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Interstitial lung disease in systemic sclerosis: data from the spanish scleroderma study group

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Abstract

Objectives

To evaluate the clinical characteristics of patients with interstitial lung disease (ILD) in the setting of a large cohort of systemic sclerosis (SSc) patients, and to analyse the differences according to the SSc subtype (following the modification of classification criteria of the American College of Rheumatology for SSc proposed by LeRoy and Medsger), factors are associated with moderate-to-severe impairment of lung function, as well as mortality and causes of death.

Methods

A descriptive study was performed, using the available data from the Spanish Scleroderma Study Group.

Results

Twenty-one referral centers participated in the registry. By April 2014, 1374 patients with SSc had been enrolled, and 595 of whom (43%) had ILD: 316 (53%) with limited cutaneous SSc (lcSSc), 240 (40%) with diffuse cutaneous SSc (dcSSc), and 39 (7%) with SSc sine scleroderma (ssSSc). ILD in the lcSSc and the ssSSc subsets tended to develop later, and showed a less impaired forced vital capacity (FVC) and a ground glass pattern on high-resolution computed tomography (HRCT) less frequently, compared with the dcSSc subset. Factors related to an FVC < 70% of predicted in the multivariate analysis were: dcSSc, positivity to anti-topoisomerase I antibodies, a ground glass pattern on HCRT, an active nailfold capillaroscopy pattern, lower DLco, older age at symptoms onset, and longer time between symptoms onset and ILD diagnosis. Finally, SSc-associated mortality and ILD-related mortality were highest in dcSSc patients, whereas that related to pulmonary arterial hypertension was highest in those with lcSSc-associated ILD.

Conclusions

Our study indicates that ILD constitutes a remarkable complication of SSc with significant morbidity and mortality, which should be borne in mind in all three subgroups (lcSSc, dcSSc, and ssSSc).

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Acknowledgements

We gratefully acknowledge all investigators who form part of the RESCLE Registry. This project was possible thanks to an educational unrestricted scholarship granted by Laboratorios Actelion. We also thank the RESCLE Registry Coordinating Center, S & H Medical Science Service, for their quality control data, logistic and administrative support and Prof. Salvador Ortiz, Universidad Autónoma de Madrid and Statistical Advisor S& H Medical Science Service for the statistical analysis of the data presented in this paper

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Authors and Affiliations

Authors

Contributions

DSC, CPSA: Substantial contributions to study conception and design. DSC, CPSA, NOC, JLC, VFP, RRF, CTV, GEG, DCA, MVEA, MRR, MF, JJRB, LTM, MRC, AMB, XPS: Substantial contributions to acquisition of data. DSC, CPSA: Substantial contributions to analysis and interpretation of data. DSC, CPSA, NOC: Drafting the article or revising it critically for important intellectual content. DSC, CPSA, VFP, CTV: Final approval of the version of the article to be published. Other critical study activities (specify).

Corresponding author

Correspondence to N. Ortego-Centeno.

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Conflict of interest

The authors declare that they have no competing interest.

Research involving animal participants

This article does not contain any studies with animals performed by any of the authors.

Ethical standards

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Hospital

Reference, file number or approval date

Hospital Universitario Vall d´Hebron (Barcelona)

30/11/2010

Corporación Sanitaria Universitaria Parc Taulí (Barcelona)

2011518

Hospital Universitario Cruces (Bizkaia)

3/11

Hospital Universitario San Cecilio (Granada)

21/01/2011

Hospital Universitario Virgen del Rocio (Sevilla)

3/11

Hospital Universitario Miguel Servet (Zaragoza)

19/2011

Hospital Universitario Clínic (Barcelona)

2011/6413

Hospital Universitario Central de Asturias

09/2011

Hospital Universitario La Paz (Madrid)

3/2011

Hospital Campus de la Salud. Complejo Universitario de Granada

01/10/2012

Hospital Universitario y Politécnico La Fe (Valencia)

2013/0152

Hospital Universitario de Bellvitge (Barcelona)

09/13

Hospital Mutua Terrassa (Barcelona)

28/03/2012

Hospital Universitario Nuestra Señora de la Candelaria (Santa Cruz de Tenerife)

02/02/2012

Complejo Hospitalario Universitario de Vigo (Pontevedra)

2012/046

Hospital de Manises (Valencia)

25/05/2011

Hospital de Cabueñes (Asturias)

24/2013

Hospital Vega Baja de Orihuela (Alicante)

20/12/2011

Hospital Monte San Isidro (León)

24/01/2012

Hospital Clínico Universitario “Lozano Blesa” (Zaragoza)

CP15/2013

Hospital d’Igualada (Barcelona)

13/14

Hospital Universitari Sant Joan de Reus (Tarragona)

13-11-28/11proj2

Complexo Hospitalario Universitario de Ferrol (A Coruña)

01/12/2014

Hospital Universitario Fundación Alcorcón (Madrid)

02/12/2014

Hospital Universitario Reina Sofía (Córdoba)

235/2691

Consorci Hospitalari de Vic (Barcelona)

2014869

Hospital General San Jorge (Huesca)

06/2015

Complejo Asistencial Universitario de Salamanca

01/12/2015

Hospital Universitario Rey Juan Carlos (Madrid)

17/15

Hospital Universitario Ramón y Cajal (Madrid)

25/09/2017

Hospital de Sagunto (Valencia)

26/04/2017

Hospital Universitario Virgen de Valme (Sevilla)

0572-N-17

Appendix: RESCLE Registry members

Appendix: RESCLE Registry members

Callejas JL, Castillo MJ, Colunga D, Egurbide MV, de Escalante B, Espinosa G, Fonollosa V, Freire M, Guillén A, Iniesta N, Marí B, Marín A, Márquez-Fernández V, Ortego-Centeno N, Pla X, Ríos-Blanco JJ, Ríos-Fernández R, Rivera A, Robles A, Rodero MM, Rodríguez-Carballeira M, Rubio M, Sánchez-Cano D, Simeón CP, Soto A, Todolí JA, Tolosa C, Trapiella L, Vargas JA.

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Sánchez-Cano, D., Ortego-Centeno, N., Callejas, J.L. et al. Interstitial lung disease in systemic sclerosis: data from the spanish scleroderma study group. Rheumatol Int 38, 363–374 (2018). https://doi.org/10.1007/s00296-017-3916-x

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  • DOI: https://doi.org/10.1007/s00296-017-3916-x

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