (United Kingdom)",

issn = "1462-0324",

publisher = "Oxford University Press",

number = "2",

}

Peytrignet, S, Denton, CP, Lunt, M, Hesselstrand, R, Mouthon, L, Silman, A, Pan, X, Brown, E, Czirják, L, Distler, JHW, Distler, O, Fligelstone, K, Gregory, WJ, Ochiel, R, Vonk, M, Ancuţa, C, Ong, VH, Farge, D, Hudson, M, Matucci-Cerinic, M, Balbir-Gurman, A, Midtvedt, Ø, Jordan, AC, Stevens, W, Moinzadeh, P, Hall, FC, Agard, C, Anderson, ME, Diot, E, Madhok, R, Akil, M, Buch, MH, Chung, L, Damjanov, N, Gunawardena, H, Lanyon, P, Ahmad, Y, Chakravarty, K, Jacobsen, S, MacGregor, AJ, McHugh, N, Müller-Ladner, U, Riemekasten, G, Becker, M, Roddy, J, Carreira, PE, Fauchais, AL, Hachulla, E, Hamilton, J, Inanç, M, McLaren, JS, van Laar, JM, Pathare, S, Proudman, S, Rudin, A, Sahhar, J, Coppere, B, Serratrice, C, Sheeran, T, Veale, DJ, Grange, C, Trad, GS & Herrick, AL 2018, 'Disability, fatigue, pain and their associates in early diffuse cutaneous systemic sclerosis: The European Scleroderma Observational Study', Rheumatology (United Kingdom), vol. 57, no. 2, kex410, pp. 370-381. https://doi.org/10.1093/rheumatology/kex410

Disability, fatigue, pain and their associates in early diffuse cutaneous systemic sclerosis: The European Scleroderma Observational Study. / Peytrignet, Sébastien; Denton, Christopher P.; Lunt, Mark et al.
In: Rheumatology (United Kingdom), Vol. 57, No. 2, kex410, 01.02.2018, p. 370-381.

Research output: Journal Articles › Journal articles › Research › peer-review

TY - JOUR

T1 - Disability, fatigue, pain and their associates in early diffuse cutaneous systemic sclerosis: The European Scleroderma Observational Study

AU - Peytrignet, Sébastien

AU - Denton, Christopher P.

AU - Lunt, Mark

AU - Hesselstrand, Roger

AU - Mouthon, Luc

AU - Silman, Alan

AU - Pan, Xiaoyan

AU - Brown, Edith

AU - Czirják, László

AU - Distler, Jörg H.W.

AU - Distler, Oliver

AU - Fligelstone, Kim

AU - Gregory, William J.

AU - Ochiel, Rachel

AU - Vonk, Madelon

AU - Ancuţa, Codrina

AU - Ong, Voon H.

AU - Farge, Dominique

AU - Hudson, Marie

AU - Matucci-Cerinic, Marco

AU - Balbir-Gurman, Alexandra

AU - Midtvedt, Øyvind

AU - Jordan, Alison C.

AU - Stevens, Wendy

AU - Moinzadeh, Pia

AU - Hall, Frances C.

AU - Agard, Christian

AU - Anderson, Marina E.

AU - Diot, Elisabeth

AU - Madhok, Rajan

AU - Akil, Mohammed

AU - Buch, Maya H.

AU - Chung, Lorinda

AU - Damjanov, Nemanja

AU - Gunawardena, Harsha

AU - Lanyon, Peter

AU - Ahmad, Yasmeen

AU - Chakravarty, Kuntal

AU - Jacobsen, Søren

AU - MacGregor, Alexander J.

AU - McHugh, Neil

AU - Müller-Ladner, Ulf

AU - Riemekasten, Gabriela

AU - Becker, Michael

AU - Roddy, Janet

AU - Carreira, Patricia E.

AU - Fauchais, Anne Laure

AU - Hachulla, Eric

AU - Hamilton, Jennifer

AU - Inanç, Murat

AU - McLaren, John S.

AU - van Laar, Jacob M.

AU - Pathare, Sanjay

AU - Proudman, Susanna

AU - Rudin, Anna

AU - Sahhar, Joanne

AU - Coppere, Brigitte

AU - Serratrice, Christine

AU - Sheeran, Tom

AU - Veale, Douglas J.

AU - Grange, Claire

AU - Trad, Georges Selim

AU - Herrick, Ariane L.

