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

Abstract

Airway-centered fibroelastosis is a distinct entity characterized by prominent airway-centered elastosis of the upper lobe with little or no pleural involvement. Little is known regarding its etiology; however, it was reported to have an idiopathic or asthma-associated etiology. We document, for the first time, 2 women (19 and 60 years old) who developed pleuroparenchymal fibroelastosis with a predominantly airway-centered distribution as a late complication (6 and 9 years later, respectively) of chemotherapy. The disease rapidly progressed following the manifestation of symptoms, and they subsequently died (3 and 2 years later, respectively). Therefore, post-chemotherapy long-term monitoring for this disease is warranted.

Two cases of airway-centered fibroelastosis treated with an antifibrotic agent and corticosteroids

“…Airway-centered fibroelastosis is a newly recognized, rare form of ILD, with only nine cases reported in the English literature [ [1] , [2] , [3] , [4] ]. The cases are summarized in Table 1 .…”

“… Abbreviations: ALL, acute lymphoblastic leukemia; DLBCL, diffuse large B-cell lymphoma. References: patients 1–5 [ 1 ]; patient 6 [ 2 ]; patients 7, 8 [ 3 ]; patient 9 [ 4 ]. …”

“…Primary studies have described drug-induced PPFE. It has been noted that cyclophosphamide was used in the majority of cases of late-onset PPFE [ 3 ]. Katayama et al reported two cases of airway-centered fibroelastosis induced by chemotherapy with cyclophosphamide [ 3 ].…”

“…It has been noted that cyclophosphamide was used in the majority of cases of late-onset PPFE [ 3 ]. Katayama et al reported two cases of airway-centered fibroelastosis induced by chemotherapy with cyclophosphamide [ 3 ]. In this study, case 2 had undergone chemotherapy with cyclophosphamide, and the pathological and radiological findings were similar to those previously reported.…”

“…Some patients experienced acute attacks of wheezing and dyspnea [ 1 ]; however, the presentation of airway-centered fibroelastosis remains to be elucidated. This condition was first described in 2016 [ 1 ], and few cases have been reported worldwide [ [1] , [2] , [3] , [4] ]. Herein, we present two cases of airway-centered fibroelastosis to increase the awareness pertaining to this disease.…”