Graft Versus Host Disease

Graft versus Host Disease (GVHD) is a complication of bone marrow, stem cell or cord blood transplantation that can affect the eyes, as well as other organs including the lungs, liver, skin and gut. GVHD is the most frequent and serious complication of allogeneic hematopoietic stem cell transplantation (HSCT).1

Ocular involvement of GVHD occurs in 60-90% of HSCT patients and can have potentially devastating ocular symptoms.1 Patients may have signs and symptoms of severe keratoconjunctivitis sicca (dry eye) that can lead to corneal involvement and loss of vision. Conjunctivitis may also be present.

The inflammation that’s associated with this condition can also lead to a number of ocular conditions that can impair vision, cause severe discomfort, and even affect physical appearance. Indeed, the disabling dryness that GVHD commonly causes can significantly affect quality of life and must be managed closely by an eye care professional.

What Causes GVHD?

GVHD is an autoimmune condition in which donor immune cells react against host tissues. A very high percentage of people who have hematopoietic stem cell transplantation will develop acute GVHD.1 The exact risk depends on the donor source and the prophylaxis selected. Often, GVHD becomes a chronic condition (cGVHD).

Ocular symptoms of GVHD can develop in patients who have the acute or chronic form of the disease. However, ocular symptoms are more common in patients with the chronic form.

How Is GVHD Diagnosed?

Diagnosing GVHD is fairly straightforward since its clinical signs and symptoms are routinely encountered in patients who have other inflammatory diseases that affect the eyes. The key piece of evidence is most often revealed in the patient history, since all cases involve a history of transplant.

Following the history taking, a standard comprehensive evaluation will likely reveal dry eye, accompanied by symptoms such as ocular irritation or foreign body sensation, excessive tearing, sensitivity to light, blurry vision and, in many cases, varying degrees of pain. The doctor may also note that your eyes are red, and you may suffer from recurring bouts of pink eye (conjunctivitis).

If a doctor suspects that you are at risk of GVHD, a Schirmer's test will likely be performed to measure the amount of tears you produce. To perform a Schirmer's test, the doctor inserts a small strip of paper under your lower eyelid. The procedure is simple, quick and painless. The doctor may also put a bit of dye in your eye to see how quickly your tears evaporate and to assess the extent to which dryness may be damaging your ocular structures.

Medical and Surgical Treatments for GVHD

In addition to systemic medications, people with GVHD usually also need to use topical eye drops. These might include topical steroids, topical antibiotics, topical cyclosporine 0.05%, autologous serum eye drops and other drops for inflammation control. Autologous serum eye drops are made from components taken from your own blood and contain growth factors and vitamins that can help heal the ocular surface. In addition, over-the-counter preservative-free artificial tears and lubricants can be applied to supplement the natural tear film.2,3,4,5

If drops alone cannot suitably address the condition, other options include temporary or permanent punctual occlusion to prevent tear drainage, or tarsorrhaphy to narrow the eyelid opening in an effort to decrease the exposed surface area and prevent tear evaporation. Amniotic membrane can also be placed on damaged corneas to help promote healing. In more advanced cases, irreversible damage and scarring may occur at the ocular surface, and corneal transplantation may be required.

Scleral Lenses for GVHD

Because severe dry eye is so common with GVHD, reducing friction and tear evaporation are primary treatment goals. For this reason, doctors are increasingly fitting GVHD patients in scleral lenses. Whether or not vision correction is needed, these lenses can benefit those who suffer with GVHD. That is because these uniquely designed lenses vault across the entire corneal surface and rest on the white part of the eye, known as the sclera. A liquid reservoir is formed between the lens and the cornea, protecting the cornea from the ongoing mechanical shearing effect of the eyelids that occurs when you blink. This liquid layer also continuously bathes your sensitive corneal tissue. Multiple studies have indicated improvement in ocular symptoms including a reduction in eye pain, improvement in photophobia and an improvement in quality of life.6,7

For people with GVHD who need vision correction in addition to dry eye symptom relief, the fluid layer helps smooth out defects caused by dry eyes, providing a more uniform refractive surface. Scleral lenses also help improve vision and ocular comfort in patients with chronic GVHD.8,9

Another benefit of scleral lenses is that they can help patients reduce the amount of medication that’s typically needed to manage ocular GVHD. In fact, in some cases, patients have been able to completely eliminate prescription eye drops after successfully being fit in a scleral lens.

If you have GVHD, it is critical to see your eye doctor as regularly as recommended so you can be closely monitored for ocular surface damage and properly treated for any discomfort.

 


References:

[1] Espana EM, Shah S, Santhiago MR, Singh AD. Graft versus host disease: clinical evaluation, diagnosis and management. Graefes Arch Clin Exp Ophthalmol. 2013;251:1257-66.

[2] Robinson MR, Lee SS, Rubin BI. Topical corticosteroid therapy for cicatricial conjunctivitis associated with chronic graft-versus-host disease. Bone Marrow Transplant. 2004;33:1031-1035.

[3] Rao SN, Rao RD. Efficacy of topical cyclosporine 0.05% in the treatment of dry eye associated with graft versus host disease. Cornea. 2006;25:674-8.

[4] Malta JB, Soong HK, Shtein RM, et al. Treatment of ocular graft-versus-host disease with topical cyclosporine 0.05%. Cornea. 2010;29:1392-1396.

[5] Ogawa Y, Okamoto S, Mori T, et al. Autologous serum eye drops fro the treatment of severe dry eye in patients with chronic graft-versus-host disease. Bone Marrow Transplant. 2003;31:579-583.

[6]Takahide K, Parker PM, Wu M, et al. Use of fluid-ventilated, gas-permeable scleral lens for management of severe keratoconjunctivitis sicca secondary to chronic graft-versus-host disease. Biol Blood Marrow Transplant. 2007;13:1016-1021.

[7] Jacobs DS, Rosenthal P. Boston scleral lens prosthetic device for treatment of severe dry eye in chronic graft-versus-host disease. Cornea. 2007;26:1195-9.

[8] Theophanous C, Irvine JA, Parker P, Chiu GB. Use of prosthetic replacement of the ocular surface ecosystem scleral lenses in patients with ocular chronic graft-versus-host disease. Biol Blood Marrow Transplant. 2015;21:2180-4.

[9] Schornack MM, Baratz KH, Patel SV, Maguire LJ. Jupiter scleral lens in the management of chronic graft versus host disease. Eye Contact Lens. 2008;34:302-5.