First clinical symptom as a prognostic factor in systemic sclerosis: results of a retrospective nationwide cohort study
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.
KeywordsOnset Prognosis Raynaud Scleroderma Systemic sclerosis
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.
Compliance with ethical standards
- 1.Steen VD, Medsger TA Jr (2000) Severe organ involvement in systemic sclerosis with diffuse scleroderma. Arthritis Rheum 43(11):2437–2444. https://doi.org/10.1002/1529-0131(200011)43:11<2437::AID-ANR10>3.0.CO;2-U CrossRefPubMedGoogle Scholar
- 2.Simeón-Aznar CP, Fonollosa-Pla V, Tolosa-Vilella C et al (2012) Registry of the Spanish network for systemic sclerosis: clinical pattern according to cutaneous subsets and immunological status. Semin Arthritis Rheum 41(6):789–800. https://doi.org/10.1016/j.semarthrit.2011.10.004 CrossRefPubMedGoogle Scholar
- 3.Simeón-Aznar CP, Tolosa-Vilella C, Gabarró-Juliá L et al (2014) Systemic sclerosis sine scleroderma and limited cutaneous systemic sclerosis: similarities and differences. Clin Exp Rheumatol 32:33–40Google Scholar
- 6.Van den Hoogen F, Khanna D, Fransen J et al (2013) 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis 72(11):1747–1755. https://doi.org/10.1136/annrheumdis-2013-204424 CrossRefPubMedGoogle Scholar
- 9.Simeón-Aznar CP, Fonollosa-Plá V, Tolosa-Vilella C, Espinosa-Garriga G, Campillo-Grau M, Ramos-Casals M, García-Hernández FJ, Castillo-Palma MJ, Sánchez-Román J, Callejas-Rubio JL, Ortego-Centeno N, Egurbide-Arberas MV, Trapiellla-Martínez L, Caminal-Montero L, Sáez-Comet L, Velilla-Marco J, Camps-García MT, de Ramón-Garrido E, Esteban-Marcos EM, Pallarés-Ferreres L, Navarrete-Navarrete N, Vargas-Hitos JA, Gómez de la Torre R, Salvador-Cervello G, Rios-Blanco JJ, Vilardell-Tarrés M, Spanish Scleroderma Study Group (SSSG), Autoimmune Diseases Study Group (GEAS), Spanish Society of Internal Medicine (SEMI) (2015) Registry of the Spanish network for systemic sclerosis: survival, prognostic factors, and causes of death. Medicine (Baltimore) 94(43):e1728. https://doi.org/10.1097/MD.0000000000001728 CrossRefGoogle Scholar
- 11.Somme D, Duterque M, Verdaguer M, Lardoux H (1999) CREST syndrome presenting as pulmonary hypertension. Ann Cardiol Angeiol (Paris) 48(2):109–112Google Scholar
- 18.Rubio-Rivas M (2014) Changes in the pattern of death from the Spanish Scleroderma Registry. Doctoral Thesis. Universitat Autònoma de BarcelonaGoogle Scholar