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Chronic graft-versus-host disease

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Added 2024 ERS/EBMT and 2012 BCSH/BSBMT guidelines for the diagnosis and management of chronic graft-versus-host disease.

Background

Overview

Definition
Chronic GvHD is a serious complication that occurs after allo-SCT, leading to significant morbidity and mortality.
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Pathophysiology
The pathophysiology of chronic GvHD involves a coordinated response from donor T-cells. The donor T-cells recognize the host tissues as foreign, initiating an immune response. B cells also play a significant role in chronic GvHD, as evidenced by the presence of autoantibodies and alloantibodies contributing to immune dysregulation.
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Epidemiology
Chronic GvHD is reported to occur in approximately 50% of all patients following allo-SCT and is increased with the use of unrelated and/or mismatched donors, reduced intensity conditioning regimens, and peripheral blood stem cells. The incidence of chronic GvHD in children is estimated at 20-40%.
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Risk factors
Risk factors for chronic GvHD include the degree of donor-recipient HLA mismatch, history of acute GvHD, and the use of peripheral blood stem cells as the source of the graft. Other factors such as recipient age, disease type, and conditioning regimen can also influence the risk.
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Disease course
Chronic GvHD can affect multiple organ systems, including the skin and nails, liver, lungs (bronchiolitis obliterans syndrome), gastrointestinal tract, oral cavity, muscle and fascia, and eyes. It often resembles an autoimmune disorder, with manifestations such as lichenoid lesions, hyperkeratotic plaques, and limited oral aperture secondary to sclerosis.
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Prognosis and risk of recurrence
Chronic GvHD is associated with a high risk of morbidity and mortality. The prognosis varies based on the extent of organ involvement and response to treatment, with a reported cure rate of 50% and a mortality rate of 40% at 7 years.
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Guidelines

Key sources

The following summarized guidelines for the evaluation and management of chronic graft-versus-host disease are prepared by our editorial team based on guidelines from the European Respiratory Society (ERS/EBMT 2024), the British Committee for Standards In Haematology (BCSH/BSBMT 2012), and the American Society for Gastrointestinal Endoscopy (ASGE 2010).
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Screening and diagnosis

Diagnostic criteria: as per BCSH/BSBMT 2012 guidelines, diagnose chronic GvHD and overlap syndrome primarily using clinical criteria, supported by biopsy when possible.
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Diagnostic investigations

Initial evaluation
As per BCSH/BSBMT 2012 guidelines:
Use the NIH consensus criteria to classify chronic GvHD as mild, moderate, or severe.
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Assess all patients presenting with signs or symptoms suggestive of chronic GvHD in one organ for involvement of other organs.
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Diagnostic procedures

Gastrointestinal endoscopy: as per BCSH/BSBMT 2012 guidelines, obtain upper gastrointestinal endoscopy (with duodenal aspirate and biopsies) and lower gastrointestinal endoscopy (flexible sigmoidoscopy and biopsy) in patients with diarrhea in the absence of associated jaundice or rash suggestive of GvHD.
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Medical management

General principles: as per BCSH/BSBMT 2012 guidelines, review patients with chronic GvHD with a team experienced in managing transplant-related complications.
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  • Stepwise management (first-line therapy)

  • Stepwise management (second-line therapy)

  • Stepwise management (third-line therapy)

  • Management of pulmonary chronic GvHD (pharmacotherapy)

  • Management of pulmonary chronic GvHD (extracorporeal photopheresis)

  • Management of pulmonary chronic GvHD (lung transplantation)

  • Management of pulmonary chronic GvHD (supportive therapy)

  • Management of pulmonary chronic GvHD (follow-up)

  • Management of gastrointestinal chronic GvHD

  • Management of liver chronic GvHD

  • Management of oral chronic GvHD

  • Management of cutaneous chronic GvHD (referral)

  • Management of cutaneous chronic GvHD (topical therapy)

  • Management of cutaneous chronic GvHD (systemic therapy)

  • Management of cutaneous chronic GvHD (extracorporeal photopheresis)

  • Management of cutaneous chronic GvHD (physiotherapy)

  • Management of musculoskeletal chronic GvHD

  • Management of ocular chronic GvHD

Preventative measures

Infection prophylaxis: as per BCSH/BSBMT 2012 guidelines, consider administering prophylaxis against viral, fungal, Pneumocystis jiroveci, and S. pneumoniae infections in all patients receiving immunosuppressive therapy for chronic GvHD.
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  • Routine immunizations