PT  - JOURNAL ARTICLE
AU  - Erik Palmer
AU  - Veronika Müller
AU  - Anikó Bohács
AU  - Krisztina Vincze
AU  - Noémi Eszes
TI  - Longitudinal functional evaluation of interstitial lung disease in systemic sclerosis
AID  - 10.1183/13993003.congress-2020.3540
DP  - 2020 Sep 07
TA  - European Respiratory Journal
PG  - 3540
VI  - 56
IP  - suppl 64
4099  - http://erj.ersjournals.com/content/56/suppl_64/3540.short
4100  - http://erj.ersjournals.com/content/56/suppl_64/3540.full
SO  - Eur Respir J2020 Sep 07; 56
AB  - Scleroderma interstitial lung disease (SSc-ILD) is the leading cause of SSc related mortality.As limited data are available about longitudinal lung function evaluation in prevalent SSc-ILD patients, our aim was longitudinal evaluation of lung disease progression.We retrospectively analyzed clinical manifestations and pulmonary function changes in SSc-ILD patients in a time period of 18 months at our university center. SSc-ILD was identified using ICD criteria for both diseases in 54 cases, while 41 had at least 2 visits in this time period. Patients characteristic, pulmonary symptoms, spirometry with pletysmography, CO diffusion (DLco and KLco), high resolution CT (HRCT) pattern and treatment were analyzed.Data of 41 SSc-ILD patients were analyzed (12% men, mean age 58±13 years), majority never smokers (74%). Dyspnea and/or cough was present in 25%, 70% of patients had a history of Raynaud-syndrome and 20% pulmonary hypertension. Pulmonary function tests showed minimal decrease in FVC (89.8 ± 23.2% predicted) with a moderate impairment inCO diffusion (DLco: 75.2 ± 22.0%; KLco: 70.0 ± 18.2% predicted). HRCT confirmed usual interstitial pneumonia, non-specific interstitial pneumonia or isolated ground glass opacities in similar numbers. All patients received immunosuppressive therapy. Longitudinal functional examinations showed an annual decrease of 3.9 ± 6.0% in FVC, 4.8 ± 6.6% in DL CO and 6.7 ±7.6% in KL CO .Our prevalent SSc-ILD patients presented with mild restrictive ventilatory disorder, showing small annual decline in FVC and more pronounced decline in DL CO and KLco. Identification of progressive phenotypes needs regular functional follow-up especially in symptomatic patients.FootnotesCite this article as: European Respiratory Journal 2020; 56: Suppl. 64, 3540.This abstract was presented at the 2020 ERS International Congress, in session “Respiratory viruses in the "pre COVID-19" era”.This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).