Abstract
ObjectivesPleuroparenchymal fibroelastosis (PPFE) is a rare idiopathic interstitial pneumonia (IIP) with variable clinical and radiological features. Diagnosis is based on histology obtained by surgical lung biopsy, which is associated with significant mortality and morbidity. This study aims to briefly review PPFE and discuss the role of CT-guided transthoracic core lung biopsy (TTB) in its diagnosis.Materials/MethodsFour cases of PPFE diagnosed at our institution with TTB are reported and discussed.ResultsClinical, radiological and histological features are in agreement with the previous literature cases. TTB provided the diagnosis in all cases. Iatrogenic pneumothorax was the main complication in all patients. Placement of a chest tube was needed in three patients. An overlap between PPFE and other interstitial lung diseases (ILD) was documented.ConclusionPPFE is an underdiagnosed IIP, so radiologist awareness of it needs to be widespread in patients with fibrosis with apical-caudal distribution. Coexistence of different lung diseases strengthens the idea of a predisposing factor. TTB proved to be a good diagnostic tool and can be considered the first choice for invasive assessment of these patients. PFFE has a variable course with no established therapeutic options; therefore a multidisciplinary team is crucial in the approach to patients with ILD.Main messages/Teaching Points• PPFE should be considered in the differential diagnosis of fibrosis with apical-caudal distribution.• CT-guided TTB can be considered the first choice for invasive assessment of PPFE.• Site of biopsy has to be chosen carefully in order not to miss PPFE.• Coexistence of different lung diseases strengthens the idea of a predisposing factor.• A multidisciplinary team is crucial in the approach to patients with ILD.
Highlights
Pleuroparenchymal fibroelastosis (PPFE) is a rare idiopathic interstitial pneumonia (IIP) first described as idiopathic pulmonary upper lobe fibrosis in 1992 in the Japanese literature by Aminati et al [1] and with approximately 70 patients described in the English-language literature until now [1,2,3,4,5,6,7,8,9,10,11]
The aetiology of PPFE is considered idiopathic in most cases, an association with a wide range of factors has been reported such as genetic predisposition; recurrent lower respiratory tract infections; underlying diseases or conditions such as collagen vascular diseases, bonemarrow transplantation or lung transplant patients suffering from restrictive allograft syndrome [5,6,7,8]
Patients 3 and 4 had occupational inhalation exposure: patient 3 has current environmental inhalation exposure and familiar history of interstitial lung disease; patient 4 had a previous diagnosis of chronic hypersensitivity pneumonitis
Summary
Pleuroparenchymal fibroelastosis (PPFE) is a rare idiopathic interstitial pneumonia (IIP) first described as idiopathic pulmonary upper lobe fibrosis in 1992 in the Japanese literature by Aminati et al [1] and with approximately 70 patients described in the English-language literature until now [1,2,3,4,5,6,7,8,9,10,11] This entity is characterised by fibrotic and elastotic thickening of the pleura and subpleural lung parenchyma, mainly in the upper lobes [4]. No specific data related with survival is defined, but an overall bad prognosis can be assumed based on available data
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