Table 2.

Idiopathic PPFE diagnostic criteria

Idiopathic PPFE diagnostic criteria
1. Slowly progressive dry cough or exertional dyspnoea.
2. Alveolar consolidation just below the pleura with traction bronchiectasis, predominantly in the bilateral upper lobes on HRCT.
3. Upward shift of bilateral pulmonary hilum on chest x-ray or volume reduction of the upper lobes on HRCT.
4. Exclusion of other diseases with known causes, such as fibrotic hypersensitivity pneumonitis, connective tissue disease, occupational lung disease, hematopoietic stem cells, or lung transplant-related lung disease, were excluded.

Radiologically possible idiopathic PPFE criteria described in [12], partially modified

Abbreviations: HRCT High-resolution CT, PPFE Pleuroparenchymal fibroelastosis