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cGVHD criteria

Author : Sanofi
Editorial Team

cGvHD is characterized by a combination of tissue inflammation and fibrosis

Global grading of cGvHD1

The diagnosis and assessment of cGVHD are guided by the National Institutes of Health (NIH) Consensus Criteria, which provide a standardized framework for identifying, staging, and managing the disease. This section outlines the global severity grading of cGVHD, guidance on diagnosis, and diagnostic characteristics of cGvHD.

Organ Involvement Table
No of organs involved Mild Grade Moderate Grade Severe Grade
1 Score 1 Score 2 Score 3
2 Score 1 Score 2 Score 3
3 Score 1 Score 3
Lung Score 1 Score 2
Mild grade: 1 or 2 organs (but not lung) with maximum score of 1.
Moderate grade: Lung score 1 or ≥3 organs at score 1 or at least one organ at score 2.
Severe grade: Lung score 2 or score 3 in any organ.
cGvHD Responsive Layout
Fibrotic lesions can develop across multiple organs2-5
SkinSKIN
MouthMOUTH
EyesEYES
GI TractGI TRACT
LiverLIVER
LungsLUNGS
JointsJOINTS AND FASCIA
Genital TractGENITAL TRACT
Fibrosis is a major contributor to life-threatening complications and significant morbidity6
  • Immune cells and their target tissues produce various cytokines and chemokines, which cause inflammation and tissue damage.7
  • The sclerotic skin manifestations of cGvHD (ScGvHD) result from inflammation and fibrosis of the dermis, subcutaneous tissue, or fascia, leading to significant functional disability.8
  • Sclerotic fibrosis of the perioral tissue owing to chronic inflammation can lead to restricted oral range-of-motion in patients with oral cGvHD.9
  • Conjunctival inflammation may be a primary manifestation of cGvHD or secondary to severe dry eye.10
  • The intestine can be the target of several immunologically mediated diseases, including cGvHD and inflammatory bowel disease.11
  • Pulmonary cGvHD leads to BOS which results in obstructive and/or restrictive changes.12
  • Esophageal involvement in cGvHD results in mucosal inflammation, leading to submucosal fibrosis and, occasionally, formation of webs and strictures.13
  • Liver cGvHD usually presents as an indolent cholestatic disease in patients with skin, mouth, and eye involvement.14

Guidance on diagnosis in the 2014 NIH cGvHD consensus criteria15

NIH cGvHD Diagnosis Criteria

NIH categorizes clinical features as

  • Diagnostic
  • Distinctive
  • Other features or unclassified manifestations
  • Common

A diagnosis of cGvHD requires the presence of ≥1 diagnostic features OR ≥1 distinctive features plus additional testing.

(e.g., biopsy, laboratory tests, evaluation by a specialist, radiographic imaging)

Diagnostic

Clinical features that establish the presence of cGvHD and do not require additional testing or evidence of other organ involvement

Distinctive

Clinical features that are not typically found in aGvHD and do require additional testing (e.g., biopsy) to establish a diagnosis of cGvHD

Other features or unclassified

Rare, controversial or nonspecific characteristics of cGvHD that cannot be used to establish a diagnosis

Common

Clinical features shared by cGvHD and aGvHD

Diagnostic Characteristics of cGvHD

Diagnostic characteristics of cGvHD

To provide structure and standardization in diagnosing cGvHD, particularly in the clinical trial setting, the NIH Consensus Development Project on Criteria for Clinical Trials in cGvHD published the 2014 guidance on diagnosing and staging, commonly referred to as the 2014 NIH cGvHD Consensus Criteria.15

arrow-down

The clinical features of cGvHD are grouped into 4 categories: diagnostic, distinctive, other features or unclassified manifestations and common.15

arrow-down

Prior to the 2014 NIH cGvHD Consensus Criteria, the characteristics of cGvHD were classified as limited or extensive.16

The 2014 NIH cGvHD consensus criteria also guide assessment of the severity of cGvHD

Organ Involvement
Skin SKIN
Mouth MOUTH
Eyes EYES
GI Tract GI TRACT
Liver LIVER
Lungs LUNGS
Joints JOINTS AND FASCIA
Genital Tract GENITAL TRACT
  • Chronic GvHD is classified into mild, moderate or severe, based on a global score
  • The global score takes into consideration the involvement and severity of 8 organs or sites
  • Each organ or site score ranges from 0 (no involvement) to 3 (severe impairment
Mild cGvHD
  • 1 or 2 organs involved with ≤ score 1+
  • Lung score 0
Moderate cGvHD
  • ≥ 3 organs involved with ≤ score 1
  • OR
  • ≥ 1 organ (not lung) with a score of 2
  • OR
  • Lung score 1
Severe cGvHD
  • ≥ 1 organ with a score of 3
  • OR
  • Lung score of 2 or 3

Key points

  1. In skin: higher of the two scores to be used for calculating global severity.

  2. In lung: FEV1 is used instead of clinical score for calculating global severity.

  3. 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.

  4. 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).

aGvHD, acute graft-versus-host disease; BOS, Bronchiolitis Obliterans Syndrome; cGvHD, chronic graft-versus-host disease; GI, gastrointestinal; GvHD, graft-versus-host disease; NIH, National Institutes of Health.

  1. Kim D.D.H et al. Curr Oncol. 2024;31:1426-1444;
  2. Kitko CL et al. Biol Blood Marrow Transplant. 2012;18(1 suppl):S46-S52. doi:10.1016/j.bbmt.2011.10.021;
  3. Cooke KR et al. Biol Blood Marrow Transplant. 2017;23(2):211-234. doi:10.1016/j.bbmt.2016.09.023;
  4. MacDonald KPA et al. Blood. 2017;129(1):13-21. doi:10.1182/blood-2016-06-686618;
  5. Fiuza-Luces C et al. Bone Marrow Transplant. 2016;51(1):13-26. doi:10.1038/bmt.2015.195;
  6. Henden AS, Hill GR. J Immunol. 2015;194(10):4604-4612. doi:10.4049/jimmunol.1500117;
  7. Presland RB. World J Transplant. 2016;6(4):608-619. doi.org/10.5500/wjt.v6.i4.608;
  8. Martires KJ et al. Blood. 2011;118(15):4250-7. doi.org/10.1182/blood-2011-04-350249;
  9. Bassim CW et al. J Dent Res. 2015;94(4):547-54. doi.org/10.1177%2F0022034515570942;
  10. Ogawa Y et al. Sci Rep. 2013;3:3419. doi.org/10.1038%2Fsrep03419;
  11. Haring E et al. Front Immunol. 2021;12:705342. doi.org/10.3389/fimmu.2021.705342;
  12. Flowers MED, Martin PJ. Blood. 2015;125(4):606-615. doi:10.1182/blood-2014-08-551994;
  13. Schima W et al. Abdom Imaging. 1994;19(3):191-4. 10.1007/BF00203504;
  14. Strasser SI et al. Hepatology. 2000;32(6):1265-71;
  15. Jagasia MH et al. Biol Blood Marrow Transplant. 2015;21(3):389-401.e1. doi:10.1016/j.bbmt.2014.12.001.
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