Signs and symptoms 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)	Other features or unclassified entities^¶	Common^Δ (seen with both acute and chronic GVHD)
Skin	Poikiloderma Lichen planus-like features Sclerotic features Morphea-like features Lichen sclerosus-like features	Depigmentation Papulosquamous lesions	Sweat impairment Ichthyosis Keratosis pilaris Hypopigmentation Hyperpigmentation	Erythema Maculopapular rash Pruritus
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) Loss of body hair Scaling	Thinning scalp hair, typically patchy, coarse or dull (not explained by endocrine or other causes) Premature gray hair
Mouth	Lichen planus-like changes	Xerostomia Mucoceles Mucosal atrophy Ulcers Pseudomembranes		Gingivitis Mucositis Erythema Pain
Eyes		New-onset dry, gritty, or painful eyes Cicatricial conjunctivitis KCS Confluent areas of punctate keratopathy	Photophobia Periorbital hyperpigmentation Blepharitis (erythema of the eyelids with edema)
Genitalia	Lichen planus-like features Lichen sclerosus-like features	Erosions Fissures
Females	Vaginal scarring or clitoral/labial agglutination	Ulcers
Males	Phimosis or urethral/meatus scarring or stenosis
GI tract	Esophageal web Strictures or stenosis in the upper to mid third of the esophagus		Exocrine pancreatic insufficiency	Anorexia Nausea Vomiting Diarrhea Weight loss Failure to thrive (infants and children)
Liver				Total bilirubin, alkaline phosphatase >2 × upper limit of normal ALT >2 × upper limit of normal
Lung	Bronchiolitis obliterans diagnosed with lung biopsy BOS^◊	Air trapping and bronchiectasis on chest CT	Cryptogenic organizing pneumonia Restrictive lung disease^§
Muscles, fascia, joints	Fasciitis Joint stiffness or contractures secondary to fasciitis or sclerosis	Myositis or polymyositis^¥	Edema Muscle cramps Arthralgia or arthritis
Hematopoietic and immune			Thrombocytopenia Eosinophilia Lymphopenia Hypo- or hypergammaglobulinemia Autoantibodies (AIHA, ITP) Raynaud's phenomenon
Other			Pericardial or pleural effusions Ascites Peripheral neuropathy Nephrotic syndrome Myasthenia gravis Cardiac conduction abnormality or cardiomyopathy

GVHD: graft-versus-host disease; KCS: keratoconjunctivitis sicca; GI: gastrointestinal; ALT: alanine aminotransferase; BOS: bronchiolitis obliterans syndrome; CT: computed tomography; AIHA: autoimmune hemolytic anemia; ITP: idiopathic thrombocytopenic purpura.
* In all cases, infection, drug effect, malignancy, or other causes must be excluded.
¶ Can be acknowledged as part of the chronic GVHD manifestations if diagnosis is confirmed.
Δ Common refers to shared features by both acute and chronic GVHD.
◊ BOS can be diagnostic for lung chronic GVHD only if distinctive sign or symptom present in another organ.
§ Pulmonary entities under investigation or unclassified.
¥ Diagnosis of chronic GVHD requires biopsy.

Reproduced 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. Table used with the permission of Elsevier Inc. All rights reserved.

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Signs and symptoms of chronic graft-versus-host disease