SESSION TYPE: ILD Posters
PRESENTED ON: Wednesday, October 24, 2012 at 01:30 PM - 02:30 PM
PURPOSE: Pulmonary function tests help define severity of restrictive ventilator defect in diffuse fibrotic lung diseases like Idiopathic pulmonary fibrosis (IPF) and scleroderma. VC, TLC and DLCO are used interchangeably to define disease severity. VC is easily measured with office based spirometry, whereas measuring TLC and DLCO requires pulmonary physiology Lab. We sought to define the pattern of PFT abnormality and prevalence of reduced VC, TLC and diffusion capacity in patients with idiopathic pulmonary fibrosis (IPF) and Sclerodema.
METHODS: Study was performed in a 400 bed University based hospital in northern B’klyn, NYC. PFT data and imaging were reviewed on all patients with IPF and scleroderma from January 2007 to January 2012.
RESULTS: 33 patients (Scleroderma-11 and IPF-22) were included. In IPF goroup; 8 were males and 14 were females, with median age of 37 yeas (range: 24-72). 13/22 (60 %) had restriction. VC was reduced in 9/22 and TLC was reduced in 11/22 patients. DLCO was decreased in 19/22 (86%) of the patients. 2 patients had obstruction. 3 patients had normal PFT. All patients had extensive fibrotic disease in imaging except for patients who had normal PFT’s. In Scleroderma group; all patients were female with median age of 50 years (range 30-75). All patients had restrictive physiology. TLC was low in all patients; VC was reduced in 6/11 (54%) and DLCO was reduced in 81% of the patients. All patients had lung fibrosis on imaging except for 2; who had restrictive physiology on PFT.
CONCLUSIONS: DLCO is the most sensitive parameter, followed by reduced TLC (because of increased elastic recoil) and then VC in patients with pulmonary fibrosis. TLC was universally low in scleroderma group even more than DLCO
CLINICAL IMPLICATIONS: Spirometry results in underdiagnosis of restriction in both scleroderma and IPF. In symptomatic patients, complete PFT including static lung volume and Diffusion measurement is critical to identify restrictive ventilatory defect to accurately define prognosis and guide therapy.
DISCLOSURE: The following authors have nothing to disclose: Ameer Rasheed, Viswanath Vasudevan, Farhad Arjomand
No Product/Research Disclosure InformationThe Brooklyn Hospital Center, Brooklyn, NY