Pleuroparenchymal Fibroelastosis: Role of High-resolution Computed Tomography (HRCT) and CT-guided Transthoracic Core Lung Biopsy

Overview

Journal Insights Imaging

Publisher Springer

Specialty Radiology

Date 2015 Nov 19

PMID 26577607

Citations 12

Authors

Catia Esteves

Francisco R Costa

Margarida T Redondo

Conceicao S Moura

Susana Guimaraes

Antonio Morais

Jose M Pereira

Affiliations

Soon will be listed here.

Abstract

Objectives: Pleuroparenchymal 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/methods: Four cases of PPFE diagnosed at our institution with TTB are reported and discussed.

Results: Clinical, 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.

Conclusion: PPFE 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.

Citing Articles

Multidisciplinary Approach to the Diagnosis of Idiopathic Interstitial Pneumonias: Focus on the Pathologist's Key Role.

Luca S, Pagliuca F, Perrotta F, Ronchi A, Mariniello D, Natale G Int J Mol Sci. 2024; 25(7).

PMID: 38612431 PMC: 11011777. DOI: 10.3390/ijms25073618.

The world of rare interstitial lung diseases.

Buschulte K, Cottin V, Wijsenbeek M, Kreuter M, Diesler R Eur Respir Rev. 2023; 32(167).

PMID: 36754433 PMC: 9910344. DOI: 10.1183/16000617.0161-2022.

Advanced Warning of Aortic Dissection on Non-Contrast CT: The Combination of Deep Learning and Morphological Characteristics.

Yi Y, Mao L, Wang C, Guo Y, Luo X, Jia D Front Cardiovasc Med. 2022; 8:762958.

PMID: 35071345 PMC: 8767113. DOI: 10.3389/fcvm.2021.762958.

Pleuroparenchymal fibroelastosis: from diagnosis to pulmonary rehabilitation.

Candemir I, Ergun P, Demir N BMJ Case Rep. 2021; 14(6).

PMID: 34130969 PMC: 8208016. DOI: 10.1136/bcr-2020-236754.

HRCT Diagnosis of Pleuroparenchymal fibroelastosis: Report of two cases.

Morshid A, Moshksar A, Das A, Duarte A, Palacio D, Villanueva-Meyer J Radiol Case Rep. 2021; 16(6):1564-1569.

PMID: 33981378 PMC: 8085788. DOI: 10.1016/j.radcr.2021.03.051.

References

Kusagaya H, Nakamura Y, Kono M, Kaida Y, Kuroishi S, Enomoto N . Idiopathic pleuroparenchymal fibroelastosis: consideration of a clinicopathological entity in a series of Japanese patients. BMC Pulm Med. 2012; 12:72. PMC: 3539991. DOI: 10.1186/1471-2466-12-72. View

Macedo P, Coker R, Partridge M . Is there a uniform approach to the management of diffuse parenchymal lung disease (DPLD) in the UK? A national benchmarking exercise. BMC Pulm Med. 2007; 7:3. PMC: 1829398. DOI: 10.1186/1471-2466-7-3. View

Klose K, Biesterfeld S, Bocking A . [Percutaneous CT-controlled cutting-needle biopsy of diffuse interstitial and alveolar lung diseases--the technic and results]. Rofo. 1992; 157(1):26-33. DOI: 10.1055/s-2008-1032960. View

Becker C, Gil J, Padilla M . Idiopathic pleuroparenchymal fibroelastosis: an unrecognized or misdiagnosed entity?. Mod Pathol. 2008; 21(6):784-7. DOI: 10.1038/modpathol.2008.56. View

Redondo M, Melo N, Mota P, Jesus J, Moura C, Guimaraes S . Idiopathic pleuroparenchymal fibroelastosis: a rare but increasingly recognized entity. Rev Port Pneumol (2006). 2015; 21(1):41-4. DOI: 10.1016/j.rppnen.2014.04.008. View

