Resumen

QUADRELLI, Silvia et al. Supervivencia en pacientes con neumonía intersticial usual en contexto de fibrosis pulmonar idiopática y enfermedad del tejido conectivo. Rev. am. med. respir. [online]. 2015, vol.15, n.1, pp.36-50. ISSN 1852-236X.

Background: Usual interstitial pneumonia (UIP) is a histologic pattern that implies poor prognosis. However, some studies have suggested that UIP associated to connective tissue diseases (CTD-UIP) may have a different outcome than that associated with idiopathic pulmonary fibrosis (IPF). Objectives: To compare disease severity and survival between IPF and UIP associated to connective tissue diseases including scleroderma, rheumatoid arthritis, polymyositis and mixed CTD. Methods: The study included the analysis of clinical features and survival of 102 patients (81 with IPF and 21with CTD-UIP) diagnosed through surgical biopsy or high resolution computed tomography (HRCT) in patients with definitive UIP. Results: Median follow-up was 24 months (0 to 146 months). Forty-four patients died during the follow-up; the proportion of deaths was significantly higher amongst patients with IPF than amongst patients with CTD-UIP (49.4 vs 19.0%, p = 0.014). The 3 and 5 year survival was higher in patients with UIP secondary to CTD than in patients with IPF. Patients with CTD-UIP showed 3 and 5-year case fatality rate of 19.5% and 20.0% respectively, compared to 3 and 5-year case fatality rate of 35.0%, and 65.9% respectively in patients with IPF (p = 0.014). Patients with IPF were older than patients with CTD-UIP (age 67.95 ±9.4 vs 57.78 ±14.5, p = 0.021) and were more likely to be male (67.9% vs 33.3%, p = 0.006). There were no significant differences among baseline lung function, time between onset of symptoms and diagnosis, number of patients biopsied and the proportion of patients with dyspnea at the time of diagnosis between IPF and CTD-UIP patients. By multivariate analysis, the diffusing capacity of the lung for carbon monoxide (DLCO) and the presence of IPF were independent prognostic factors. Conclusions: Our data suggest that patients with UIP associated to CTD have a better survival than patients with IPF related UIP despite similar disease severity at the time of the diagnosis.

Palabras clave : Interstitial Lung Disease; Usual Interstitial Pneumonia; Connective Tissue Disease; Idiopathic Pulmonary Fibrosis; Survival.

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