Diagnosis and treatment of pulmonary chronic GVHD: report from the consensus conference on clinical practice in chronic GVHD

G C Hildebrandt, T Fazekas, A Lawitschka, H Bertz, H Greinix, J Halter, S Z Pavletic, E Holler, D Wolff, G C Hildebrandt, T Fazekas, A Lawitschka, H Bertz, H Greinix, J Halter, S Z Pavletic, E Holler, D Wolff

Abstract

This consensus statement established under the auspices of the German working group on BM and blood stem cell transplantation (DAG-KBT), the German Society of Hematology and Oncology (DGHO), the Austrian Stem Cell Transplant Working Group, the Swiss Blood Stem Cell Transplantation Group (SBST) and the German-Austrian Pediatric Working Group on SCT (Päd-Ag-KBT) summarizes current evidence for diagnosis, immunosuppressive and supportive therapy to provide practical guidelines for the care and treatment of patients with pulmonary manifestations of chronic GVHD (cGVHD). Pulmonary cGVHD can present with obstructive and/or restrictive changes. Disease severity ranges from subclinical pulmonary function test (PFT) impairment to respiratory insufficiency with bronchiolitis obliterans being the only pulmonary complication currently considered diagnostic of cGVHD. Early diagnosis may improve clinical outcome, and regular post-transplant follow-up PFTs are recommended. Diagnostic work-up includes high-resolution computed tomography, bronchoalveolar lavage and histology. Topical treatment is based on inhalative steroids plus beta-agonists. Early addition of azithromycin is suggested. Systemic first-line treatment consists of corticosteroids plus, if any, continuation of other immunosuppressive therapy. Second-line therapy and beyond includes extracorporeal photopheresis, mammalian target of rapamycin inhibitors, mycophenolate, etanercept, imatinib and TLI, but efficacy is limited. Clinical trials are urgently needed to improve understanding and treatment of this deleterious complication.

Conflict of interest statement

Hildegard Greinix served as member of a steering committee for a chronic GVHD study of Therakos and received honoraria from Therakos for research presentations in corporate symposia at international scientific meetings. The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Diagnostic and clinical care work-up algorithm for lung injury following allo-SCT. (1) Routine PFT screening is recommended 3, 6, 9, 12, 18 and 24 months after allo-SCT and then once per year. (2)Impaired PFT should be at least a drop in FEV1 >5% over the last 12 months or a LFS ⩾2 or a FEV1 0.7. (3) The decision, whether a transbronchial or an open lung biopsy may be performed, has to be made carefully on a case by case basis in the context of radiographical findings, the risk of potential complications and the expected clinical consequences to be made depending upon biopsy results.
Figure 2
Figure 2
Therapeutic algorithm for lung injury following allo-SCT. TKI=tyrosine kinase inhibitor.

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Source: PubMed

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