Classification systems for chronic graft-versus-host disease

Abstract

Chronic graft versus host disease (GVHD) is a major cause of morbidity and mortality after allogeneic hematopoietic cell transplantation. Clinically, chronic GVHD is a pleiotropic, multiorgan syndrome involving tissue inflammation and fibrosis that often results in permanent organ dysfunction. Chronic GVHD is fundamentally caused by replacement of the host's immune system with donor cells, although the heterogeneity of clinical manifestations suggests that patient, donor, and transplant factors modulate the phenotype. The diagnosis of chronic GVHD and determination of treatment response largely rely on clinical examination and patient interview. The 2005 and 2014 National Institutes of Health Consensus Development Projects on Criteria for Clinical Trials in Chronic GVHD standardized the terminology around chronic GVHD classification systems to ensure that a common language and procedures are being used in clinical research. This review provides a summary of these recommendations and illustrates how they are being used in clinical research and the potential for their use in clinical care.

Figure 1.

Acute, late acute, chronic overlap, and classic chronic GVHD. The box sizes do not reflect prevalence.

Figure 2.

Diagnostic skin and mouth manifestations. (A) Lichen planus and poikiloderma. (B) Fasciitis and sclerosis. (C) Sclerosis. (D) Oral lichen planus.

Figure 3.

2014 NIH skin score. BSA, body surface area.

Figure 4.

Calculation of mild, moderate, and severe global severity, with examples. If the entire abnormality in an organ is noted to be unequivocally explained by a non-GVHD documented cause, that organ is not included for calculation of the global severity. If the abnormality in an organ is attributed to multifactorial causes (GVHD plus other causes) the scored organ will be used for calculation of the global severity regardless of the contributing causes (no downgrading of organ severity score). ADL, activities of daily living; FEV1, forced expiratory volume in 1 second.