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Clinical features and outcome of acute exacerbation in connective tissue disease‐associated interstitial lung disease: A single‐center study from India
Author(s) -
Singh Pratima,
Thakur Bhaskar,
Mohapatra Amrut Kumar,
Padhan Prasanta
Publication year - 2019
Publication title -
international journal of rheumatic diseases
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.795
H-Index - 41
eISSN - 1756-185X
pISSN - 1756-1841
DOI - 10.1111/1756-185x.13666
Subject(s) - medicine , interstitial lung disease , exacerbation , connective tissue disease , idiopathic pulmonary fibrosis , ctd , gastroenterology , mechanical ventilation , lung , disease , autoimmune disease , oceanography , geology
Background Acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) is associated with high mortality, but there is limited clinical data on AE of interstitial lung disease (ILD) in connective tissue disease‐associated ILD (CTD ILD). The present study was conducted to provide prevalence and clinical features of AE, as well as various risk factors associated with mortality among AE CTD ILD patients. Methods Between May 2013 and April 2018, 15 patients who developed AE among 105 consecutive patients with CTD with chronic ILD were included. AE was defined using the criteria recently proposed by the IPF net, with slight modification for adaptation to CVD‐IP6 (collagen vascular disease‐associated interstitial pneumonia), and patients having CTD with AE met all the criteria. Results Fifteen patients with mean age of 45.8 ± 13.9 years developed AE; the most common subgroup (n = 5, 33%) was systemic sclerosis. The mean duration (months) between diagnosis of ILD and AE was 56.5 ± 38.0 with mean follow‐up duration of 24 ± 18.1 months. The baseline arterial oxygen pressure (PaO 2 ) was 81.7 ± 8.1 mm Hg and mean forced vital capacity (%) was 57.9 ± 8.9. Five patients requiring mechanical ventilation died. Patients with shorter duration (months) of disease between onset of ILD to AE had higher mortality, 40.4 ± 45.1 vs 64.6 ± 33.6. Those who had significantly lower baseline PaO 2 (mean ± SD), 72.6 ± 3.4 vs 86.2 ± 5.3 mm Hg ( P = .002) had higher mortality. Conclusions In our study, the majority of patients with AE CTD ILD had systemic sclerosis. Patients with lower baseline PaO 2 and those requiring mechanical ventilation had higher mortality.