PURPOSE: UIP is an histologic pattern that implies poor prognosis. However, in the past few years it has been suggested by some studies that UIP associated with CTD may have a different outcome than that associated with IPF. The aim of our study was to compare survival in patients in these two groups of patients with UIP.
METHODS: The clinical features, pulmonary function test (PFT) parameters and survival of 97 UIP patients diagnosed based on surgical biopsy or definitive UIP high resolution computed tomography (HRCT) were analyzed.
RESULTS: From the 97 UIP patients 68% were male, 78 (80,4%) had IPF and 19 (19,6%) CVD (17 rheumatoid arthritis, 1 systemic sclerosis, 1 dermatomyositis). UIP diagnosis was made using surgical biopsy in 31 and definite HRCT in 66. Patients with UIP/IPF were older than patients with CVD(67.95±9.4 vs 57.78±14.5, p=0,004). Although baseline lung function (FEV1% 78.1±25 vs 75.4±29.8, p=0,721, FVC% 70.6±25.5 vs 66.1±23.2, p=0,513 and DLCO% 51.7±23.8 vs 51.8±18.8, p=0,990)and the proportion of patients with dyspnea at the time of diagnosis (18/19 vs 61/78 p=0.84) were not significantly different, survival calculated by Kapman Meier was higher in patients with UIP secondary to CVD than in patients with UIP/IPF (median survival 63.4±0,5 vs FPI 47,5±0,06%, p=0,02).
CONCLUSIONS: Our data suggest that patients with UIP associated to CVD have a better 4 year survival than patients with IPF related UIP despite similar disease severity at the time of the diagnosis.
CLINICAL IMPLICATIONS: As suggested in previous studies, our findings show that the natural history of idiopathic UIP (IPF) cannot be extrapolated to CVD associated UIP. Physicians should make this clear to patients when discussing prognosis and therapeutic strategies.
DISCLOSURE: The following authors have nothing to disclose: Maria Otaola, Silvia Quadrelli, Gabriela Tabaj, Luciana Molinari, Valentina Di Boscio
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