Restrictive allograft syndrome post lung transplantation is characterized by pleuroparenchymal fibroelastosis

Ofek, Efrat; Sato, Masaaki; Saito, Tomohito; Wagnetz, Ute; Roberts, Heidi C.; Chaparro, Cecilia; Waddell, Thomas K.; Singer, Lianne G.; Hutcheon, Michael; Keshavjee, Shaf; Hwang, David

doi:10.1038/modpathol.2012.171

Cited by 208 publications

(199 citation statements)

References 18 publications

(35 reference statements)

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“…This additional scar tissue was mainly situated in the upper lobes (as seen in figure 1d). The reason is unknown, however in idiopathic pleuroparenchymal fibroelastosis, which seems to be similar to RAS and where mainly apical fibrosis is also seen, venous and arterial fibrosis was seen in the majority of patients [5,18]. This might indicate hypoperfusion and ischaemia of these areas, although the mechanism of this is unknown.…”

Section: Discussionmentioning

confidence: 99%

“…In contrast, RAS is defined by the presence of a mixed restrictive and obstructive pattern of lung physiology, histopathological evidence of pleuroparenchymal fibro-elastosis as well as radiological evidence of interstitial fibrosis. Roughly speaking, approximately 70% of post-transplant patients affected by CLAD have BOS and 30% have the RAS clinical phenotype of CLAD [2,3,5,6]. Compared with the much better known BOS clinical phenotype, patients with RAS experience a worse prognosis (3.5 versus 1.5 years); the reasons for this difference in prognosis are poorly understood [7].…”

Section: Introductionmentioning

confidence: 99%

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Linking clinical phenotypes of chronic lung allograft dysfunction to changes in lung structure

et al. 2015

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Chronic lung allograft dysfunction (CLAD) remains the major barrier to long-term success after lung transplantation. This report compares gross and microscopic features of lungs removed from patients receiving a redo-transplant as treatment for CLAD.Lungs donated by patients with either the bronchiolitis obliterans syndrome (BOS) or restrictive allograft syndrome (RAS) phenotype of CLAD and appropriate control lungs (eight per group) were airinflated, frozen solid and kept frozen while a multi-detector computed tomography (MDCT) was obtained. The lung was then cut into 2-cm thick transverse slices and sampled for micro-CT and histopathology.The MDCT showed reduced lung volume with increased lung weight and density in RAS versus BOS and control ( p<0.05). Although pre-terminal bronchioles were obstructed in both phenotypes, RAS lungs showed a reduction of pre-terminal bronchioles ( p<0.01). Micro-CT and matched histopathology showed that RAS was associated with reduced numbers of terminal bronchioles/lung compared to BOS and controls ( p<0.01), with expansion of the interstitial compartment and obliteration of the alveolar airspaces by fibrous connective tissue.RAS is associated with greater destruction of both pre-terminal and terminal bronchioles. Additionally, the interstitial compartments are expanded and alveolar airspaces are obliterated by accumulation of fibrous connective tissue. @ERSpublications Restrictive allograft syndrome is associated with greater destruction of both pre-terminal and terminal bronchioles

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Linking clinical phenotypes of chronic lung allograft dysfunction to changes in lung structure

et al. 2015

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“…8 In fact, areas of diffuse alveolar damage in patients with restrictive allograft syndrome after lung transplantation with ensuing PPFE appear to support this hypothesis. 15 Nonetheless, whatever the pathogenesis may be, it remains clear that once PPFE pattern is identified, 3,5 the disease is often rapidly progressive, with frequent complication of pneumothorax and poor prognosis. No effective therapy is available at this juncture, 5 and hence the importance of diagnosing this entity in conjunction with clinical, radiologic, and pathologic findings.…”

Section: Discussionmentioning

confidence: 99%

Pleuroparenchymal Fibroelastosis of the Lung: A Review

Cheng¹,

Chuah

2016

Archives of Pathology &Amp; Laboratory Medicine

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Described in Japan by Amitani et al in 1992, the entity of idiopathic upper lobe fibrosis was subsequently given the name pleuroparenchymal fibroelastosis (PPFE) in the English-speaking world. Pleuroparenchymal fibroelastosis is believed to be a rare disease characterized by a fibrosing process affecting the pleura and the subpleural lung parenchyma, with a predilection for the upper lobes. Uniquely, the fibrosing process is elastotic in nature, being associated with intra-alveolar fibrosis. The etiology of PPFE is unclear at this juncture, with many cases being considered as idiopathic forms of the disease. Conditions associated with PPFE include infections, bone marrow transplantation, and autoimmunity. In this review, we explore the clinical, radiologic, and pathologic features associated with PPFE in light of current understanding of the disease. Recent studies implicated that PPFE may not be as uncommon as claimed. The various differential diagnoses and implications of diagnosing PPFE are discussed.

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“…We used the term CLAD as not all patients immediately develop the typical restrictive pulmonary function defect and we aimed to describe the radiological changes starting from the moment that the FEV 1 consistently remained under 20% of the best post-operative values. Pathology reports were available when patients underwent re-transplantation, open lung biopsy or autopsy and the typical findings were previously described by Ofek et al and include extensive alveolar fibrosis, septal thickening but also obliterative bronchiolitis (7).…”

Section: Patient Characteristicsmentioning

confidence: 99%

Functional and computed tomographic evolution and survival of restrictive allograft syndrome after lung transplantation

Verleden

Jong

Ruttens

et al. 2014

The Journal of Heart and Lung Transplantation

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Restrictive allograft syndrome post lung transplantation is characterized by pleuroparenchymal fibroelastosis

Cited by 208 publications

References 18 publications

Linking clinical phenotypes of chronic lung allograft dysfunction to changes in lung structure

Linking clinical phenotypes of chronic lung allograft dysfunction to changes in lung structure

Pleuroparenchymal Fibroelastosis of the Lung: A Review

Functional and computed tomographic evolution and survival of restrictive allograft syndrome after lung transplantation

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