PURPOSE: Systemic sclerosis (SSc) is frequently associated with interstitial lung disease (ILD). There is controversy in literature between the tomographics findings and the prognosis of patients with diagnosis of SSc. The tomographic grading systems are difficult in daily practice.Objectives: Determine if the presence of interstitial pulmonary disease and the type of affectation (suspicion of UIP Vs non-UIP) by high resolution computed tomography (HRCT) correlate with mortality at 5 years.
METHODS: Cohort study of 70 consecutively patients included between 2001 and 2004. All patients underwent standardized clinical records, HRCT and functional studies.The patients were maintained in pursuit by at least 5 years and if they did not remain in pursuit were contacted before July 2009 (more than 5 years of the diagnosis) to state mortality.
RESULTS: 70 patients were included (59 women, age 54.7 ± 9 years). 57% had ILD, 17% were considered highly suspicious of UIP. The FVC (73.1 ± 17.8% vs 84.5 ± 19.3% p = 0.016) and the DLCO (57.4 ± 16.6% vs 66.6 ± 16.9% p = 0.028) of patients with diagnosis of ILD were significantly smaller than those of the group with normal HRCT. The pulmonary function of patients with ILD highly suspect UIP vs non-UIP was not different. The ILD presence did not increase mortality at five years (15 vs 3% p = 0.226). The patients with non-UIP vs highly suspicious UIP in HRCT did not present significant differences in survival at five years (81 Vs 100%, p = 0.567). In a logistic regression model neither the presence of ILD in HRCT (OR 0.195 IC 0.0223 to 1.7150 p = 0.140) nor the suspicion of UIP in HRCT (p = 0.967) predicted mortality at 5 years.
CONCLUSION: The ILD presence does not make worse survival in the first 5 years of the diagnosis although it implies functional commitment identified by TLCO and FVC.
CLINICAL IMPLICATIONS: The UIP pattern in HRCT in patients with SSc did not generate the impact in survival observed in idiopathic disease.
DISCLOSURE: Nicolas Baillieau, No Financial Disclosure Information; No Product/Research Disclosure Information