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Restrictive allograft syndrome and idiopathic pleuroparenchymal fibroelastosis: do they really have the same histology?
Author(s) -
Montero Maria A,
Osadolor Tina,
Khiroya Reena,
Salcedo Maria Teresa,
Robertus Jan L,
Rice Alexandra,
Nicholson Andrew G,
Roman Antonio,
Monforte Victor
Publication year - 2017
Publication title -
histopathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.626
H-Index - 124
eISSN - 1365-2559
pISSN - 0309-0167
DOI - 10.1111/his.13171
Subject(s) - medicine , pathology , fibrosis , histology , histopathology , pathogenesis , usual interstitial pneumonia , lung transplantation , lung , bronchiolitis obliterans , bronchiolitis , transplantation , biopsy , idiopathic pulmonary fibrosis , respiratory system
Aims Restrictive allograft syndrome ( RAS ) and idiopathic pleuroparenchymal fibroelastosis ( IPPFE ) are two different diseases reported to share the same histology. RAS relates to chronic allograft dysfunction in lung transplantation, with IPPFE being a rare condition in native lungs. Our aim is to compare their histologies alongside biopsies of usual interstitial pneumonia ( UIP ), to determine if there are differences that might help to elucidate the pathogenesis. Methods and results We selected four postmortem allograft lungs from patients who developed a clear clinical RAS pattern, five biopsies diagnosed as IPPFE , five UIP biopsies and five sections of normal lung. Histopathological features were described without knowledge of clinical and radiological features. Both RAS allografts and IPPFE biopsies showed intra‐alveolar fibrosis and elastosis ( IAFE ), but RAS allografts also showed concomitant obliterative bronchiolitis, vascular lymphoplasmacytic inflammation within fibrointimal thickening, less fibroblastic foci ( FF ) at the advancing edge of the fibrosis (one against 14.4 FF in 2 mm 2 ) and a slight reduction of the capillary network compared to UIP ( P = 0.07) and controls ( P = 0.06). The main differences seen in UIP were the lack of IAFE and the presence of honeycomb change. Conclusions RAS and PPFE histopathology both show IAFE , but display various differences, particularly in their vascular morphology that may allow further understanding of pathogenesis.