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Chronic GvHD is a pattern of injuries that can appear in several places at once: skin that thickens, a dry sore mouth, dry painful eyes, difficulty swallowing, abnormal liver tests, stiff joints, genital narrowing. Several of the best treatments are local rather than systemic. The eye and genital problems are the ones least often raised.
The eight sites scored by the 2014 NIH criteria are listed below with what involvement looks like and what is done about it. The organ-specific treatments are given alongside whatever systemic treatment is running, not instead of it.
Skin, and sclerosis. The diagnostic signs include poikiloderma, lichen planus-like features, sclerotic features, morphoea-like changes and lichen sclerosus-like changes. The scoring runs on body surface area involved and, separately, on whether there is sclerosis, because a small patch of deep sclerosis matters more than a wide thin rash. Sclerotic disease is the form that restricts: skin binds to the tissue beneath, movement is lost at the shoulders and hips, and the limitation can outlast the inflammation. Topical steroids and calcineurin inhibitors treat the surface; physiotherapy and a stretching programme treat the restriction, and are the part most often under-prescribed. Photoprotection matters for a second reason: squamous cell carcinoma of the skin is one of the second cancers chronic GvHD itself raises the risk of.
Mouth. Lichen planus-like changes are diagnostic; ulcers, mucoceles, atrophy and dryness are supportive. Treatment is mostly topical: high-potency steroid rinses or gels, topical tacrolimus, saliva substitutes and intensive dental prevention, because a dry mouth decays. Oral involvement responds better than most sites, and in the extracorporeal photopheresis crossover study oral chronic GvHD showed the highest extracutaneous response rate, 70 per cent complete and partial resolution after 24 weeks.
Eyes. The 2014 revision tightened the diagnostic criteria here. The picture is a severe dry eye from lacrimal gland damage and ocular surface inflammation, with grittiness, pain, light sensitivity and, if it is not treated, corneal damage. Treatment is preservative-free lubricants, punctal occlusion, topical ciclosporin or steroid, autologous or allogeneic serum eye drops, and scleral lenses for the worst surfaces. An ophthalmologist who knows ocular GvHD is the referral that changes the outcome; a general dry eye clinic may not reach for serum drops or scleral lenses.
Gastrointestinal tract. Oesophageal web or stricture is the diagnostic sign; otherwise it is difficulty or pain on swallowing, early satiety, nausea, diarrhoea and weight loss, and the scoring hangs on weight loss and on whether the person can eat. Endoscopic dilatation treats a stricture mechanically. Weight loss here is a scored item in its own right and a reason to involve a dietitian early rather than late.
Liver. There is no diagnostic sign: abnormal liver tests with a cholestatic pattern, raised alkaline phosphatase and bilirubin, in the right context and after other causes are excluded. Ursodeoxycholic acid is used; the mainstay is the systemic treatment.
Joints and fascia. Fasciitis and joint stiffness from sclerosis are the diagnostic signs, scored by range of motion. This is the site where physiotherapy is the treatment with the clearest mechanism: the limitation is mechanical, and a stretching and range-of-motion programme can preserve function that drugs will not restore once fibrosis is fixed.
Genital tract. The 2014 revision also tightened these criteria. In women, lichen planus-like or lichen sclerosus-like changes, erosions, fissures, vaginal scarring and stenosis; in men, lichen planus-like changes and phimosis or urethral scarring. Treatment is topical steroid or calcineurin inhibitor, dilators where stenosis is forming, lubricants and, where appropriate, topical oestrogen. This is the site most often missed, because it is rarely examined unless asked about and rarely volunteered. It is worth asking for a genital examination at follow-up rather than waiting to be offered one.
The ones outside the list. A quarter of chronic GvHD includes manifestations the eight-organ scheme does not score: immune-mediated cytopenias in 24.5 per cent of the atypical cases in one 623-patient series, renal involvement and serositis in 13.7 per cent each, and peripheral neuropathy. Renal involvement, restrictive lung disease and peripheral neuropathy were the atypical manifestations that drove non-relapse mortality in that series; thyroid, musculoskeletal and pancreatic involvement did not.
Chronic GvHD injures epithelial and glandular tissue first and then replaces it with fibrosis. That is why the organs affected are the barrier and secretory surfaces, skin, mouth, eye, gut and genital epithelium, and why the late phase of involvement at any of them is stiffness, stenosis or dryness rather than inflammation. It is also why topical treatment works at accessible surfaces and why physiotherapy, dilatation and dental prevention are not adjuncts but treatments.
Query for this technology: (TITLE:"Chronic GvHD organ by organ: skin, mouth, eyes, gut, liver, joints and genital tract" OR ABSTRACT:"Chronic GvHD organ by organ: skin, mouth, eyes, gut, liver, joints and genital tract" OR TITLE:"sclerotic GvHD" OR ABSTRACT:"sclerotic GvHD" OR TITLE:"ocular GvHD" OR ABSTRACT:"ocular GvHD" OR TITLE:"oral GvHD" OR ABSTRACT:"oral GvHD") AND (cancer OR tumor OR tumour OR oncology OR carcinoma OR lymphoma OR leukemia OR leukaemia OR myeloma OR sarcoma OR melanoma OR glioma). Results are unfiltered search hits about Chronic GvHD organ by organ: skin, mouth, eyes, gut, liver, joints and genital tract, not a curated reading list.
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