Chronic Lung Allograft Dysfunction: Definition and Update of Restrictive Allograft Syndrome

A Consensus Report from the Pulmonary Council of the ISHLT

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Published 3 April 2019

Allan R. Glanville, MBBS, MD; Geert M. Verleden, MD, PhD; Jamie L. Todd, MD; Christian Benden, MD, FCCP; Fiorella Calabrese, MD; Jens Gottlieb, MD; Ramsey R. Hachem, MD; Deborah Levine, MD; Federica Meloni, MD, PhD; Scott M. Palmer, MD, MHS; Antonio Roman, MD; Masaaki Sato, MD, PhD; Lianne G. Singer, MD, FRCPC; Sofya Tokman, MD; Stijn E. Verleden, PhD; Jan von der Thüsen, MBBS, PhD; Robin Vos, MD, PhD; Gregory Snell, MD

J Heart Lung Transplant. 2019 May;38(5):483-92

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In 1993, the International Society for Heart and Lung Transplantation (ISHLT) established an ad-hoc working group that published a working formulation to describe chronic dysfunction of the allograft. The group concluded that a decline in the forced expiratory volume in 1 second (FEV1) was the most reliable and consistent indicator of allograft dysfunction after other identifiable causes were excluded. The acronym BOS (bronchiolitis obliterans syndrome) was introduced to describe such dysfunction, but restrictive physiology related to parenchymal with or without pleural fibrosis was not included in the syndrome.

In 2011, Sato et al introduced the term restrictive allograft syndrome (RAS). In the series, RAS was diagnosed in 30% of bilateral LTx patients with CLAD. The diagnosis was based on finding a restrictive ventilatory defect, defined as FEV1≤80% and total lung capacity (TLC) ≤90% of baseline values. Many patients with RAS had radiographic findings of interstitial or ground-glass opacities, of whom 41% had upper zone involvement. Patients with RAS had an inferior median survival from diagnosis compared to patients with BOS.

In this article, authors provide a consensus standardized definition of RAS for use across centers; provide a state-of-the-art literature review supporting the development of the definition; and describe in detail the current understanding of clinical, physiologic, radiologic, and histologic manifestations of RAS. In addition, available data on risk factors and treatment approaches to RAS are discussed and key research priorities for future consideration in areas where critical data are still lacking are identified.

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To best understand how RAS fits in with the revised definition of CLAD, it is strongly advised to read the current RAS article in conjunction with the CLAD consensus article.

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