Diagnosis and manifestations of chronic graft-versus-host disease

Chronic graft-versus-host disease (GVHD) is an immunoregulatory post-transplant disorder which shares features of autoimmunity and immunodeficiency. This chapter describes the major recommendations of the National Institutes of Health (NIH) consensus process on chronic GVHD conducted in 2004 and 2005 with respect to new guidelines for the diagnosis and staging of GVHD. Acute and chronic GVHD were redefined to emphasize the central importance of distinct diagnostic manifestations differentiating the two entities, rather than time of onset post-transplant. The diagnosis of chronic GVHD requires, at a minimum, the presence of at least one diagnostic clinical sign of chronic GVHD or the presence of at least one distinctive clinical manifestation confirmed by biopsy or other relevant tests in the same or another organ. Diagnostic criteria include signs and symptoms which are sufficient alone to establish the diagnosis of chronic GVHD. They can involve the skin and appendages, mouth, eyes, female genitalia, esophagus, lungs, and connective tissues. The NIH consensus project also generated a chronic GVHD scoring system and suggestions for overall grading of severity of chronic GVHD. The expectation is that the provisional guidelines described here will be widely used and refined with additional observer experience.