Changes in Immunosuppressive Treatment of Chronic Graft-versus-Host Disease: Comparison of 2 Surveys within Allogeneic Hematopoietic Stem Cell Transplant Centers in Germany, Austria, and Switzerland

Abstract

Chronic graft-versus-host disease (cGVHD) remains the leading cause of late morbidity and mortality. Despite the growing number of treatment options in cGVHD, evidence remains sparse. The German-Austrian-Swiss GVHD Consortium performed a survey on clinical practice in treatment of cGVHD among transplant centers in Germany, Austria, and Switzerland in 2009 and 2018 and compared the results. The survey performed in 2009 contained 20 questions on first-line treatment and related issues and 4 questions on second-line scenarios followed by a survey on all systemic and topic treatment options known and applied, with 31 of 36 transplant centers (86%) responding. The survey in 2018 repeated 7 questions on first-line treatment and 3 questions on second-line scenarios followed by an updated survey on all current systemic treatment options known and applied, with 29 of 66 centers (43%) responding. In summary, the results show a large overlap of first-line treatment practice between centers and the 2 surveys because of a lack of new data that changes practice, except significant heterogeneity of treatment of cGVHD progressive onset type, which can be explained by the lack of trials focusing on this high-risk entity. In contrast, treatment options applied to second-line therapy vary considerably, with new agents like ibrutinib and ruxolitinib entering clinical practice. Moreover, treatment of bronchiolitis obliterans syndrome demonstrates heterogeneity in applied therapeutic options and sequence because of a lack of controlled data and different conclusions from already existing evidence. In summary, the survey results demonstrate an increasing number of treatment options applied to cGVHD accompanied by a significant heterogeneity in second-line treatment and underline the urgent need for clinical trials and registry analyses on rare entities with high mortality like progressive onset type and lung involvement of cGVHD.

Extracorporeal photopheresis in acute and chronic steroid‑refractory graft-versus-host disease: an evolving treatment landscape

“…Besides ruxolitinib ECP has been widely used for treatment of GvHD [33]. ECP, a cell-based immunotherapy that involves the reinfusion of autologous mononuclear cells after exposure to 8methoxypsoralen and ultraviolet A (UVA) light irradiation, is an established, clinically effective second-line therapy for SR acute and cGvHD (Table 1b) [34][35][36][37][38][39][40][41][42][43][44][45][46][47][48][49][50].…”

Challenges and concepts in the diagnosis and management of ocular graft-versus-host disease

“…Second line treatment is indicated in case of steroid-refractory chronic GHVD with an increasing number of treatment options ( 160 ). Up to now, there is no standard yet ( 161 ). Levels of evidence for efficacy and treatment costs vary considerably and numbers of patients reported for eye response are usually low ( 162 ).…”

Ibrutinib for Chronic Graft-Versus-Host Disease

“…One study described a survey of clinicians in 3 European countries and reported consistency in the approach to managing newly diagnosed CGvHD (i.e., usually corticosteroids), but found significant variability in the preferred approaches to second-and third-line treatment. 40 This lack of clinical consensus is thought to be driven largely by the limited body of available and/or robust evidence informing best practice for the management of treatment-refractory CGvHD. 40 This need for more (and more rigorous) evidence is a common theme in the literature describing the management of treatment-refractory CGvHD.…”

“…40 This lack of clinical consensus is thought to be driven largely by the limited body of available and/or robust evidence informing best practice for the management of treatment-refractory CGvHD. 40 This need for more (and more rigorous) evidence is a common theme in the literature describing the management of treatment-refractory CGvHD. 12,39 Likewise, additional studies are needed to inform the diagnosis and prevention of CGvHD and are critical areas of future research.…”