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Diffuse Lung Disease |

Pulmonary Function Tests and 5-Year Survival in Patients Systemic Sclerosis and Interstitial Lung Disease (ILD)

Maria Otaola*, MD; Silvia Quadrelli, PhD; Gabriela Tabaj, MD; Raquel Aguirre, MD; Luciana Molinari, MD; Valentina Di Boscio, MD
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British Hospital Buenos Aires, Buenos Aires, Argentina


Chest. 2012;142(4_MeetingAbstracts):433A. doi:10.1378/chest.1386405
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Abstract

SESSION TYPE: Interesting Topics in ILD

PRESENTED ON: Tuesday, October 23, 2012 at 02:45 PM - 04:15 PM

PURPOSE: Pulmonary complication, including ILD, have now surpassed renal involvement as the most common cause of death in patients with systemic sclerosis (SSc). Pulmonary function tests (PFTs), including Forced Vital Capacity (FVC) and diffusion capacity of Carbon Monoxide (DLCO), and high-resolution CT (HRCT) are the tools with which to identify pulmonary fibrosis in systemic sclerosis. However, which parameters are associated with increased mortality is still under revision. The aim of our study was to determine the predictive value of PFT on 5 year mortality in a cohort of 70 patients with SSc associated ILD.

METHODS: Prospective evaluation of 70 patients that met American Rheumatism Association criteria for SSc and were evaluated at the Buenos Aires British Hospital Interstitial Lung Disease Institute between January 2001 and November 2004. The clinical features, pulmonary function test (PFT) and high resolution computed tomography (HRCT) findings were registered. Survival status was obtained from telephone interview and/or medical records. Patients lost of follow-up or with incomplete data were excluded.

RESULTS: From the 70 ILD-SSc patients included, 84% were female and the mean age was 54.7±9 years. At 5 years of follow up 63patients (90%) were alive. Baseline FVC% 80.0±18.6 vs 60.0±15.0, p=0,011, TLC% 82.8±17.8 vs 61.6±6.4, p=<0,0001 and SVC% 80.5±19.4 vs 50.1±3.9, p=<0,0001 were significantly higher in patients who survived. However, DLCO% 62.5±17.7 vs 50.1±15.5, p=0,086 and total meters walked on 6MWT 435.0±98.4 vs 350.1±113.5, p=0,1 were not significantly different between the two groups. In a logistic regression model a DLCO <35% was not capable of predicting 5 year mortality (OR: 0,1818, IC 0,0153 a 2,1541, p = 0,1765) whereas a FVC < 50% was a associated with a lower probability of being alive at 5 years (OR: 0,0847, IC 0,0098 a 0,7364, p = 0,0253).

CONCLUSIONS: Our research shows that the pulmonary function tests that are significantly decreased in SSc patients who had died at 5 years are FVC, TLC and SVC. In our cohort of patients a FVC < 50% was the best predictor of 5 year survival.

CLINICAL IMPLICATIONS: As suggested in previous studies, our findings show that FVC is the best predictor of advanced fibrosis in ILD-SSc patients. DLco reduction is seen earlier in the course of the disease, but is sometimes misleading because it may reflect pulmonary vascular disease. However, there is no universally accepted cutoff DLco or FVC level at which therapeutic intervention becomes mandatory.

DISCLOSURE: The following authors have nothing to disclose: Maria Otaola, Silvia Quadrelli, Gabriela Tabaj, Raquel Aguirre, Luciana Molinari, Valentina Di Boscio

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British Hospital Buenos Aires, Buenos Aires, Argentina

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