Cryobiopsies are diagnostic in Pleuroparenchymal and Airway-centered Fibroelastosis

Abstract

BackgroundIdiopathic pulmonary fibroelastosis (iPPFE) is a rare lung lesion characterized by pleural and subpleural parenchymal thickening due to accumulation of fibroelastotic tissue. Only recently, a few cases with a peribronchiolar distribution of fibroelastotic tissue have been reported. These lesions are more prominent in the upper lobes. Even though high resolution computed tomography (HRCT) scan features are considered characteristic, a histological confirmation is suggested, mainly when the clinical setting is not clearly defined. However, due to non-negligible complications, a surgical lung biopsy is not often recommended. The prognosis is usually poor and currently, the only effective treatment is lung transplantation.MethodPatients with a multidisciplinary diagnosis of iPPFE or airway-centered fibroelastosis (airway-centered FE), with histological confirmation by transbronchial cryobiopsy, were identified from an ongoing interstitial lung disease registry. Data on patient demographics, HRCT patterns, size and number of biopsies, histology patterns and complications were registered.ResultsSeven patients were diagnosed with iPPFE and one patient was diagnosed with airway-centered FE. Pneumothorax was documented in three cases, but none of them required a chest tube. No other complications during or after the procedure were observed.ConclusionThis study suggests that using cryobiopsies in the diagnostics of PPFE and airway-centered FE is safe and effective.

Pleuroparenchymal Fibroelastosis with a Predominantly Airway-centered Distribution: A Late Complication of Chemotherapy

“…Airway-centered fibroelastosis has recently been described as a unique and distinct entity in five non-smoking middleaged women with evidence of prominent airway-centered elastosis in their lung samples (9). Following the publication of this report, a few cases with similar characteristics have been reported (10)(11)(12). The clinical course of previously reported cases indicates that patients with airway-centered fibroelastosis have progressive disease and a poor prognosis.…”

“…The condition of our two patients also deteriorated rapidly, and they both died a few years following the onset of symptoms. Thus, recent reports have shown no consistency regarding a history of asthma or smoking in these patients (10)(11)(12). In a previous report, patients with airway-centered fibroelastosis were more likely to have asthma as a clinical symptom, and the authors did not describe pathological findings of asthmatype bronchitis/bronchiolitis (9).…”

“…Since this report, three patients diagnosed with airway-centered fibroelastosis were reported. Kronborg-White et al reported a 55-year-old man who was a former smoker and had been diagnosed with an airway-centered fibroelastosis of unknown etiology who did not complain of asthma but had alveolar consolidations around centriacinar regions mimicking sarcoidosis on radiological findings (10). Bargagli et al reported a 48-year-old non-smoking woman who was diagnosed with airway-centered fibroelastosis associated with non-necrotizing granulomas of unknown etiology (11).…”

Pleuroparenchymal fibroelastosis (PPFE) treated with lung transplantation and review of the literature

“…Whether a surgical lung biopsy can be obtained, a multidisciplinary discussion should be the cornerstone of making the PPFE diagnosis. Some case reports have also suggested a utility of transbronchial cryobiopsies and CT guided transthoracic needle aspirates for diagnosing PPFE 20 21…”

Transbronchial Cryobiopsies

“…86 Kronborg-White et al were able to diagnosis seven patients with PPFE based on a combination of TBLCB and MDD. 87 PAP is a rare disease characterized by abundant accumulation of surfactant material in the alveolar spaces and is often encountered in the setting of autoimmunity to granulocyte-macrophage colony stimulating factor. 88 Shen et al reported a case in a 54-year-old man where BAL and fTBB failed to confirm the diagnosis that was ultimately demonstrated by TBLCB.…”