ABSTRACT
OBJECTIVES: Peripheral microangiopathy is a hallmark of systemic sclerosis (SSc) and can be early detected by nailfold capillaroscopy (NFC). This study aimed to examine whether more severe peripheral microangiopathy at NFC are predictive factor for death in SSc patients. METHODS: 135 SSc patients who performed NFC between June 2001 and July 2009 were included. The following NFC parameters were evaluated: number of capillary loops/mm, avascular score (scored from 0 to 3), and number of enlarged and giant capillary loops. Univariate and multivariate regression models were used to analyse the association of mortality with NFC and clinical parameters. RESULTS: At the time of the analysis (August 2010), 123 patients were alive, and 12 were dead. By univariate analysis, male gender, forced vital capacity <75% predicted, higher number of giant capillary loops, and an avascular score >1.5 on NFC were associated with a significantly increase risk of death. By multivariate analysis, an avascular score >1.5 was the only independent predictor of death (hazard ratio 2.265). Survival rates from diagnosis at 1, 5 and 10 years were lower in patients with avascular score >1.5 (97%, 86%, and 59%, respectively) compared with those with avascular score ≤1.5 (97%, 97%, and 91% respectively) (p=0.009 by log rank test). CONCLUSIONS: Avascular scores higher than 1.5 at NFC was an independent predictor of death in SSc, suggesting that NFC can be useful for predicting SSc outcome.
Subject(s)
Microscopic Angioscopy , Nails/blood supply , Scleroderma, Systemic/mortality , Scleroderma, Systemic/physiopathology , Adult , Female , Follow-Up Studies , Humans , Kaplan-Meier Estimate , Male , Middle Aged , Multivariate Analysis , Predictive Value of Tests , Raynaud Disease/mortality , Raynaud Disease/physiopathology , Retrospective Studies , Risk Factors , Vital CapacityABSTRACT
Se presentan dos casos de esclerodermia con restricción pulmonar grave: un paciente masculino de 23 años con fiebre de 20 días de evolución asociada a disnea de medianos esfuerzos y enfermedad de Raynaud; y una paciente de 31 años con disnea de medianos esfuerzos y enfermedad de Raynaud. Ambos fallecieron por insuficiencia respiratoria aguda. La trascendencia de mostrar estos dos casos radica en la súbita presentación de la insuficiencia respiratoria en esclerodermia de reciente inicio