Abstract
Idiopathic pulmonary fibrosis (IPF) is the most common parenchymal lung disease. Patients with IPF sometimes develop acute exacerbation (AE), which predicts a poor prognosis. To evaluate the predictors of 90-day mortality of AE in patients with IPF based on the new 2016 criteria. Sixty-five patients with AE were studied retrospectively between January 2001 and December 2016 at Okinawa Chubu Hospital. The mean age of the patients was 74 years, with 40 (61.5%) men and 25 (38.5%) women. Among our cohort, 37 were current or ex-smokers, with a mean exposure of 32.4 pack-years. The mean grade of the modified Medical Research Council breathlessness scale was 2.8, and the mean duration of dyspnea prior to admission was 6.5 days. Clubbed fingernails were present in 29% of patients. Triggered AE occurred in 12 (18%) of patients. Patients with triggered AE had more extensive ground-glass opacity and higher consolidation scores than the idiopathic AE group (7.3 vs. 4.2, p=0.01). The triggered group had shorter survival than the idiopathic group (1.4 vs. 11.4 months, p=0.094). Serum lactate dehydrogenase (LDH), ΔLDH, and the ratio of partial pressure of oxygen to the fraction of inspiratory oxygen ratio were strong predictors of 90-day mortality. Hazard ratios were 1.003 (p=0.004), 1.004 (p=0.02), and 0.994 (p=0.010), respectively. Compared with idiopathic AE, triggered AE in patients with IPF had more extensive infiltration and tended toward shorter survival. Serial trends of serum LDH >2 weeks can help predict prognosis of AE in patients with IPF.
Highlights
Idiopathic pulmonary fibrosis (IPF) is a relentless progressive parenchymal disease of unknown etiology [1]
The study population consisted of all patients who presented with acute exacerbation (AE) of IPF from January 2001 to December 2016 based on the 2016 American Thoracic Society (ATS) new criteria [9]
We showed that patients with IPF with triggered AE had more extensive fibrosis than those with idiopathic IPF and demonstrated the prognostic utility of the trend of the serum lactate dehydrogenase (LDH) levels
Summary
Idiopathic pulmonary fibrosis (IPF) is a relentless progressive parenchymal disease of unknown etiology [1]. Patients with IPF have variable clinical courses, from stable disease without significant symptoms to acute exacerbation (AE) [2, 3]. During AE in IPF, patients have acute progressive deterioration of their respiratory condition superimposed on a background of fibrosis [4, 5]. The American Thoracic Society (ATS) released the new revised criteria last year, which categorized AE in patients with IPF as either triggered or idiopathic, depending upon whether a “trigger,” such as infection, drug toxicity, aspiration, surgery, or other procedural intervention, was felt to be present. Diagnosis of AE in IPF consists of first excluding alternative causes of acute respiratory deterioration and determining whether the episode is associated with a trigger versus an idiopathic event [9]. The purpose of the current study is to evaluate the clinical characteristics and clinical course of the new categorization based on the revised criteria
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