Manhire A, Charig M, Clelland C, Gleeson F, Miller R, Moss H . Guidelines for radiologically guided lung biopsy. Thorax. 2003; 58(11):920-36. PMC: 1746503. DOI: 10.1136/thorax.58.11.920. View

von der Thusen J . Pleuroparenchymal Fibroelastosis: Its Pathological Characteristics. Curr Respir Med Rev. 2014; 9(4):238-247. PMC: 3949414. DOI: 10.2174/1573398X113096660025. View

Reddy T, Tominaga M, Hansell D, Von Der Thusen J, Rassl D, Parfrey H . Pleuroparenchymal fibroelastosis: a spectrum of histopathological and imaging phenotypes. Eur Respir J. 2012; 40(2):377-85. DOI: 10.1183/09031936.00165111. View

von der Thusen J, Hansell D, Tominaga M, Veys P, Ashworth M, Owens C . Pleuroparenchymal fibroelastosis in patients with pulmonary disease secondary to bone marrow transplantation. Mod Pathol. 2011; 24(12):1633-9. DOI: 10.1038/modpathol.2011.114. View

10.

Lohela P, Tikkakoski T, Ammala K, Strengell L, Suramo I, Repo U . Diagnosis of diffuse lung disease by cutting needle biopsy. Acta Radiol. 1994; 35(3):251-4. View

11.

Hirota T, Fujita M, Matsumoto T, Higuchi T, Shiraishi T, Minami M . Pleuroparenchymal fibroelastosis as a manifestation of chronic lung rejection?. Eur Respir J. 2013; 41(1):243-5. DOI: 10.1183/09031936.00103912. View

12.

Metzger F, Pernet D, Manzoni P, Ranfaing E, Dalphin J . [The contribution of CT-guided transthoracic lung biopsy to the diagnosis of organizing pneumonia]. Rev Mal Respir. 2008; 25(5):539-50. DOI: 10.1016/s0761-8425(08)71611-5. View

13.

Cuppens K, Verbeken E, Coolen J, Verschakelen J, Wuyts W . Idiopathic pleuroparenchymatous fibroelastosis: A case report and brief review of the literature. Respir Med Case Rep. 2015; 12:7-9. PMC: 4061435. DOI: 10.1016/j.rmcr.2013.12.005. View

14.

Blackhall V, Asif M, Renieri A, Civitelli S, Kirk A, Jilaihawi A . The role of surgical lung biopsy in the management of interstitial lung disease: experience from a single institution in the UK. Interact Cardiovasc Thorac Surg. 2013; 17(2):253-7. PMC: 3715203. DOI: 10.1093/icvts/ivt217. View

15.

Travis W, Costabel U, Hansell D, King Jr T, Lynch D, Nicholson A . An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013; 188(6):733-48. PMC: 5803655. DOI: 10.1164/rccm.201308-1483ST. View

16.

Piciucchi S, Tomassetti S, Casoni G, Sverzellati N, Carloni A, Dubini A . High resolution CT and histological findings in idiopathic pleuroparenchymal fibroelastosis: features and differential diagnosis. Respir Res. 2011; 12:111. PMC: 3197499. DOI: 10.1186/1465-9921-12-111. View

17.

Kramer M, Berkman N, Mintz B, Godfrey S, Saute M, Amir G . The role of open lung biopsy in the management and outcome of patients with diffuse lung disease. Ann Thorac Surg. 1998; 65(1):198-202. DOI: 10.1016/s0003-4975(97)01081-3. View

18.

Lorenz J, Blum M . Complications of percutaneous chest biopsy. Semin Intervent Radiol. 2011; 23(2):188-93. PMC: 3036363. DOI: 10.1055/s-2006-941449. View

19.

Frankel S, Cool C, Lynch D, Brown K . Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity. Chest. 2004; 126(6):2007-13. DOI: 10.1378/chest.126.6.2007. View

20.

Rena O, Casadio C, Leo F, Giobbe R, Cianci R, Baldi S . Videothoracoscopic lung biopsy in the diagnosis of interstitial lung disease. Eur J Cardiothorac Surg. 2000; 16(6):624-7. DOI: 10.1016/s1010-7940(99)00320-6. View