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
Nails		Dystrophy Longitudinal ridging, splitting, or brittle features Onycholysis Pterygium unguis Nail loss (usually symmetric; affects most nails)*
Scalp and body hair		New onset of scarring or nonscarring scalp alopecia (after recovery from chemoradiotherapy) Scaling, papulosquamous lesions
Mouth	Lichen-type features Hyperkeratotic plaques Restriction of mouth opening from sclerosis	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 Vaginal 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 diagnosed with PFTs and radiology^¶
Muscles, fascia, joints	Fasciitis Joint stiffness or contractures secondary to sclerosis	Myositis or polymyositis^¶

Diagnosis of chronic GVHD requires the presence of at least one diagnostic clinical sign of chronic GVHD or the presence of at least one distinctive manifestation confirmed by pertinent biopsy or other relevant tests in the same or another organ. Furthermore, other possible diagnoses for clinical symptoms must be excluded. No time limit is set for the diagnosis of chronic GVHD.

GVHD: graft-versus-host disease; PFTs: pulmonary function tests.
* In all cases, infection, drug effects, malignancy, or other causes must be excluded.
¶ Diagnosis of chronic GVHD requires biopsy or radiology confirmation (or Schirmer test for eyes).

Original figure 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.

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