PY - 2018/2/1

Y1 - 2018/2/1

N2 - Objectives. Our aim was to describe the burden of early dcSSc in terms of disability, fatigue and pain in the European Scleroderma Observational Study cohort, and to explore associated clinical features. Methods. Patients completed questionnaires at study entry, 12 and 24 months, including the HAQ disability index (HAQ-DI), the Cochin Hand Function Scale (CHFS), the Functional Assessment of Chronic Illness Therapy-fatigue and the Short Form 36 (SF36). Associates examined included the modified Rodnan skin score (mRSS), current digital ulcers and internal organ involvement. Correlations between 12-month changes were also examined. Results. The 326 patients recruited (median disease duration 11.9 months) displayed high levels of disability [mean (S.D.) HAQ-DI 1.1 (0.83)], with 'grip' and 'activity' being most affected. Of the 18 activities assessed in the CHFS, those involving fine finger movements were most affected. High HAQ-DI and CHFS scores were both associated with high mRSS (r = 0.34, P < 0.0001 and r = 0.35, P < 0.0001, respectively). HAQ-DI was higher in patients with digital ulcers (P = 0.004), pulmonary fibrosis (P = 0.005), cardiac (P = 0.005) and muscle involvement (P = 0.002). As anticipated, HAQ-DI, CHFS, the Functional Assessment of Chronic Illness Therapy and SF36 scores were all highly correlated, in particular the HAQ-DI with the CHFS (ρ = 0.84, P < 0.0001). Worsening HAQ-DI over 12 months was strongly associated with increasing mRSS (ρ = 0.40, P < 0.0001), decreasing hand function (ρ = 0.57, P < 0.0001) and increasing fatigue (ρ =-0.53, P < 0.0001). Conclusion. The European Scleroderma Observational Study highlights the burden of disability in early dcSSc, with high levels of disability and fatigue, associating with the degree of skin thickening (mRSS). Impaired hand function is a major contributor to overall disability.

AB - Objectives. Our aim was to describe the burden of early dcSSc in terms of disability, fatigue and pain in the European Scleroderma Observational Study cohort, and to explore associated clinical features. Methods. Patients completed questionnaires at study entry, 12 and 24 months, including the HAQ disability index (HAQ-DI), the Cochin Hand Function Scale (CHFS), the Functional Assessment of Chronic Illness Therapy-fatigue and the Short Form 36 (SF36). Associates examined included the modified Rodnan skin score (mRSS), current digital ulcers and internal organ involvement. Correlations between 12-month changes were also examined. Results. The 326 patients recruited (median disease duration 11.9 months) displayed high levels of disability [mean (S.D.) HAQ-DI 1.1 (0.83)], with 'grip' and 'activity' being most affected. Of the 18 activities assessed in the CHFS, those involving fine finger movements were most affected. High HAQ-DI and CHFS scores were both associated with high mRSS (r = 0.34, P < 0.0001 and r = 0.35, P < 0.0001, respectively). HAQ-DI was higher in patients with digital ulcers (P = 0.004), pulmonary fibrosis (P = 0.005), cardiac (P = 0.005) and muscle involvement (P = 0.002). As anticipated, HAQ-DI, CHFS, the Functional Assessment of Chronic Illness Therapy and SF36 scores were all highly correlated, in particular the HAQ-DI with the CHFS (ρ = 0.84, P < 0.0001). Worsening HAQ-DI over 12 months was strongly associated with increasing mRSS (ρ = 0.40, P < 0.0001), decreasing hand function (ρ = 0.57, P < 0.0001) and increasing fatigue (ρ =-0.53, P < 0.0001). Conclusion. The European Scleroderma Observational Study highlights the burden of disability in early dcSSc, with high levels of disability and fatigue, associating with the degree of skin thickening (mRSS). Impaired hand function is a major contributor to overall disability.

UR - https://www.scopus.com/pages/publications/85041545966

U2 - 10.1093/rheumatology/kex410

DO - 10.1093/rheumatology/kex410

M3 - Journal articles

C2 - 29207002

AN - SCOPUS:85041545966

SN - 1462-0324

VL - 57

SP - 370

EP - 381

JO - Rheumatology (United Kingdom)

JF - Rheumatology (United Kingdom)

IS - 2

M1 - kex410

ER -

Disability, fatigue, pain and their associates in early diffuse cutaneous systemic sclerosis: The European Scleroderma Observational Study

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