Restrictive allograft syndrome (RAS)

Summary

In the 2019 ISHLT phenotype scheme, as tabulated in an open-access review, RAS is the phenotype with restrictive findings (a decline in total lung capacity of at least 10 percent from baseline) together with parenchymal opacities or pleural thickening on computed tomography, without obstruction. An open-access 2020 review characterises RAS as a diffuse fibrotic process across the airway, pleura, septum, alveoli and vasculature, in contrast to the airway-centred process of BOS. RAS was described as a distinct form in 2011, and a companion consensus report in 2019 gave an update. The consensus wording itself was not opened for this record.

Sources and links

• Glanville et al. 2019, RAS update (DOI) (primary)
• Verleden et al. 2019 (DOI)
• Yoshiyasu and Sato 2020, phenotype table (open access)
• Sato 2020 (open access)

Related

Links from this record

• defined in: Chronic lung allograft dysfunction: Definition and update of restrictive allograft syndrome: A consensus report from the Pulmonary Council of the ISHLT
• defined in: Chronic lung allograft dysfunction: Definition, diagnostic criteria, and approaches to treatment: A consensus report from the Pulmonary Council of the ISHLT
• first described in: Restrictive allograft syndrome (RAS): A novel form of chronic lung allograft dysfunction
• summarised in: Chronic lung allograft dysfunction post-lung transplantation: The era of bronchiolitis obliterans syndrome and restrictive allograft syndrome
• summarised in: Bronchiolitis obliterans syndrome and restrictive allograft syndrome after lung transplantation: why are there two distinct forms of chronic lung allograft dysfunction?

Linked from (derived)

• proposed to be refined by: Molecular endotypes of CLAD (proposed framework)
• used to define: Chest CT: parenchymal patterns and density in CLAD
• used to define: Spirometry: FEV1 decline from baseline in the ISHLT 2019 CLAD definition
• compared in: Retransplantation
• reported association: Acute cellular rejection
• reported association: Airway colonisation: Pseudomonas and fungal infection
• reported association: Antibody-mediated rejection and donor-specific antibodies
• reported association: Cytomegalovirus and respiratory infection
• reviews: Acute Rejection and Chronic Lung Allograft Dysfunction: Obstructive and Restrictive Allograft Dysfunction
• compares: Donor-derived cell-free DNA in chronic lung allograft dysfunction phenotypes: a pilot study
• discusses: Clinical predictors for restrictive allograft syndrome: A nested case-control study.
• discusses: A transcriptomic atlas of chronic lung allograft dysfunction.
• classifies: Phenotyping CLAD after single lung transplant: Limits and prognostic assessment of the 2019 ISHLT classification system
• discusses: CLAD: translating basic science to clinical practice
• discusses: Identification and characterization of inflammatory LILR and fibrotic SPP1 macrophages in chronic lung allograft dysfunction
• describes: Chronic lung allograft dysfunction: Definition and update of restrictive allograft syndrome: A consensus report from the Pulmonary Council of the ISHLT
• discusses: Quantitative chest CT for subtyping chronic lung allograft dysfunction and its association with survival.
• discusses: Distinct fibrotic, epithelial and immune transcriptomic programs in phenotypes of chronic lung allograft dysfunction
• compares: Ventilatory capacity in CLAD is driven by dysfunctional airway structure
• compares: Epithelial cell death markers in bronchoalveolar lavage correlate with chronic lung allograft dysfunction subtypes and survival in lung transplant recipients-a single-center retrospective cohort study
• compares: Risk assessment of chronic lung allograft dysfunction phenotypes: Validation and proposed refinement of the 2019 International Society for Heart and Lung Transplantation classification system
• compares: Pulmonary epithelial markers in phenotypes of chronic lung allograft dysfunction
• models features of: Spectrum of chronic lung allograft pathology in a mouse minor-mismatched orthotopic lung transplant model
• discusses: Periostin defines a pathological fibroblast program enriched in restrictive allograft syndrome
• describes tissue findings of: Restrictive allograft syndrome post lung transplantation is characterized by pleuroparenchymal fibroelastosis
• discusses: Spectrum of chronic lung allograft dysfunction pathology in human lung transplantation.
• compares: Detailed cellular and spatial characterization of chronic lung allograft dysfunction using imaging mass cytometry
• describes: Restrictive allograft syndrome (RAS): A novel form of chronic lung allograft dysfunction
• describes: Bronchiolitis obliterans syndrome and restrictive allograft syndrome after lung transplantation: why are there two distinct forms of chronic lung allograft dysfunction?
• reports outcomes of: Survival determinants in lung transplant patients with chronic allograft dysfunction
• compares risk factors of: Bronchiolitis obliterans syndrome and restrictive allograft syndrome: do risk factors differ?
• discusses: A new classification system for chronic lung allograft dysfunction
• compares: Impact of CLAD Phenotype on Survival After Lung Retransplantation: A Multicenter Study
• reviews: Restrictive chronic lung allograft dysfunction: Where are we now?
• reviews: Chronic lung allograft dysfunction phenotypes and treatment
• associates with: Donor-specific and -nonspecific HLA antibodies and outcome post lung transplantation
• defines: Chronic lung allograft dysfunction: Definition, diagnostic criteria, and approaches to treatment: A consensus report from the Pulmonary Council of the ISHLT
• discusses: Chronic lung allograft dysfunction and restrictive allograft syndrome: are phenotypes robust and helpful?
• describes: Chronic lung allograft dysfunction post-lung transplantation: The era of bronchiolitis obliterans syndrome and restrictive allograft syndrome
• profiles: GEO GSE284081: single-nucleus RNA-seq of the fibrotic front in restrictive allograft syndrome
• affects: Clinical CLAD phenotypes are hard to assign reproducibly
• affects: No therapy of proven benefit for established CLAD

Known gaps in this record

• quotation of the consensus wording (full text not opened)