Diagnosis and treatment of bronchiolitis obliterans syndrome accessible universally

摘要

The incidence of bronchiolitis obliterans syndrome (BOS), a devastating manifestation of chronic graft-versus-host-disease, may rise globally due to steady increases in utilization of allogeneic hematopoietic cell transplantation (HCT). Though some advances have occurred in the past decade regarding understanding of the pathogenesis, diagnosis and treatment of BOS, the overall mortality and morbidity remain very high. We sought to determine the current diagnostic and therapeutic challenges, which can potentially hinder optimal management of BOS both in developed and developing countries. We performed a comprehensive systematic review of both modern diagnostic modalities and treatments and then assessed which of them would be universally accessible. The 2014 National Institutes of Health chronic GVHD criteria remains the gold standard tool for diagnosing BOS. Important elements of treatment involve early and accurate detection, as well as utilizing the treatment modalities with known (but variable efficacy) e.g. fluticasone-azithromycin-montelukast [FAM] combination, etanercept, extra-corporeal photopheresis [ECP], lung transplantation, and prompt treatment of complications including infections in sufferers of BOS. Our results indicate that optimum diagnostic tools are not readily available in some parts of the world for early detection, which include a lack of CT scanners, unavailability of pulmonary function testing tools, absence of sub-specialists, lack of certain effective treatments and late referral for lung transplant. We present a systematic review of current literature along with recommendations for available therapies to guide practitioners to optimize the long-term outcomes in HCT survivors regardless of access to experts and expensive therapies.