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Rheumatology (Oxford). 2018 Feb 1;57(2):370-381. doi: 10.1093/rheumatology/kex410.

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

Author information

1
Centre for Musculoskeletal Research, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK.
2
Centre for Rheumatology and Connective Tissue Diseases, UCL Division of Medicine, Royal Free Campus, London, UK.
3
Department of Rheumatology, Lund University, Lund, Sweden.
4
Service 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.
5
Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford, UK.
6
Department of Rheumatology and Immunology, Medical Center, University of Pécs, Pécs, Hungary, Erlangen, Germany.
7
Department of Internal Medicine 3, University of Erlangen-Nuremberg, Erlangen, Germany.
8
Department of Rheumatology, University of Zurich, Zurich, Switzerland.
9
Rehabilitation Services, Salford Royal NHS Foundation Trust, Salford, UK.
10
Department of the Rheumatic Diseases, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
11
Rheumatology 2 Department, Clinical Rehabilitation Hospital, 'Grigore T. Popa' University of Medicine and Pharmacy, Iasi, Romania.
12
Unité Clinique de Médecine Interne, Maladies Auto-immunes et Pathologie Vasculaire, Hôpital Saint-Louis, Paris, France.
13
Lady Davis Institute, Jewish General Hospital, Montreal, Canada.
14
Department of Medicine, McGill University, Montreal, Canada.
15
Department Experimental and Clinical Medicine, Division Rheumatology AOUC, University of Florence, Florence, Italy.
16
B. Shine Rheumatology Unit, Rambam Heath Care Campus, Rappaport Faculty of Medicine, Technion, Haifa, Israel.
17
Rheumatology Unit, Oslo University Hospital Rikshospitalet, Oslo, Norway.
18
Queen Elizabeth Hospital Birmingham, UHB Foundation Trust, Birmingham, UK.
19
Rheumatology Unit, St Vincent's Hospital, Melbourne, Australia.
20
Department for Dermatology, University of Cologne, Köln, Germany.
21
Department of Clinical Medicine, Cambridge University NHS Hospital Foundation Trust, Cambridge, UK.
22
Department of Internal Medicine, Hôtel-Dieu Hospital, University of Nantes, Nantes, France.
23
University of Liverpool, Aintree University Hospital, Liverpool, UK.
24
Service de Médecine Interne, Hôpital Bretonneau Tours, Tours, France.
25
Centre for Rheumatic Diseases, Royal Infirmary, Glasgow, UK.
26
Department of Rheumatology, Sheffield Teaching Hospitals, Sheffield, UK.
27
Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK.
28
NIHR Leeds Musculoskeletal Biomedical Research Unit, Leeds Teaching Hospitals NHS Trust, Leeds, UK.
29
Division of Immunology and Rheumatology, Stanford University, Stanford, CA, USA.
30
Institute of Rheumatology, University of Belgrade School of Medicine, Belgrade, Serbia.
31
Clinical and Academic Rheumatology, North Bristol NHS Trust, Bristol, UK.
32
Rheumatology, Nottingham University Hospitals NHS Trust, Nottingham, UK.
33
Rheumatology, Nottingham NHS Treatment Centre, Nottingham, UK.
34
Peter Maddison Rheumatology Centre, Llandudno Hospital, Llandudno, UK.
35
Department of Rheumatology, Queen's Hospital, Romford, UK.
36
Copenhagen Lupus and Vasculitis Clinic, Center for Rheumatology and Spine Diseases, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark.
37
Department of Medicine, Norwich Medical School, University of East Anglia, Norwich, UK.
38
Department of Rheumatology, Royal National Hospital for Rheumatic Diseases, Bath, UK.
39
Department of Rheumatology and Clinical Immunology, Justus-Liebig University Giessen, Kerckhoff Klinik, Bad Nauheim, Germany.
40
Department of Rheumatology, University of Lübeck, Lübeck, Berlin, Germany.
41
Department of Rheumatology and Clinical Immunology, University Hospital Charité Berlin, Berlin, Germany.
42
Department of Rheumatology, Royal Perth Hospital, Perth, Australia.
43
Servicio de Reumatologia, Hospital Universitario 12 de Octubre, Madrid, Spain.
44
Internal Medicine Unit, Limoges University Hospital, Limoges, France.
45
Centre National de Référence Maladies Systémiques et Auto-immunes Rares, Département de Médecine Interne et Immunologie Clinique, Université de Lille, Lille, France.
46
Department of Rheumatology, Gateshead Hospitals Foundation Trust, Gateshead, UK.
47
Department of Internal Medicine, Division of Rheumatology, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey.
48
Fife Rheumatic Diseases Unit, Whyteman's Brae Hospital, Kirkcaldy, Scotland, UK.
49
Department of Rheumatology and Clinical Immunology, UMC Utrecht, Utrecht, the Netherlands.
50
Rheumatology, James Cook University Hospital, Middlesbrough, UK.
51
Rheumatology Unit, Royal Adelaide Hospital.
52
Discipline of Medicine, University of Adelaide, Adelaide, Australia.
53
Department of Rheumatology and Inflammation Research, The Sahlgrenska Academy at Gothenburg University, Gothenburg, Sweden.
54
Monash Centre for Inflammatory Diseases, Monash University, Melbourne, Australia.
55
Department of Internal Medicine, Hôpital Edouard Herriot, Lyon, France.
56
Department of Internal Medicine, Foundation Hospital Saint Joseph, Marseille, France.
57
Department of Rheumatology, Cannock Chase Hospital, Cannock, UK.
58
Rheumatology, St Vincent's University Hospital, Dublin, Ireland.
59
Department of Internal Medicine 69310, Centre Hospitalier Lyon Sud, Pierre-Bénite, Lyon, France.
60
Internal Medecine, Ambroise Paré Hospital, Boulogne, Billancourt, France.
61
Salford Royal NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK.
62
NIHR Manchester Musculoskeletal Biomedical Research Unit, Central Manchester NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, UK.

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 (ρ = 0.34, P < 0.0001 and ρ = 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.

KEYWORDS:

disability; early diffuse cutaneous systemic sclerosis; fatigue; hand function; pain

PMID:
29207002
PMCID:
PMC5850714
DOI:
10.1093/rheumatology/kex410
[Indexed for MEDLINE]
Free PMC Article

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