PULMONARY FUNCTION TEST PARAMETERS IN DIFFUSE AND LIMITED SCLERODERMA

Abstract

SESSION TITLE: Pulmonary Manifestations of Systemic Disease SESSION TYPE: Original Investigation Posters PRESENTED ON: 10/10/2018 01:00 PM - 02:00 PM PURPOSE: Scleroderma is characterized by varying degrees of organ fibrosis and obliterative vasculopathy. Lung involvement is the leading cause of morbidity in Scleroderma. The spectrum of Pulmonary Function Test (PFT) abnormalities in Scleroderma has been reported to be variable, with a recent study revealing that PFTs have a low sensitivity in detecting Scleroderma-related interstitial lung disease, with false negative cases more common in diffuse Scleroderma. The objectives of this study are to determine the spectrum of PFT abnormalities in Scleroderma and to compare PFTs in limited and diffuse Scleroderma. METHODS: We reviewed the records of 174 Scleroderma patients followed at the rheumatology clinic in our institution. Patient demographics and PFT parameters [FEV1, FVC, FEV1/FVC, TLC and DLCO percent predicted (%)] were noted in patients with limited (Scl-Ltd) and diffuse (Scl-Diffuse) disease. Obstruction was defined as FEV1/FVC < 70%; restriction as TLC < 80% and impaired diffusion as DLCO < 80%. Severity of restriction was defined per ATS criteria. PFT parameters in the two groups were compared by Student’s t-test. p < 0.05 was deemed statistically significant. RESULTS: Mean age: 65 +/- 12.2 years; 91% were females; 76.4% had Scl-Ltd. Mean FEV1 was: 92 +/- 22%; mean FVC: 88.5 +/- 21.3%; mean TLC: 91.5 +/- 18%; mean DLCO:70.5 +/- 23%. Twenty-eight % patients had normal PFTs; 12 % had obstruction, 32% had restriction and 28% patients had isolated reduction in DLCO. Thirty-five % patients with Scl-Ltd and 24.4% patients with Scl-Diffuse had a normal DLCO. In patients with restriction, 40% had mild, 51% moderate and 9% had severe restriction. PFT abnormalities were seen in 37.6% Scl-Ltd, and 56% Scl-Diffuse. PFT parameters in the 2 groups are shown below. PFT parameters in 2 groups (Table) CONCLUSIONS: Majority of Scleroderma-patients have abnormal PFTs. Restriction and impairment in diffusion were the most common abnormalities. Over 1/3rd patients with limited disease had PFT abnormalities. CLINICAL IMPLICATIONS: Whether restriction defined by TLC corelates with fibrosis on imaging need to be studied. DISCLOSURES: No relevant relationships by Debapriya Datta, source=Web Response No relevant relationships by Carol Halasan, source=Web Response No relevant relationships by Sophie Korzan, source=Web Response