GVHD… The Saga Continues… NIH Consensus Development Project on Criteria for Clinical Trials in Chronic GVHD (June 6, 2005) • Clinical features emphasized rather than time of onset Clinical features for each organ Features grouped as Diagnostic, Distinctive, Other, Common. More specific diagnostic criteria Scoring criteria for each organ, taking function taken into account Global Score - mild, moderate or severe The NIH Consensus System is provisional, awaiting validation of its value for prognosis, correlation with quality of life, and value for therapeutic trials (sensitivity of scoring to change). Is there anyone out here who can tell us the true meaning of the NIH consensus guidelines? Classification of cGVHD ACUTE CHRONIC Day 100 NIH Classification CLASSIC ACUTE LATE ACUTE OVERLAP CHRONIC CLASSIC Classification of Features of cGVHD Diagnostic Sufficient to make a diagnosis of chronic GVHD Distinctive Not seen in acute GVHD but insufficient alone for diagnosis of cGVHD. Need biopsy, test, or other organ with Distinctive feature. Other Rare, controversial, or nonspecific features (but may be used for scoring once diagnosis has been established) Common Found in both chronic and acute GVHD (but may be used for scoring once diagnosis has been established) NIH Criteria: Steps in applying 1. Consider causes other than cGVHD 2. Minimal Criteria for diagnosis Diagnostic features in at least one organ OR Distinctive feature plus additional test, biopsy or distinctive feature in another organ OR other organ specific criteria 3. Organ scoring (0-3) 4. Global scoring (mild, moderate, severe) Mouth Diagnostic Lichen-type features (mouth) Diagnostic Hyperkeratotic plaques (mouth) Areas in which there is an enlargement or overgrowth the cells Hyperkeratosis of the tongue Distinctive Ulcer Distinctive Mucocele Skin Diagnostic Poikiloderma Diagnostic Lichen Planus Diagnostic Lichen Planus Diagnostic Deep Sclerosis Diagnostic Morphea – think “localized scleroderma” Diagnostic Lichen Sclerosus “Patchy white thin skin” “Cigarette-paper wrinkling” Distinctive Papulo-squamous chronic GVHD – Papules and small scaly plaques "Papulosquamous hyperkeratotic cutaneous conditions” – DISTINCTIVE not DIAGNOSTIC Papulosquamous hyperkeratotic cutaneous conditions are those that present with papules and scales Cowen, Edward (NIH/NCI): Pap-Sq is a wastebasket term for psoriasis-looking and other eruptions with scale that do not appear like the classic "lichen-planus-like" cGVHD [62 entities]. “Other” feature Keratosis Pilaris “Chicken Skin” Nails Distinctive features Dystrophy, Onycholysis, Pterygium unguis Dystrophy: Longitudinal ridging, splitting or brittle features Onycholysis: Loosening or separation of a fingernail or toenail from its nail bed Pterygium unguis: Forward growth of the cuticle over the nail bed Nail Loss: Usually symetric, affects most nails Scalp Muscle, Fascia, Joints Diagnostic Deep Sclerosis - Hidebound Eyes Genitalia Lungs GI Tract Liver Note: 1. Elevated ggt not included because it is too non-specific and variable 2. If one or more of LFTs listed is > 2x ULN, then the box can be checked The Tools ORGAN DIAGNOSTIC CRITERIA DISTINCTIVE CRITERIA? Skin, includes hair & nails Yes Yes 1. A diagnostic skin feature 2. Distinctive feature with biopsy confirming GVHD Mouth Yes Yes 3. A diagnostic mouth feature 4. A distinctive feature with biopsy confirming GVHD Genitalia Yes Yes 5. A diagnostic feature 6. A distinctive feature with biopsy confirming GVHD Muscles, fascia, joints Yes Yes 15. A diagnostic feature 16. Myositis or polymyositis confirmed as GVHD on biopsy Eyes No Yes 7. New ocular sicca with Schirmer < 5 mm (both eyes) at 5 min PLUS a distinctive feature in another organ 8. New onset of keratoconjunctivitis sicca by slit-lamp exam PLUS Schirmer 6 to 10 mm at 5 min PLUS a distinctive feature in another organ GI Yes No 9. A diagnostic feature 10. A common feature PLUS a biopsy/endoscopy confirming GVHD PLUS a distinctive feature in another organ 11. Exocrine pancreatic insufficiency PLUS a biopsy/endoscopy confirming GVHD PLUS a distinctive feature in another organ Liver No No 12. Abnormal LFTs (common feature) confirmed as GVHD by biopsy PLUS a distinctive feature in another organ Lungs Yes Yes 13. BO confirmed by lung biopsy 14. BO confirmed by PFT and radiology (see specific criteria) PLUS a distinctive feature in another organ DIAGNOSIS OF CHRONIC GVHD BY ORGAN A diagnosis of chronic GVHD can be made if one or more of the following situations below are present Cause due to infection/drug reaction/or other must be ruled out What happens next? Scoring happens after a diagnosis of chronic GVHD is confirmed Global Score • Mild chronic GVHD Only 1 or 2 organs or sites (except the lung: see below), with no clinically significant functional impairment (maximum of score 1 in all affected organs or sites). • Moderate chronic GVHD (1) at least 1 organ or site with clinically significant but no major disability (maximum score of 2 in any affected organ or site) OR (2) 3 or more organs or sites with no clinically significant functional impairment (maximum score of 1 in all affected organs or sites). A lung score of 1 will also be considered moderate chronic GVHD. • Severe chronic GVHD Major disability caused by chronic GVHD (score of 3 in any organ or site). A lung score of 2 or greater will also be considered severe chronic GVHD. Key Points Re Scoring • Scoring categories are a mix of functional aspects, clinical features and diagnostic tests • Score all symptoms/features related to GVHD (or if cause unknown); include symptoms/features from all clinical feature categories* • Do not score symptoms/features known to have a cause other than GVHD • Include symptoms of chronic and acute (i.e. the common symptoms) when scoring (this is overlap syndrome and falls under the chronic GVHD umbrella) *Clinical feature categories are: Diagnostic, distinctive, common, other Key Points Re Scoring (continued) • If there is a diagnosis of chronic GVHD in an organ other than the liver AND there are abnormal LFTs (> 2 x ULN) that are thought to be related to GVHD (even if not biopsy proven) then the liver should be scored and included when determining the GLOBAL SCORE • Once the initial diagnosis of chronic GVHD has been confirmed – All GVHD symptoms that occur later are scored using the chronic GVHD scoring scheme – See next slide for example Example • Month 6: First onset of chronic GVHD is confirmed • Month 12: Mild Diarrhea present, <5% weight loss Alk phos > 2 X ULN (causes other than GVHD ruled out for both) • Both organs (GI and liver) should be scored for chronic GVHD (due to previous diagnosis of chronic GVHD) • GI score = 1; Liver score =1 • Global Score = mild Teamwork! • In-person assessment at visit time points • Use Worksheet Part A & B at the visit • Worksheet = Source Document • Use the table to confirm or exclude cGVHD • Keep in regular communication to capture onset of cGVHD in real time Questions?
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