Abstract
BackgroundAcute exacerbation (AE) is the major cause of morbidity and mortality in patients with idiopathic pulmonary fibrosis (IPF). AEs also occur in other forms of fibrosing interstitial lung disease (fILD). The clinical features and prognosis of AE patients with connective tissue diseases (CTDs) associated-ILD has not been fully described.MethodsWe retrospectively reviewed 177 patients with either IPF or a characterized CTD-ILD admitted to Nanjing Drum Tower Hospital with an AE from January 2010 to December 2016.ResultsThe study cohort included 107 subjects with AE-IPF and 70 cases with AE-CTD-ILD. Female gender, prior use of corticosteroid and immunosupressants, lower serum albumin, higher D-dimer level, TLC% pred, survival, and treatment with immunosupressants and caspofungin were more common in the CTD-ILD group (all p<0.05). The incidences of AE-CTD-ILD and AE-IPF were similar in our single center (p = 0.526). TLC% pred was the risk factor for AE after ILD diagnosis for 1 year in CTD patients (p = 0.018). Log-rank tests showed patients with CTD-ILD had a significantly lower mortality rate compared with IPF patients after AEs (p = 0.029). No significant difference in survival was noted among CTD subgroups (p = 0.353). The survival was negatively correlated with WBC count, LDH and CT score, (p = 0.006, p = 0.013 and p = 0.035, respectively), and positively correlated with PaO2/FiO2 ratio (p<0.001) in the CTD-ILD group. WBC count and PO2/FiO2 ratio were the independent predictors for survival in AE-CTD-ILD after adjusting for other clinical variates in Cox regression Models (p = 0.038 and p < 0.001, respectively).ConclusionsThe clinical characteristics of patients with AE-CTD-ILD differed from those with AE-IPF, while AE incidences were similar between the two groups. Subjects with AE-CTD-fILD tended to have a better prognosis, and WBC count and PO2/FiO2 ratio were the independent survival predictors for these patients.
Highlights
Acute exacerbation (AE) is the major cause of morbidity and mortality in patients with idiopathic pulmonary fibrosis (IPF)
Recent evidence suggests that causes of acute lung injury (ALI) and histopathologic diffuse alveolar damage (DAD) such as infection, aspiration, surgery and air pollution exposure could contribute to the development of AE-IPF [3, 10, 11]
AE-IPF and AE-connective tissue diseases (CTDs)-fibrosing interstitial lung disease (fILD) were defined as an acute, clinically significant respiratory deterioration characterized by evidence of new widespread alveolar abnormality with: 1 Previous or concurrent diagnosis of IPF or a characterized CTD-fILD; 2 Acute worsening or development of dyspnea typically 1 month duration; 3 CT with new bilateral ground-glass opacity and/or consolidation superimposed on a background pattern consistent with usual interstitial pneumonia (UIP) or possible UIP pattern; 4 Deterioration not fully
Summary
Acute exacerbation (AE) is the major cause of morbidity and mortality in patients with idiopathic pulmonary fibrosis (IPF). Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive scarring disease of the lung characterized by worsening dyspnea and lung function over time [1, 2]. Some patients will experience an acute, clinically significant respiratory deterioration characterized by widespread alveolar abnormality [3]. Acute exacerbations (AEs) are a major cause of mortality of patients with. Recent evidence suggests that causes of acute lung injury (ALI) and histopathologic diffuse alveolar damage (DAD) such as infection, aspiration, surgery and air pollution exposure could contribute to the development of AE-IPF [3, 10, 11].
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