Symptoms (not all of which may be present) |
Dyspnoea |
Dry cough |
Weight loss |
Dull chest pain |
Anteroposterior flattening of the chest wall (platythorax) mostly present in advanced cases ( fig. 3), may denote advanced disease |
Smoking status |
85% never-smokers |
Significant history |
Lung transplant recipient: ∼50% |
Bone marrow transplant: 6% |
Prior exposure to chemotherapy or alkylating agents: 10% |
Family history of pulmonary fibrosis: 9% |
Idiopathic in an estimated 10–30% |
Imaging: chest radiograph and HRCT |
Bilateral apical (upper lobe + pleural dome) thickening invading the lung |
Generalised loss of volume |
The lower pleural-pulmonary zones are less involved or spared |
Interstitial markings may be increased |
Hila progressively retracted upwards |
Uni- or bilateral spontaneous partial pneumothoraces are common |
Chest ultrasound |
No data in humans at present |
Noninvasive imaging methods have been described in a donkey with PPFE [17] |
Pulmonary physiology |
Restrictive to markedly restrictive |
Obstruction present in some patients |
Over time, progressive hypoxaemia and hypercarbia may develop |
Bronchoalveolar lavage |
No consistent shifts in macrophages, lymphocytes, neutrophils or eosinophils |
PET scan |
No consistent data at present |
Pathology findings |
Upper zone collagenous fibrosis of the visceral pleura with haphazardly arranged elastic fibres |
Biopsy is NOT a prerequisite for PPFE diagnosis |
Subpleural intra-alveolar fibrosis with a sharp boundary with the underlying lung |
Alveolar septal elastosis |
Sparing of the parenchyma away from area of pleural thickening |
Mild, patchy lymphoplasmocytic infiltrates |
Fibroblastic foci are rare or absent |