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Ann Rheum Dis. 2017 Nov;76(11):1897-1905. doi: 10.1136/annrheumdis-2017-211448. Epub 2017 Aug 23.

Mapping and predicting mortality from systemic sclerosis.

Author information

1
Rheumatology A department, Paris Descartes University, INSERM U1016, Sorbonne Paris Cité, Cochin Hospital, Paris, France.
2
Department of Cardiology, Paris XIII University, INSERM UMR S-942, Bobigny Hospital, Paris, France.
3
Unit of Clinical Research, Paris Seine Saint Denis University, Bobigny, France.
4
Department of Internal Medicine, Hôpital Claude Huriez, University Lille Nord-de-France, Lille Cedex, Lille, France.
5
B Shine Rheumatology Unit, Rambam Health Care Campus, Rappaport Faculty of Medicine, Technion-Institute of Technology, Haifa, Israel.
6
Department of Rheumatology, University of Lübeck, Lübeck, Germany.
7
UO Reumatologia ed Immunologia Clinica Spedali Civili Brescia, Brescia, Italy.
8
Servicio de Reumatologia, Hospital Universitario 12 de Octubre, Madrid, Spain.
9
Department of Clinical and Experimental Medicine, 'F-Magrassi' II, Naples, Italy.
10
Rheumatology Unit, Department of Medicine, University of Padova, Padova, Italy.
11
Department of Dermatology, University Hospital of Copenhagen, Hospital Bispebjerg, Copenhagen, Denmark.
12
Department of Immunology and Rheumatology, University of Pécs, Pécs, Hungary.
13
Chris Hani Baragwanath Academic Hospital University of the Witwatersrand, Johannesburg, South Africa.
14
Department of Rheumatology and Clinical Immunology, Justus-Liebig University Giessen, Kerckhoff Clinic, Bad Nauheim, Germany.
15
Rheumatology Unit, Department of Medicine, University of Verona, Verona, Italy.
16
Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland.
17
Interdisciplinary Department of Medicine-Rheumatology Unit, Policlinico, University of Bari, Bari, Italy.
18
VA Nasonova Institute of Rheumatology, Moscow, Russian Federation.
19
Department of Clinical Sciences Lund, Section of Rheumatology, Lund University, Skåne University Hospital, Lund, Sweden.
20
Institute of Rheumatology, 1st Medical School, Charles University, Praha, Czech Republic.
21
Clinica Medica, Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle Marche, Ancona, Italy.
22
Institute of Rheumatology, University of Belgrade Medical School, Belgrade, Serbia.
23
Rheumatology Department, Hospitais da Universidade, Coimbra, Portugal.
24
Department of Internal Medicine and Medical Specialities, 'Sapienza', University of Rome, Italy, Rome, Italy.
25
Department of Internal Medicine and Rheumatology Clinic, Ion Cantacuzino Clinical Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
26
Department of Internal Medicine, Division of Rheumatology, University of Debrecen, Debrecen, Hungary.
27
Department of Rheumatology, Basel University, Unispital Basel, Basel, Switzerland.
28
Department of Dermatology, University Hospital Cologne, Cologne, Germany.
29
Department of Internal Medicine, Clinical Hospital of Split, Split, Croatia.
30
Department of Rheumatology, University of Ghent, Ghent, Belgium.
31
Rheumatology Division, Clinics Hospital, Federal University of Paraná, Curitiba, Brazil.
32
Unita Operativa e Cattedra di Reumatologia, IRCCS Policlinico S Matteo, Pavia, Italy.
33
Department of Rheumatology, St Maria Hospital, Carol Davila, University of Medicine and Pharmacy, Bucharest, Romania.
34
Dipartimento e Cattedra di Reumatologia, Università degli Studi di Milano, Istituto Ortopedico 'Gaetano Pini', Milano, Italy.
35
Department of Pathophysiology, Medical School, National University and Kapodistrian of Athens, Athens, Greece.
36
Institute for Prevention, Treatment and Rehabilitation of Rheumatic and Cardiovascular Diseases, Niska Banja, Serbia and Montenegro.
37
Dipartimento di Medicina Clinica, Centro per la Sclerosi Sistemica, Università La Sapienza, Policlinico Umberto I, Roma, Italy.
38
Department of Rheumatology, Marienhospital Stuttgart, Stuttgart, Germany.
39
Department of Internal Medicine 3, University Hospital Erlangen, Erlangen, Germany.
40
Department of Medicine, Unit of Internal Medicine, Valence cedex, France.
41
Medizinische Klinik III, University Hospital of Bonn, Bonn, Germany.
42
Rheumatology Unit, University Hospital of Cagliari, Monserrato, Italy.
43
Division of Rheumatology and Department of Development and Regeneration, University Hospital Leuven and Laboratory Tissue Homeostasis and Disease, Leuven, Belgium.
44
Department of Rheumatology and Clinical Immunology, Internal Medicine, KBC Rijeka, Rijeka, Croatia.
45
Research Laboratory and Division of Rheumatology Department of Internal Medicine, University of Genova, Genova, Italy.
46
Department of Internal Medicine, Hôpital Cochin, Paris, France.
47
Medizinische Universitätsklinik, Abt II (Onkologie, Hämatologie, Rheumatologie, Immunologie, Pulmonologie), Tübingen, Germany.
48
Department of Immunology and Allergy, University Hospital, Geneva, Switzerland.
49
Rheuma Clinic Av Carlos Gomes Porto Alegre, Porto Alegre, Brazil.
50
Rheumatology Unit, Waikato University Hospital, Hamilton City, Hamilton, New Zealand.
51
Department of Rheumatology, University of Medicine and Pharmacy 'Iuliu Hatieganu' Cluj, Cluj-Napoca, Romania.
52
Department of Medicine, University of Otago, Christchurch, New Zealand.
53
Division of Clinical Immunology and Rheumatology, Department of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia.
54
Rheumatology Granollers General Hospital, Barcelona, Spain.
55
Division of Rheumatology and Clinical Immunology, Humanitas Clinical and Research Center, Rozzano, Italy.
56
Department of Rheumatology, University of Marmara, Istanbul, Turkey.
57
Servicio de Reumatología, Hospital Ramon Y Cajal, Madrid, Spain.
58
Department of Rheumatology, University Hospital of Strasbourg-, Hôpital de Hautepierre, Service de Rhumatologie, Strasbourg, France.
59
Department of Dermatology, Charles University and General University Hospital in Prague, Prague, Czech Republic.
60
Department of Rheumatology and Clinical Immunology, James Cook University Hospital, Middlesbrough, UK.
61
Reumatologia e Inmunologia Clinica, Hospital Regional Universitario Jose Ma Cabral y Baez, Clinica Corominas, Santiago, Dominican Republic.
62
Rheumatology Department, Hospital Universitario Madrid Norte Sanchinarro, Madrid, Spain.
63
Department of Dermatology and Allergy of the TU Munich, Munich, Germany.
64
Department of Rheumatology and Collagenopathies, Osteoarticular Diseases and Osteoporosis Centre, Pharmacology and Clinical Pharmacological Research Centre, School of Medicine-University of Buenos Aires, Ramos Mejía Hospital, Buenos Aires, Argentina.
65
Department of Rheumatology, Hospital Universitario Dr Peset, Valencia, Spain.
66
Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland.
67
INSERM, CépiDc, Le Kremlin- Bicêtre, Le Kremlin-Bicêtre, France.
68
Department of Experimental and Clinical Medicine, Section of Internal Medicine and Division of Rheumatology, Azienda Ospedaliero-Universitaria Careggi (AOUC), University of Florence, Florence, Italy.

