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Clinical Rheumatology

, Volume 37, Issue 4, pp 999–1009 | Cite as

First clinical symptom as a prognostic factor in systemic sclerosis: results of a retrospective nationwide cohort study

  • Manuel Rubio-Rivas
  • Xavier Corbella
  • Melany Pestaña-Fernández
  • Carles Tolosa-Vilella
  • Alfredo Guillen-del Castillo
  • Dolores Colunga-Argüelles
  • Luis Trapiella-Martínez
  • Nerea Iniesta-Arandia
  • María Jesús Castillo-Palma
  • Luis Sáez-Comet
  • María Victoria Egurbide-Arberas
  • Norberto Ortego-Centeno
  • Mayka Freire
  • Jose Antonio Vargas-Hitos
  • Juan José Ríos-Blanco
  • Jose Antonio Todolí-Parra
  • Mónica Rodríguez-Carballeira
  • Adela Marín-Ballvé
  • Pablo Segovia-Alonso
  • Xavier Pla-Salas
  • Ana Belén Madroñero-Vuelta
  • Manuel Ruiz-Muñoz
  • Vicent Fonollosa-Pla
  • Carmen Pilar Simeón-Aznar
  • on behalf of RESCLE investigators, Autoimmune Diseases Study Group (GEAS)
  • E Callejas Moraga
  • E. Calvo
  • C. Carbonell
  • M. J. Castillo
  • A. J. Chamorro
  • D. Colunga
  • X. Corbella
  • M. V. Egurbide
  • G. Espinosa
  • V. Fonollosa
  • M. Freire
  • F. J. García Hernández
  • R. González León
  • A. Guillén del Castillo
  • N. Iniesta
  • R. Lorenzo
  • A. B. Madroñero
  • B. Marí
  • A. Marín
  • N. Ortego-Centeno
  • M. Pérez Conesa
  • M. Pestaña
  • X. Pla
  • J. J. Ríos Blanco
  • M. Rodríguez Carballeira
  • M. Rubio Rivas
  • M. Ruiz Muñoz
  • L. Sáez Comet
  • P. Segovia
  • C. P. Simeón
  • A. Soto
  • E. Tarí
  • J. A. Todolí
  • C. Tolosa
  • L. Trapiella
  • J. A. Vargas Hitos
  • G. Verdejo
Original Article

Abstract

The objective of the study is to determine the importance of the mode of onset as prognostic factor in systemic sclerosis (SSc). Data were collected from the Spanish Scleroderma Registry (RESCLE), a nationwide retrospective multicenter database created in 2006. As first symptom, we included Raynaud’s phenomenon (RP), cutaneous sclerosis, arthralgia/arthritis, puffy hands, interstitial lung disease (ILD), pulmonary arterial hypertension (PAH), and digestive hypomotility. A total of 1625 patients were recruited. One thousand three hundred forty-two patients (83%) presented with RP as first symptom and 283 patients (17%) did not. Survival from first symptom in those patients with RP mode of onset was higher at any time than those with onset as non-Raynaud’s phenomenon: 97 vs. 90% at 5 years, 93 vs. 82% at 10 years, 83 vs. 62% at 20 years, and 71 vs. 50% at 30 years (p < 0.001). In multivariate analysis, factors related to mortality were older age at onset, male gender, dcSSc subset, ILD, PAH, scleroderma renal crisis (SRC), heart involvement, and the mode of onset with non-Raynaud’s phenomenon, especially in the form of puffy hands or pulmonary involvement. The mode of onset should be considered an independent prognostic factor in systemic sclerosis and, in particular, patients who initially present with non-Raynaud’s phenomenon may be considered of poor prognosis.

Keywords

Onset Prognosis Raynaud Scleroderma Systemic sclerosis 

Notes

Acknowledgements

We would like to thank the RESCLE Registry Coordinating Center, S & H Medical Science Service, for their quality control data, logistic and administrative support, and Prof. Salvador Ortiz, from the Universidad Autónoma de Madrid, and Statistical Advisor of S & H Medical Science Service, for his support in the statistical analysis of the data presented in the present study.

Funding information

We gratefully acknowledge all investigators who form part of the nationwide Spanish Scleroderma Registry (RESCLE). This project was possible thanks to an unrestricted educational scholarship granted by Actelion Laboratories.

Compliance with ethical standards

Disclosures

None.

