PMID- 29207002 OWN - NLM STAT- MEDLINE DCOM- 20180518 LR - 20181113 IS - 1462-0332 (Electronic) IS - 1462-0324 (Print) IS - 1462-0324 (Linking) VI - 57 IP - 2 DP - 2018 Feb 1 TI - Disability, fatigue, pain and their associates in early diffuse cutaneous systemic sclerosis: the European Scleroderma Observational Study. PG - 370-381 LID - 10.1093/rheumatology/kex410 [doi] 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 (rho = 0.34, P < 0.0001 and rho = 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 (rho = 0.84, P < 0.0001). Worsening HAQ-DI over 12 months was strongly associated with increasing mRSS (rho = 0.40, P < 0.0001), decreasing hand function (rho = 0.57, P < 0.0001) and increasing fatigue (rho = -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. CI - (c) The Author 2017. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com FAU - Peytrignet, Sebastien AU - Peytrignet S AD - Centre for Musculoskeletal Research, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. FAU - Denton, Christopher P AU - Denton CP AD - Centre for Rheumatology and Connective Tissue Diseases, UCL Division of Medicine, Royal Free Campus, London, UK. FAU - Lunt, Mark AU - Lunt M AD - Centre for Musculoskeletal Research, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. FAU - Hesselstrand, Roger AU - Hesselstrand R AD - Department of Rheumatology, Lund University, Lund, Sweden. FAU - Mouthon, Luc AU - Mouthon L AD - Service de Medecine Interne, Hopital Cochin, Centre de Reference pour les Vascularites Necrosantes et la Sclerodermie Systemique, Universite Paris Descartes, Assistance Publique-Hopitaux de Paris (AP-HP), Paris, France. FAU - Silman, Alan AU - Silman A AD - Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford, UK. FAU - Pan, Xiaoyan AU - Pan X AD - Centre for Musculoskeletal Research, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. FAU - Brown, Edith AU - Brown E AD - Centre for Musculoskeletal Research, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. FAU - Czirjak, Laszlo AU - Czirjak L AD - Department of Rheumatology and Immunology, Medical Center, University of Pecs, Pecs, Hungary, Erlangen, Germany. FAU - Distler, Jorg H W AU - Distler JHW AD - Department of Internal Medicine 3, University of Erlangen-Nuremberg, Erlangen, Germany. FAU - Distler, Oliver AU - Distler O AD - Department of Rheumatology, University of Zurich, Zurich, Switzerland. FAU - Fligelstone, Kim AU - Fligelstone K AD - Centre for Rheumatology and Connective Tissue Diseases, UCL Division of Medicine, Royal Free Campus, London, UK. FAU - Gregory, William J AU - Gregory WJ AD - Rehabilitation Services, Salford Royal NHS Foundation Trust, Salford, UK. FAU - Ochiel, Rachel AU - Ochiel R AD - Centre for Rheumatology and Connective Tissue Diseases, UCL Division of Medicine, Royal Free Campus, London, UK. FAU - Vonk, Madelon AU - Vonk M AD - Department of the Rheumatic Diseases, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands. FAU - Ancuta, Codrina AU - Ancuta C AD - Rheumatology 2 Department, Clinical Rehabilitation Hospital, 'Grigore T. Popa' University of Medicine and Pharmacy, Iasi, Romania. FAU - Ong, Voon H AU - Ong VH AD - Centre for Rheumatology and Connective Tissue Diseases, UCL Division of Medicine, Royal Free Campus, London, UK. FAU - Farge, Dominique AU - Farge D AD - Unite Clinique de Medecine Interne, Maladies Auto-immunes et Pathologie Vasculaire, Hopital Saint-Louis, Paris, France. FAU - Hudson, Marie AU - Hudson M AD - Lady Davis Institute, Jewish General Hospital, Montreal, Canada. AD - Department of Medicine, McGill University, Montreal, Canada. FAU - Matucci-Cerinic, Marco AU - Matucci-Cerinic M AD - Department Experimental and Clinical Medicine, Division Rheumatology AOUC, University of Florence, Florence, Italy. FAU - Balbir-Gurman, Alexandra AU - Balbir-Gurman A AD - B. Shine Rheumatology Unit, Rambam Heath Care Campus, Rappaport Faculty of Medicine, Technion, Haifa, Israel. FAU - Midtvedt, Oyvind AU - Midtvedt O AD - Rheumatology Unit, Oslo University Hospital Rikshospitalet, Oslo, Norway. FAU - Jordan, Alison C AU - Jordan AC AD - Queen Elizabeth Hospital Birmingham, UHB Foundation Trust, Birmingham, UK. FAU - Stevens, Wendy AU - Stevens W AD - Rheumatology Unit, St Vincent's Hospital, Melbourne, Australia. FAU - Moinzadeh, Pia AU - Moinzadeh P AD - Department for Dermatology, University of Cologne, Koln, Germany. FAU - Hall, Frances C AU - Hall FC AD - Department of Clinical Medicine, Cambridge University NHS Hospital Foundation Trust, Cambridge, UK. FAU - Agard, Christian AU - Agard C AD - Department of Internal Medicine, Hotel-Dieu Hospital, University of Nantes, Nantes, France. FAU - Anderson, Marina E AU - Anderson ME AD - University of Liverpool, Aintree University Hospital, Liverpool, UK. FAU - Diot, Elisabeth AU - Diot E AD - Service de Medecine Interne, Hopital Bretonneau Tours, Tours, France. FAU - Madhok, Rajan AU - Madhok R AD - Centre for Rheumatic Diseases, Royal Infirmary, Glasgow, UK. FAU - Akil, Mohammed AU - Akil M AD - Department of Rheumatology, Sheffield Teaching Hospitals, Sheffield, UK. FAU - Buch, Maya H AU - Buch MH AD - Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK. AD - NIHR Leeds Musculoskeletal Biomedical Research Unit, Leeds Teaching Hospitals NHS Trust, Leeds, UK. FAU - Chung, Lorinda AU - Chung L AD - Division of Immunology and Rheumatology, Stanford University, Stanford, CA, USA. FAU - Damjanov, Nemanja AU - Damjanov N AD - Institute of Rheumatology, University of Belgrade School of Medicine, Belgrade, Serbia. FAU - Gunawardena, Harsha AU - Gunawardena H AD - Clinical and Academic Rheumatology, North Bristol NHS Trust, Bristol, UK. FAU - Lanyon, Peter AU - Lanyon P AD - Rheumatology, Nottingham University Hospitals NHS Trust, Nottingham, UK. AD - Rheumatology, Nottingham NHS Treatment Centre, Nottingham, UK. FAU - Ahmad, Yasmeen AU - Ahmad Y AD - Peter Maddison Rheumatology Centre, Llandudno Hospital, Llandudno, UK. FAU - Chakravarty, Kuntal AU - Chakravarty K AD - Department of Rheumatology, Queen's Hospital, Romford, UK. FAU - Jacobsen, Soren AU - Jacobsen S AD - Copenhagen Lupus and Vasculitis Clinic, Center for Rheumatology and Spine Diseases, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark. FAU - MacGregor, Alexander J AU - MacGregor AJ AD - Department of Medicine, Norwich Medical School, University of East Anglia, Norwich, UK. FAU - McHugh, Neil AU - McHugh N AD - Department of Rheumatology, Royal National Hospital for Rheumatic Diseases, Bath, UK. FAU - Muller-Ladner, Ulf AU - Muller-Ladner U AD - Department of Rheumatology and Clinical Immunology, Justus-Liebig University Giessen, Kerckhoff Klinik, Bad Nauheim, Germany. FAU - Riemekasten, Gabriela AU - Riemekasten G AD - Department of Rheumatology, University of Lubeck, Lubeck, Berlin, Germany. FAU - Becker, Michael AU - Becker M AD - Department of Rheumatology and Clinical Immunology, University Hospital Charite Berlin, Berlin, Germany. FAU - Roddy, Janet AU - Roddy J AD - Department of Rheumatology, Royal Perth Hospital, Perth, Australia. FAU - Carreira, Patricia E AU - Carreira PE AD - Servicio de Reumatologia, Hospital Universitario 12 de Octubre, Madrid, Spain. FAU - Fauchais, Anne Laure AU - Fauchais AL AD - Internal Medicine Unit, Limoges University Hospital, Limoges, France. FAU - Hachulla, Eric AU - Hachulla E AD - Centre National de Reference Maladies Systemiques et Auto-immunes Rares, Departement de Medecine Interne et Immunologie Clinique, Universite de Lille, Lille, France. FAU - Hamilton, Jennifer AU - Hamilton J AD - Department of Rheumatology, Gateshead Hospitals Foundation Trust, Gateshead, UK. FAU - Inanc, Murat AU - Inanc M AD - Department of Internal Medicine, Division of Rheumatology, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey. FAU - McLaren, John S AU - McLaren JS AD - Fife Rheumatic Diseases Unit, Whyteman's Brae Hospital, Kirkcaldy, Scotland, UK. FAU - van Laar, Jacob M AU - van Laar JM AD - Department of Rheumatology and Clinical Immunology, UMC Utrecht, Utrecht, the Netherlands. FAU - Pathare, Sanjay AU - Pathare S AD - Rheumatology, James Cook University Hospital, Middlesbrough, UK. FAU - Proudman, Susanna AU - Proudman S AD - Rheumatology Unit, Royal Adelaide Hospital. AD - Discipline of Medicine, University of Adelaide, Adelaide, Australia. FAU - Rudin, Anna AU - Rudin A AD - Department of Rheumatology and Inflammation Research, The Sahlgrenska Academy at Gothenburg University, Gothenburg, Sweden. FAU - Sahhar, Joanne AU - Sahhar J AD - Monash Centre for Inflammatory Diseases, Monash University, Melbourne, Australia. FAU - Coppere, Brigitte AU - Coppere B AD - Department of Internal Medicine, Hopital Edouard Herriot, Lyon, France. FAU - Serratrice, Christine AU - Serratrice C AD - Department of Internal Medicine, Foundation Hospital Saint Joseph, Marseille, France. FAU - Sheeran, Tom AU - Sheeran T AD - Department of Rheumatology, Cannock Chase Hospital, Cannock, UK. FAU - Veale, Douglas J AU - Veale DJ AD - Rheumatology, St Vincent's University Hospital, Dublin, Ireland. FAU - Grange, Claire AU - Grange C AD - Department of Internal Medicine 69310, Centre Hospitalier Lyon Sud, Pierre-Benite, Lyon, France. FAU - Trad, Georges-Selim AU - Trad GS AD - Internal Medecine, Ambroise Pare Hospital, Boulogne, Billancourt, France. FAU - Herrick, Ariane L AU - Herrick AL AD - Centre for Musculoskeletal Research, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK. AD - Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. AD - NIHR Manchester Musculoskeletal Biomedical Research Unit, Central Manchester NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK. LA - eng PT - Journal Article PT - Observational Study PT - Research Support, Non-U.S. Gov't PL - England TA - Rheumatology (Oxford) JT - Rheumatology (Oxford, England) JID - 100883501 SB - IM MH - Adult MH - Cost of Illness MH - *Disability Evaluation MH - Europe MH - Fatigue/etiology/*physiopathology MH - Female MH - Fingers MH - Hand Strength MH - Health Surveys MH - Humans MH - Male MH - Pain/etiology/*physiopathology MH - Prospective Studies MH - Scleroderma, Diffuse/complications/*physiopathology MH - *Severity of Illness Index MH - Skin Ulcer/etiology/physiopathology PMC - PMC5850714 OTO - NOTNLM OT - disability OT - early diffuse cutaneous systemic sclerosis OT - fatigue OT - hand function OT - pain EDAT- 2017/12/06 06:00 MHDA- 2018/05/19 06:00 PMCR- 2017/11/30 CRDT- 2017/12/06 06:00 PHST- 2017/01/30 00:00 [received] PHST- 2017/12/06 06:00 [pubmed] PHST- 2018/05/19 06:00 [medline] PHST- 2017/12/06 06:00 [entrez] PHST- 2017/11/30 00:00 [pmc-release] AID - 4675178 [pii] AID - kex410 [pii] AID - 10.1093/rheumatology/kex410 [doi] PST - ppublish SO - Rheumatology (Oxford). 2018 Feb 1;57(2):370-381. doi: 10.1093/rheumatology/kex410.