Abstract

OBJECTIVES:

To determine the causes of death and risk factors in systemic sclerosis (SSc).

METHODS:

Between 2000 and 2011, we examined the death certificates of all French patients with SSc to determine causes of death. Then we examined causes of death and developed a score associated with all-cause mortality from the international European Scleroderma Trials and Research (EUSTAR) database. Candidate prognostic factors were tested by Cox proportional hazards regression model by single variable analysis, followed by a multiple variable model stratified by centres. The bootstrapping technique was used for internal validation.

RESULTS:

We identified 2719 French certificates of deaths related to SSc, mainly from cardiac (31%) and respiratory (18%) causes, and an increase in SSc-specific mortality over time. Over a median follow-up of 2.3 years, 1072 (9.6%) of 11 193 patients from the EUSTAR sample died, from cardiac disease in 27% and respiratory causes in 17%. By multiple variable analysis, a risk score was developed, which accurately predicted the 3-year mortality, with an area under the curve of 0.82. The 3-year survival of patients in the upper quartile was 53%, in contrast with 98% in the first quartile.

CONCLUSION:

Combining two complementary and detailed databases enabled the collection of an unprecedented 3700 deaths, revealing the major contribution of the cardiopulmonary system to SSc mortality. We also developed a robust score to risk-stratify these patients and estimate their 3-year survival. With the emergence of new therapies, these important observations should help caregivers plan and refine the monitoring and management to prolong these patients' survival.

KEYWORDS:

cardiovascular disease; epidemiology; pulmonary fibrosis; systemic sclerosis

PMID:
28835464
DOI:
10.1136/annrheumdis-2017-211448
[Indexed for MEDLINE]

Conflict of interest statement

Competing interests: OD reports personal fees from 4D Science, grants and personal fees from Actelion, personal fees from Active Biotech, grants and personal fees from Bayer, personal fees from Biogen Idec, personal fees from BMS, grants and personal fees from Boehringer Ingelheim, personal fees from ChemomAb, personal fees from EpiPharm, personal fees from EspeRare Foundation, personal fees from Genentech/Roche, personal fees from GSK, personal fees from Inventiva, personal fees from Lilly, personal fees from Medac, personal fees from Mepha, personal fees from MedImmune, personal fees from Pharmacyclics, grants and personal fees from Pfizer, grants and personal fees from Sanofi, personal fees from Serodapharm, personal fees from Sinoxa, personal fees from AbbVie, personal fees from iQone Healthcare, outside the submitted work. In addition, OD has a patent mir-29 for the treatment of systemic sclerosis licensed. FI reports personal fees from AbbVie, personal fees from BMS, personal fees from MSD, personal fees from Novartis, outside the submitted work. JHWD reports personal fees from Actelion, grants and personal fees from Anamar, grants and personal fees from Bayer Pharma, grants and personal fees from Boehringer Ingelheim, grants from Celgene, personal fees from Galapagos, grants from GSK, grants and personal fees from Inventiva, personal fees from Pfizer, grants and personal fees from UCB, grants from Novartis, other from 4D Science, outside the submitted work. JvL reports personal fees from Pfizer, grants and personal fees from MSD, personal fees from Eli Lilly, personal fees from BMS, personal fees from Roche, outside the submitted work. Other coauthors have nothing to disclose.

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