Supplementary material

10067_2017_3936_MOESM1_ESM.docx (15 kb)
ESM 1 (DOCX 13 kb)

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Copyright information

© International League of Associations for Rheumatology (ILAR) 2017

Authors and Affiliations

  • Manuel Rubio-Rivas
    • 1
  • Xavier Corbella
    • 1
    • 2
  • Melany Pestaña-Fernández
    • 1
  • Carles Tolosa-Vilella
    • 3
  • Alfredo Guillen-del Castillo
    • 4
  • Dolores Colunga-Argüelles
    • 5
  • Luis Trapiella-Martínez
    • 6
  • Nerea Iniesta-Arandia
    • 7
  • María Jesús Castillo-Palma
    • 8
  • Luis Sáez-Comet
    • 9
  • María Victoria Egurbide-Arberas
    • 10
  • Norberto Ortego-Centeno
    • 11
  • Mayka Freire
    • 12
  • Jose Antonio Vargas-Hitos
    • 13
  • Juan José Ríos-Blanco
    • 14
  • Jose Antonio Todolí-Parra
    • 15
  • Mónica Rodríguez-Carballeira
    • 16
  • Adela Marín-Ballvé
    • 17
  • Pablo Segovia-Alonso
    • 18
  • Xavier Pla-Salas
    • 19
  • Ana Belén Madroñero-Vuelta
    • 20
  • Manuel Ruiz-Muñoz
    • 21
  • Vicent Fonollosa-Pla
    • 4
  • Carmen Pilar Simeón-Aznar
    • 4
  • on behalf of RESCLE investigators, Autoimmune Diseases Study Group (GEAS)
  • E Callejas Moraga
  • E. Calvo
  • C. Carbonell
  • M. J. Castillo
  • A. J. Chamorro
  • D. Colunga
  • X. Corbella
  • M. V. Egurbide
  • G. Espinosa
  • V. Fonollosa
  • M. Freire
  • F. J. García Hernández
  • R. González León
  • A. Guillén del Castillo
  • N. Iniesta
  • R. Lorenzo
  • A. B. Madroñero
  • B. Marí
  • A. Marín
  • N. Ortego-Centeno
  • M. Pérez Conesa
  • M. Pestaña
  • X. Pla
  • J. J. Ríos Blanco
  • M. Rodríguez Carballeira
  • M. Rubio Rivas
  • M. Ruiz Muñoz
  • L. Sáez Comet
  • P. Segovia
  • C. P. Simeón
  • A. Soto
  • E. Tarí
  • J. A. Todolí
  • C. Tolosa
  • L. Trapiella
  • J. A. Vargas Hitos
  • G. Verdejo
  1. 1.Department of Internal MedicineHospital Universitario de Bellvitge-IDIBELL, L’Hospitalet de LlobregatBarcelonaSpain
  2. 2.Faculty of Medicine and Health SciencesUniversitat Internacional de CatalunyaBarcelonaSpain
  3. 3.Department of Internal MedicineCorporación Sanitaria Universitaria Parc TaulíBarcelonaSpain
  4. 4.Autoimmune Unit, Department of Internal MedicineHospital Universitario Vall d’HebronBarcelonaSpain
  5. 5.Department of Internal MedicineHospital Universitario Central de AsturiasOviedoSpain
  6. 6.Department of Internal MedicineHospital de CabueñesGijónSpain
  7. 7.Department of Autoimmune Diseases, Institut Clinic de Medicina i DermatologíaHospital ClínicBarcelonaSpain
  8. 8.Collagenosis and Pulmonary Hypertension Unit, Department of Internal MedicineHospital Universitario Virgen del RocíoSevillaSpain
  9. 9.Department of Internal MedicineHospital Universitario Miguel ServetZaragozaSpain
  10. 10.Department of Internal MedicineHospital Universitario CrucesBarakaldoSpain
  11. 11.Department of Internal MedicineHospital Universitario San CecilioGranadaSpain
  12. 12.Thrombosis and Vasculitis Unit, Department of Internal MedicineComplexo Hospitalario Universitario de VigoVigoSpain
  13. 13.Department of Internal MedicineHospital Universitario Virgen de las NievesGranadaSpain
  14. 14.Department of Internal MedicineHospital Universitario La PazMadridSpain
  15. 15.Department of Internal MedicineHospital Universitario y Politécnico La FeValenciaSpain
  16. 16.Department of Internal MedicineHospital Universitari Mútua TerrassaBarcelonaSpain
  17. 17.Department of Internal MedicineHospital Clínico Universitario Lozano BlesaZaragozaSpain
  18. 18.Department of Internal MedicineComplejo Asistencial Universitario de SalamancaSalamancaSpain
  19. 19.Department of Internal MedicineConsorci Hospitalari de VicBarcelonaSpain
  20. 20.Department of Internal MedicineHospital General San JorgeHuescaSpain
  21. 21.Department of Internal MedicineHospital Universitario Fundación AlcorcónMadridSpain

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