Physiological Aberrations in IPF

What Are the Best Physiological Tests to Assess Clinical Course or Response to Therapy  

Chest radiographs in UIP typically reveal diffuse, bilateral, interstitial (reticular) infiltrates, with a predilection for basilar and peripheral (subpleural) regions (2,18). Similar radiographic features may be noted in asbestosis (2) and CVD-associated pulmonary fibrosis (3,18). 

High-resolution thin section (1-2 mm) CT scans are an integral component of the initial evaluation of suspected ILD. HRCT can be performed without contrast and has diagnostic and prognostic value (17,18,123). HRCT can demonstrate the 

The global extent of disease on HRCT correlates roughly with severity of functional impairment in IPF (18,131). The extent of disease of CT correlated best with FVC and DLco (18,85,131). In a study of 54 patients with IPF without emphysema, extent of disease on CT correlated best with percent predicted DLco (P = 0.68), 

Several studies assessed the evolution of CT over time (18,131,142). Pulmonary functional parameters improved only when GGO regressed on HRCT (18,131,142). Reticular or HC patterns reflect fibrosis and do not regress with treatment (18,131,142). Severe HC on CT is a strong predictor of mortality ( 80% mortality within two years) (11,122,143). CT patterns typical of IPF/ UIP were associated with a higher mortality compared to atypical CT scans (114,144), suggesting that CT features typical of UIP likely reflect advanced disease. 

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