RESUMEN
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.
Asunto(s)
Artralgia/etiología , Hipertensión Pulmonar/etiología , Enfermedades Pulmonares Intersticiales/etiología , Enfermedad de Raynaud/etiología , Esclerodermia Sistémica/diagnóstico , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Pronóstico , Sistema de Registros , Estudios Retrospectivos , Esclerodermia Sistémica/complicaciones , Esclerodermia Sistémica/fisiopatología , Índice de Severidad de la Enfermedad , Evaluación de SíntomasRESUMEN
A literature review about Castleman's disease after a clinical case with different manifestations of this entity was done. Special emphasis on the histological and clinical aspects of the sickness was given. The different pathological patterns and their broader spread do not match necessarily with the different clinical presentations described. Further conclusions regarding their evolution, prognosis and treatment response can not be easily obtained from these data.
