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There is an agreed way to say how bad chronic GvHD is, and every trial, drug label and treatment decision uses it. Each affected organ scores 0 to 3, and the pattern gives an overall verdict of mild, moderate or severe. Worth asking: which organs are scored, what each score is, and what the global severity is.
The framework comes from the 2005 National Institutes of Health consensus conference and was revised by the 2014 Diagnosis and Staging Working Group. The 2014 report states that it maintains the framework of the prior consensus with refinement based on new evidence, and that revisions address areas of controversy or confusion such as the overlap chronic GvHD subcategory and the distinction between active disease and past tissue damage. Diagnostic criteria for the mouth, eyes, genitalia and lungs were revised, and the report says that attribution of organ-specific abnormalities to chronic GvHD has been addressed, a change the authors describe as a paradigm shift that provides greater specificity and more accurately measures the global burden of disease attributed to GvHD.
The practical shape of the system is this. Diagnosis rests on at least one diagnostic manifestation, a sign that is sufficient on its own, or a distinctive manifestation plus confirmation by biopsy, laboratory test or imaging. Eight organs or sites are scored from 0, meaning no involvement, to 3, meaning severe: skin, mouth, eyes, gastrointestinal tract, liver, lungs, joints and fascia, and genital tract. A performance score and a weight-loss assessment accompany them. The global severity is derived from the pattern: mild disease involves one or two organs at score 1 with no lung involvement; moderate involves three or more organs at score 1, or any organ at score 2, or lung score 1; severe means any organ at score 3, or lung score 2 or 3. Lung involvement moves the verdict up a grade at every level, which is the system's way of saying that the lungs are the organ that changes the outlook most.
Time no longer defines the disease. Chronic GvHD is diagnosed by its manifestations rather than by the hundred-day mark that once separated acute from chronic; a person can develop classic acute GvHD late, or have features of both at once, which the criteria call overlap syndrome. The system also separates active inflammation that may respond to treatment from fixed damage that will not, because treating fibrosis that has already set with more immunosuppression adds risk without benefit.
Response is scored by a companion document, the NIH response criteria, which trials use as their endpoint. The axatilimab label, for example, defines overall response as complete or partial response according to the 2014 NIH consensus response criteria. The modified Lee Symptom Scale, a patient-reported measure, sits alongside the clinician scores and is a key secondary endpoint in the ruxolitinib and axatilimab trials, which matters because a person can feel better before a clinician can measure it, or the reverse.
The honest limits: the criteria are a consensus, not a biological test. There is no validated blood biomarker that diagnoses chronic GvHD or predicts its course, which is one reason the 2014 revision emphasised biomarker association studies as a goal. The provisional criteria for atypical manifestations are, as their name says, provisional, and a quarter of chronic GvHD involves them.
A syndrome with no diagnostic test is made comparable by agreeing in advance which signs count, scoring each affected site on a common 0 to 3 scale, and deriving a global severity by rule rather than by impression. Weighting the lungs more heavily than the other organs encodes the observation that pulmonary involvement drives mortality.
Query for this technology: (TITLE:"How chronic GvHD is diagnosed and scored: the NIH consensus criteria" OR ABSTRACT:"How chronic GvHD is diagnosed and scored: the NIH consensus criteria" OR TITLE:"NIH consensus criteria" OR ABSTRACT:"NIH consensus criteria" OR TITLE:"NIH global severity score" OR ABSTRACT:"NIH global severity score" OR TITLE:"chronic GVHD scoring" OR ABSTRACT:"chronic GVHD scoring") AND (cancer OR tumor OR tumour OR oncology OR carcinoma OR lymphoma OR leukemia OR leukaemia OR myeloma OR sarcoma OR melanoma OR glioma). Results are unfiltered search hits about How chronic GvHD is diagnosed and scored: the NIH consensus criteria, not a curated reading list.
Shares After ruxolitinib: belumosudil, axatilimab and ibrutinib in chronic GvHD, When steroids fail: ruxolitinib for steroid-refractory GvHD, Chronic GvHD organ by organ: skin, mouth, eyes, gut, liver, joints and genital tract, Chronic graft-versus-host disease and the tags rejuvenation, survivorship, transplant, gvhd.
Shares When steroids fail: ruxolitinib for steroid-refractory GvHD, Chronic graft-versus-host disease, Graft-versus-host disease (GVHD) and graft-versus-leukaemia, After a transplant or cell therapy: what to ask for and the tags rejuvenation, survivorship, transplant, gvhd.
Shares Chronic graft-versus-host disease, Graft-versus-host disease (GVHD) and graft-versus-leukaemia, After a transplant or cell therapy: what to ask for, Quality of life and the tags rejuvenation, survivorship, transplant.
Shares The lungs after transplant: bronchiolitis obliterans syndrome, Chronic GvHD organ by organ: skin, mouth, eyes, gut, liver, joints and genital tract, After a transplant or cell therapy: what to ask for, Survivorship care and late-effects surveillance and the tags rejuvenation, survivorship, transplant.
Shares Chronic graft-versus-host disease, After a transplant or cell therapy: what to ask for, Survivorship care and late-effects surveillance, Late effects and survivorship toxicity and the tags rejuvenation, survivorship, transplant.
Shares Chronic GvHD organ by organ: skin, mouth, eyes, gut, liver, joints and genital tract, Chronic graft-versus-host disease, After a transplant or cell therapy: what to ask for, Survivorship care and late-effects surveillance and the tags rejuvenation, survivorship, transplant.
Shares Chronic graft-versus-host disease, After a transplant or cell therapy: what to ask for, Quality of life, Survivorship care and late-effects surveillance and the tags rejuvenation, survivorship, transplant.
Shares Chronic graft-versus-host disease, Graft-versus-host disease (GVHD) and graft-versus-leukaemia, After a transplant or cell therapy: what to ask for, Late effects and survivorship toxicity and the tags rejuvenation, survivorship, transplant.