Clinical features and outcome of acute exacerbation in connective tissue disease-associated interstitial lung disease: A single-center study from India.

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. 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. 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 (PaO2 ) 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 PaO2 (mean ± SD), 72.6 ± 3.4 vs 86.2 ± 5.3 mm Hg (P = .002) had higher mortality. In our study, the majority of patients with AE CTD ILD had systemic sclerosis. Patients with lower baseline PaO2 and those requiring mechanical ventilation had higher mortality.

Singh P ，Thakur B ，Mohapatra AK ，Padhan P ... -  《-》

Acute exacerbations of fibrosing interstitial lung disease associated with connective tissue diseases: a population-based study.

Cao M ，Sheng J ，Qiu X ，Wang D ，Wang D ，Wang Y ，Xiao Y ，Cai H ... -  《BMC Pulmonary Medicine》

Differences in clinical features of acute exacerbation between connective tissue disease-associated interstitial pneumonia and idiopathic pulmonary fibrosis.

Enomoto N ，Oyama Y ，Enomoto Y ，Yasui H ，Karayama M ，Kono M ，Hozumi H ，Suzuki Y ，Furuhashi K ，Fujisawa T ，Inui N ，Nakamura Y ，Suda T ... -  《-》

Comparison of characteristics of connective tissue disease-associated interstitial lung diseases, undifferentiated connective tissue disease-associated interstitial lung diseases, and idiopathic pulmonary fibrosis in Chinese Han population: a retrospectiv

Pan L ，Liu Y ，Sun R ，Fan M ，Shi G ... -  《-》

Interstitial lung disease evaluation: detecting connective tissue disease.

Interstitial lung disease (ILD) is a common manifestation of connective tissue disease (CTD) and is often associated with significant morbidity and mortality. The evaluation of ILD in patients with CTD is complex because of the heterogeneity of the CTD spectrum, the various patterns and degrees of severity of ILD encountered, and because ILD can be identified at any point in time in these patients. A thorough - and optimally multidisciplinary - evaluation is needed when CTD patients develop ILD or when evaluating ILD patients for the presence of occult CTD. Determining whether ILD is associated with a preexisting CTD requires the exclusion of alternative etiologies, and thorough assessments of the clinical features of both the CTD and ILD. The detection of occult CTD in patients with presumed idiopathic interstitial pneumonia requires careful integration of clinical, serologic, and thoracic imaging and histopathologic features.

Fischer A ，Lee JS ，Cottin V 《-》