Canterbury DHB

Management of oral chronic GVHD

Diagnosis

It is important to differentiate cGVHD from other conditions which may mimic it, including infections and other ulcerative conditions.

The diagnostic features of chronic GVHD are:

• Lichen planus (white lines and lacy appearance of the mucosa)
• Leukoplakia (hyperkeratotic plaques)
• Decreased oral opening due to sclerosis

Distinctive but not diagnostic features:

• Dryness
• Ulceration
• Mucocoeles
• Mucosal atrophy
• Gingivitis
• Erythema
• Pain
• Mucositis

Assessment and Grading

Oral cGVHD has three distinct profiles:

• Mucosal lesions
• Xerostomia
• Sclerotic

Screening questions

• Any mouth sores or ulcers – using analgesics or unable to eat?
• Any pain with spicy foods, fizzy drinks, hot/cold, toothpaste?
• Does you mouth feel dry most of the time?
• Is it difficult to open your mouth wide?
• Do mouth symptoms limit your oral intake?

Examination

The mouth should be assessed for the four cardinal features of GHVD:

• Erythema

  

  This image provides a frame of reference for grading oral erythema:

  A - Normal mucosa has the approximate colour of uncooked lean pork.

  B - Mild erythema appears similar to the inner part of an almost ripe strawberry. Moving outwards, the more intense the colour of the skin approximates moderate erythema.

  C - Severe erythema is most often the colour of a fully ripened strawberry, raspberry, or red beet.

  From Carpenter, P. A. (2011). "How I conduct a comprehensive chronic graft-versus-host disease assessment." Blood 118(10): 2679-2687.

• Ulceration

  

• Lichenoid

  

• Mucocoeles

  

Grading of severity

The NIH global score for oral GVHD is a simplified 0-3 scale for screenng and routine calendar assessment:

• 0 - No symptoms
• 1 - Mild symptoms with disease signs but not limiting oral intake significantly.
• 2 - Moderate symptoms with disease signs and partial limitation of roal intake.
• 3 - Severe symptoms with diesase signs and major limitation of oral intake.

For more comprehensive assessment of oral GVHD and response to treatment, use the more detailed NIH scoring system (0-15).

When assessing the area of the mouth involved, a rule of thumb is:

• Left and right buccal mucosa – 20% each.
• Lips and lower labial mucosa – 20%.
• Dorsal tongue – 20%.
• Soft palate and ventrolateral tongue – 20%.
• The hard palate and gums are inspected but do not contribute to the assessment of area.

Management

1. Swab erythematous lesions for fungal disease.
2. Mouth ulcers:
   • Swab ulceration for HSV infection.
   • Orabase ointment (barrier).
   • Oracort (with steroid).
3. Educate on home oral hygiene:
   • Use flouride toothpaste.
   • Use children’s toothpaste if burning occurs:
     • Try “Dora” or “Superman”!
   • Use sugar-free beverages.
   • Alcohol-free mouthwashes, e.g. “Colgate Dry Mouth Relief”, “Biotene” mouthwash, or “Oral 7”.
4. Dry mouth:
   • Prescribe dry mouth products for symptomatic relief (these are available online, e.g. www.toothshop.co.nz).
     • GC Dry Mouth Gel.
     • Recaldent gum for saliva stimulation (only effective if some residual function).
     • OraLube 125 mls, 2 sprays as required (prescribable).
   • Frequent water sipping.
   • Avoid xerogenic medications if possible, e.g. some antidepressants, antihistamines.
   • Cholinergic agonists, e.g. pilocarpine eye drops 4% (40 mg/ml; 15 mls) 5 mg QDS. A local chemist may be able to make it up to 1 mg/ml solution. Will only work if some residual salivary gland function so stop if no benefit after 2 weeks. Contra-indicated in glaucoma, ischaemic heart disease, and COPD.
5. Topical steroids:
   • Dexamethasone 4 mg tablets crushed in 20 mls water - Rinse for 3-4 minutes and spit for generalised cGVHD and/or
   • Diprosone OV ointment 0.05% (30 g) - Twice daily, apply to gauze and leave in place for 10-15 minutes for localised lesions or triamcinolone 0.1% dental paste.
   • Nystatin100,000 u/ml, 5 mls - Rinse and swallow QDS with topical steroids to prevent candidiasis.
6. Topical cyclosporin, oral liquid preparation 100 mg/ml - Dilute 1 ml in 20 mls water in a glass container, rinse for 3-4 minutes and spit.
7. Consider proton pump inhibitor, e.g. omeprazole 20 mg BD to control GORD.
8. Annual general dental review.
9. Annual review for secondary cancer screening – Refer to dental department.

Further Reading

• Carpenter, P. A., et al. (2015). "National Institutes of Health Consensus Development Project on Criteria for Clinical Trials in Chronic Graft-versus-Host Disease: V. The 2014 Ancillary Therapy and Supportive Care Working Group Report." Biology of Blood and Marrow Transplant 21(7): 1167-1187.
• Mays, J. W., et al. (2013). "Oral chronic graft-versus-host disease: current pathogenesis, therapy, and research." Oral Dis 19(4): 327-346.
• Carpenter, P. A. (2011). "How I conduct a comprehensive chronic graft-versus-host disease assessment." Blood 118(10): 2679-2687.

Sjogen’s Syndrome Society website

Topic Code: 253743