ILD associated with connective tissue disease may present with fibrosing lung injury, and 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, although the subpleural lung is sometimes spared.
With respect to serum biomarkers, measuring Krebs von den Lungen 6, serum matrix metalloproteinase 7, surfactant protein D, or chemokine ligand 18 for the purpose of distinguishing IPF from other ILDs 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, although honeycombing may be seen in the upper zones of the lungs. Imaging more often reveals faint reticulations to dense linear bands, cystic lung disease, traction bronchiectasis, and airway distortion.
Learn more about advances in the management of PF-ILD.
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Cite this: Zab Mosenifar. Fast Five Quiz: Progressive Fibrosing Interstitial Lung Disease Presentation and Diagnosis - Medscape - Jun 16, 2022.