Fast Five Quiz: Progressive Fibrosing Interstitial Lung Disease Presentation and Diagnosis

Zab Mosenifar, MD


April 24, 2023

Approximately 15% of patients with ILD have an underlying collagen vascular disease. HRCT assessments have shown that nonspecific interstitial pneumonia is the most common pattern, per Boland and colleagues. However, patients may also present with a pattern of usual interstitial disease. In such cases, it is important to differentiate the disease from IPF.

Honeycombing is normally absent from HRCT findings of patients with idiopathic nonspecific interstitial pneumonia. Common findings include peripheral, basal, symmetric, predominantly lower-lung reticular ground-glass opacities with irregular lines, traction bronchiectasis, consolidation and lower lobe volume loss that is usually diffuse or subpleural in the axial dimension.

With respect to serum biomarkers, measuring Krebs von den Lungen 6, may be useful in differentiating the four common ILDs. However, serum biomarker testing is not recommended due to high false-positive and false-negative results.

Patients with fibrosing pulmonary sarcoidosis rarely present with a UIP pattern of lung injury. Imaging more often reveals faint reticulations to dense linear bands, cystic lung disease, traction bronchiectasis, and airway distortion, typically involving the middle and upper lung zones.

Learn more about advances in the management of PF-ILD.


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