Acute Exacerbation of Idiopathic Pulmonary Fibrosis

Abstract

Idiopathic Pulmonary Fibrosis (IPF) is most common idiopathic interstitial pneumonias. IIPF is often seen in elderly smoker man. Diagnosis of IPF is integration of detailed clinical history, specific physical examination, laboratory findings, pulmonary function test, chest high-resolution computed tomography (HRCT) and pathology. IPF have heterogeneous clinical course from asymptomatic stable state to progressive respiratory failure or acute exacerbation (AE). AE of IPF have several important differential diagnosis such as heart failure and volume overload. International working project proposed new criteria of AE of IPF in 2016. They divided into triggered and idiopathic AE. On the basis of this criteria, physician can capture AE of IPF more easily. Recent international IPF guideline emphasized the utility of chest HRCT. In addition, two anti-fibrotic agents have been available. We should pay attention to not only management of AE, but also prevention it. I review diagnostic process, laboratory findings, typical chest imaging, management and prognosis of AE.

Detection of interstitial pneumonia with autoimmune features and idiopathic pulmonary fibrosis are enhanced by involvement of matrix metalloproteinases levels and clinical diagnosis

“…Several studies have given eloquent proof that IPAF is associated with age, female sex, and smoking status [4,7,[50][51][52][53][54]. In addition, our group et al found that KL-6 can re ect the progression of the disease and play a key role in re ecting the degree of lung epithelial cell injury and brosis in IPAF.…”

“…Several studies have given eloquent proof that IPAF is associated with age, female sex, and smoking status. 4,7,[25][26][27][28][29] In addition, we also found that KL-6 can reflect the disease progression and play a key role in the display of the degree of lung epithelial cell injury and fibrosis in patients with IPAF. In spite of MMPs combined with clinical risk factors that could detect the presence of IPAF in this study, it should be noted that MMP-7 and MMP-12 were weak differences between IPAF and IPF cohorts.…”

Patterns of lung fibrosis in patients with interstitial pneumonia with autoimmune features and connective tissue diseases-associated interstitial lung disease—a narrative review

“…It was also suggested that honeycombing with predilection to the anterior upper lobe is distinctive of RA-ILD with UIP or UIP/NSIP mixed pattern. In SSc-ILD and PM/DM-ILD, the most predominant pattern was fibrosing NSIP without honeycombing (116).…”

Radiological and Physiological Predictors of IPF Mortality

“…The chest radiograph is easy to use and cost effective in clinical practice, as an alternative to HRCT, and provides useful new information for clinicians. Regarding the role of the chest radiograph for IPF patients, both distribution of fibrosis and volume loss of the bilateral lower lung field have been addressed [ 24 , 25 ]. Chest HRCT has played a major role in the diagnosis and treatment response of IPF patients [ 26 , 27 , 28 ].…”