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

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Autores de IIS La Fe

Participantes ajenos a IIS La Fe

  • Rubio-Rivas M
  • Corbella X
  • Pestaña-Fernández M
  • Tolosa-Vilella C
  • Guillen-Del Castillo A
  • Colunga-Argüelles D
  • Trapiella-Martínez L
  • Iniesta-Arandia N
  • Castillo-Palma MJ
  • Sáez-Comet L
  • Egurbide-Arberas MV
  • Ortego-Centeno N
  • Freire M
  • Vargas-Hitos JA
  • Ríos-Blanco JJ
  • Rodríguez-Carballeira M
  • Marín-Ballvé A
  • Segovia-Alonso P
  • Pla-Salas X
  • Madroñero-Vuelta AB
  • Ruiz-Muñoz M
  • Fonollosa-Pla V
  • Simeón-Aznar CP
  • RESCLE investigators, Autoimmune Diseases Study Group (GEAS)
  • Callejas Moraga E
  • Calvo E
  • Carbonell C
  • Castillo MJ
  • Chamorro AJ
  • Colunga D
  • Corbella X
  • Egurbide MV
  • Espinosa G
  • Fonollosa V
  • Freire M
  • García Hernández FJ
  • González León R
  • Guillén Del Castillo A
  • Iniesta N
  • Lorenzo R
  • Madroñero AB
  • Marí B
  • Marín A
  • Ortego-Centeno N
  • Pérez Conesa M
  • Pestaña M
  • Pla X
  • Ríos Blanco JJ
  • Rodríguez Carballeira M
  • Rubio Rivas M
  • Ruiz Muñoz M
  • Sáez Comet L
  • Segovia P
  • Simeón CP
  • Soto A
  • Tarí E
  • Tolosa C
  • Trapiella L
  • Vargas Hitos JA
  • Verdejo G

Grupos

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.

Datos de la publicación

ISSN/ISSNe:
0770-3198, 1434-9949

CLINICAL RHEUMATOLOGY  SPRINGER LONDON LTD

Tipo:
Article
Páginas:
999-1009
PubMed:
29214548
Factor de Impacto:
0,948 SCImago
Cuartil:
Q1 SCImago

Citas Recibidas en Web of Science: 25

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Keywords

  • Onset; Prognosis; Raynaud; Scleroderma; Systemic sclerosis

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