Biology of chronic graft-vs-host disease: Immune mechanisms and progress in biomarker discovery

Table 1 Signs, symptoms and prevalence of chronic graft-vs-host disease in selected organs and tissues

Organ or tissue	Prevalence, %1	Diagnostic features2	Distinctive features3
Skin	75%	Poikiloderma Lichen planus-like features Sclerosis Morphea-like features	Depigmentation Papulosquamous lesions
Mouth	51%-63%	Lichen planus-like features	Xerostomia Mucoceles Mucosal atrophy Ulcers Pseudomembranes
Liver	29%-51%	None4	None4
Eye	22%-33%		Dry, gritty or painful eyes Cicatricial conjunctivitis Keratoconjunctivitis-Sicca syndrome
GI tract and esophagus	7%-45%	Esophageal web Strictures or stenosis in upper esophagus
Lung	4%-19%	Bronchiolitis obliterans	Air trapping and bronchiectasis on chest CT scan
Muscles, fascia and joints	6%	Fascitis Sclerosis Joint stiffness or contractures	Myositis or polymyositis
Genitalia	1%	Lichen planus-like features Lichen sclerosus-like features	Erosions, Fissures, Ulcers

¹Frequency of tissue involvement at initial cGVHD diagnosis (from Lee et al[4]);

²Clinical symptoms that are sufficient for cGVHD diagnosis. Information adapted from references 8 and 13;

³Clinical symptoms that are frequently seen in cGVHD, but insufficient for cGVHD diagnosis. Information adapted from references 8 and 13;

⁴While no diagnostic or distinctive features have been identified for liver cGVHD, hepatitis is often seen (and also sometimes in acute GVHD) with elevated serum levels of bilirubin, alkaline phosphatase and alanine aminotransferase (ALT)[13]. cGVHD: Chronic graft-vs-host disease; CT: Computed tomography.