This consensus document is intended to serve 3 functions. First, it standardizes the
criteria for diagnosis of chronic graft-versus-host disease (GVHD). Second, it proposes
a new clinical scoring system (0-3) that describes the extent and severity of chronic
GVHD for each organ or site at any given time, taking functional impact into account.
Third, it proposes new guidelines for global assessment of chronic GVHD severity that
are based on the number of organs or sites involved and the degree of involvement
in affected organs (mild, moderate, or severe). Diagnosis of chronic GVHD requires
the presence of at least 1 diagnostic clinical sign of chronic GVHD (e.g., poikiloderma
or esophageal web) or the presence of at least 1 distinctive manifestation (e.g.,
keratoconjunctivitis sicca) confirmed by pertinent biopsy or other relevant tests
(e.g., Schirmer test) in the same or another organ. Furthermore, other possible diagnoses
for clinical symptoms must be excluded. No time limit is set for the diagnosis of
chronic GVHD. The Working Group recognized 2 main categories of GVHD, each with 2
subcategories. The acute GVHD category is defined in the absence of diagnostic or
distinctive features of chronic GVHD and includes (1) classic acute GVHD occurring
within 100 days after transplantation and (2) persistent, recurrent, or late acute
GVHD (features of acute GVHD occurring beyond 100 days, often during withdrawal of
immune suppression). The broad category of chronic GVHD includes (1) classic chronic
GVHD (without features or characteristics of acute GVHD) and (2) an overlap syndrome
in which diagnostic or distinctive features of chronic GVHD and acute GVHD appear
together. It is currently recommended that systemic therapy be considered for patients
who meet criteria for chronic GVHD of moderate to severe global severity.