title: National Institutes of Health chronic graft-versus-host disease staging in severely affected patients: organ and global scoring correlate with established indicators of disease severity and prognosis.
abstract: Between 2004 and 2010, 189 adult patients were enrolled on the National Cancer Institute's cross-sectional chronic graft-versus-host disease (cGVHD) natural history study. Patients were evaluated by multiple disease scales and outcome measures, including the 2005 National Institutes of Health (NIH) Consensus Project cGVHD severity scores. The purpose of this study was to assess the validity of the NIH scoring variables as determinants of disease severity in severely affected patients in efforts to standardize clinician evaluation and staging of cGVHD. Out of 189 patients enrolled, 125 met the criteria for severe cGVHD on the NIH global score, 62 of whom had moderate disease, with a median of 4 (range, 1-8) involved organs. Clinician-assigned average NIH organ score and the corresponding organ scores assigned by subspecialists were highly correlated (r = 0.64). NIH global severity scores showed significant associations with nearly all functional and quality of life outcome measures, including the Lee Symptom Scale, Short Form-36 Physical Component Scale, 2-minute walk, grip strength, range of motion, and Human Activity Profile. Joint/fascia, skin, and lung involvement affected function and quality of life most significantly and showed the greatest correlation with outcome measures. The final Cox model with factors jointly predictive for survival included the time from cGVHD diagnosis (>49 versus ≤49 months, hazard ratio [HR] = 0.23; P = .0011), absolute eosinophil count at the time of NIH evaluation (0-0.5 versus >0.5 cells/μL, HR = 3.95; P = .0006), and NIH lung score (3 versus 0-2, HR = 11.02; P < .0001). These results demonstrate that NIH organs and global severity scores are reliable measures of cGVHD disease burden. The strong association with subspecialist evaluation suggests that NIH organ and global severity scores are appropriate for clinical and research assessments, and may serve as a surrogate for more complex subspecialist examinations. In this population of severely affected patients, NIH lung score is the strongest predictor of poor overall survival, both alone and after adjustment for other important factors.
authors: Pediatric Oncology Branch, National Cancer Institute, Center for Cancer Research, National Institutes of Health, Bethesda, MD 20892, USA. kbaird@mail.nih.gov
authors: Kristin
authors: K
authors: Baird
authors: Seth M
authors: SM
authors: Steinberg
authors: Lana
authors: L
authors: Grkovic
authors: Drazen
authors: D
authors: Pulanic
authors: Edward W
authors: EW
authors: Cowen
authors: Sandra A
authors: SA
authors: Mitchell
authors: Kirsten M
authors: KM
authors: Williams
authors: Manuel B
authors: MB
authors: Datiles
authors: Rachel
authors: R
authors: Bishop
authors: Carol W
authors: CW
authors: Bassim
authors: Jacqueline W
authors: JW
authors: Mays
authors: Dean
authors: D
authors: Edwards
authors: Kristen
authors: K
authors: Cole
authors: Daniele N
authors: DN
authors: Avila
authors: Tiffany
authors: T
authors: Taylor
authors: Amanda
authors: A
authors: Urban
authors: Galen O
authors: GO
authors: Joe
authors: Leora E
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authors: Comis
authors: Ann
authors: A
authors: Berger
authors: Pamela
authors: P
authors: Stratton
authors: Dan
authors: D
authors: Zhang
authors: James H
authors: JH
authors: Shelhamer
authors: Juan C
authors: JC
authors: Gea-Banacloche
authors: Claude
authors: C
authors: Sportes
authors: Daniel H
authors: DH
authors: Fowler
authors: Ronald E
authors: RE
authors: Gress
authors: Steven Z
authors: SZ
authors: Pavletic
mesh_terms: D000328:Adult; D003430:Cross-Sectional Studies; D005260:Female; D006086:Graft vs Host Disease / Q000145:classification* / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D008137:Longitudinal Studies; D008168:Lung / Q000276:immunology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009316:National Institutes of Health (U.S.); D011379:Prognosis; D016016:Proportional Hazards Models; D012720:Severity of Illness Index; D012867:Skin / Q000276:immunology / Q000473:pathology*; D016019:Survival Analysis; D014184:Transplantation, Homologous; D014481:United States