Diagnostic and distinctive clinical manifestations of chronic graft-versus-host disease

Organ or site	Diagnostic (sufficient to establish the diagnosis of chronic GVHD)	Distinctive (seen in chronic GVHD, but insufficient alone to establish a diagnosis of chronic GVHD)
Skin	Poikiloderma Lichen planus-like features Sclerotic features Morphea-like features Lichen sclerosus-like features	Depigmentation Papulosquamous lesions
Nails		Dystrophy Longitudinal ridging, splitting, or brittle features Onycholysis Pterygium unguis Nail loss (usually symmetric and affects most nails)
Scalp and body hair		New onset of scarring or nonscarring scalp alopecia (not associated with recovery from chemotherapy or radiotherapy) Loss of body hair Scaling
Mouth	Lichen planus-type changes	Xerostomia Mucocele Mucosal atrophy Pseudomembranes Ulcers
Eyes		New-onset dry, gritty, or painful eyes Cicatricial conjunctivitis Keratoconjunctivitis sicca Confluent areas of punctate keratopathy
Genitalia	Lichen planus-like features Lichen sclerosus-like features Females: Vaginal scarring or clitoral/labial agglutination Males: Phimosis or urethral/meatus scarring or stenosis	Erosions Fissures Ulcers
GI tract	Esophageal web Strictures or stenosis in the upper to mid third of the esophagus
Lung	Bronchiolitis obliterans diagnosed with lung biopsy	Bronchiolitis obliterans syndrome (BOS) diagnosed with PFTs and imaging
Muscles, fascia, joints	Fasciitis Joint stiffness or contractures secondary to fasciitis or sclerosis	Myositis or polymyositis

Diagnosis of chronic GVHD requires the presence of at least one diagnostic clinical sign or at least one distinctive manifestation confirmed by pertinent biopsy, other relevant tests in the same or another organ (eg, Schirmer's test, slit lamp examination), or evaluation by a relevant specialist (eg, gynecology, ophthalmology). Other possible diagnoses (eg, infection, drug effect, malignancy) must be excluded. There is no time limit for the diagnosis of chronic GVHD.

GVHD: graft-versus-host disease; PFTs: pulmonary function tests; BOS: Bronchiolitis obliterans syndrome.

Original table modified for this publication. Filipovitch AH, Weisdorf D, Pavletic S, et al. National Institutes of Health Consensus Development Project on Criteria for Clinical Trials in Chronic Graft-versus-Host Disease: 1. Diagnosis and Staging Working Group Report. Biol Blood Marrow Transplant 2005; 11:945. Table used with the permission of Elsevier Inc. All rights reserved.

Updated with information from:

Jagasia MH, Greinix HT, Arora M, et al. National Institutes of Health Consensus Development Project on Criteria for Clinical Trials in Chronic Graft-versus-Host Disease: I. The 2014 Diagnosis and Staging Working Group Report. Biol Blood Marrow Transplant 2015; 21:389.

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Diagnostic and distinctive clinical manifestations of chronic graft-versus-host disease