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Mooren’s ulcer resolved with campath-1H
  1. J van der Hoek,
  2. A Azuara-Blanco,
  3. K Greiner,
  4. J V Forrester
  1. Department of Ophthalmology, Aberdeen Royal Infirmary, Aberdeen, UK
  1. Correspondence to: Augusto Azuara Blanco, MD PhD, Ophthalmology Department, Aberdeen Royal Infirmary, The Eye Clinic, Foresterhill, Aberdeen AB25 2ZN, UK; aazblanco{at}

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Mooren’s ulcer is a rare idiopathic peripheral ulcerative keratitis. The diagnosis is usually based on characteristic clinical features and absence of other causes of peripheral keratitis. The clinical course can be unremitting, particularly in bilateral disease, occasionally leading to total loss of stroma.1,2

An autoimmune process is recognised as being central to the pathogenesis.

Calgranulin-C (CaGC), produced by granulocytes and also expressed by keratocytes, appears to be the target protein for the autoimmune response that leads to Mooren’s ulcer. Previous corneal trauma2 and a higher prevalence of HLA class II subtypes have been associated with Mooren’s ulcer.

The disease responds to immunosuppression with variable success. Surgical treatments such as conjunctival recession have been proposed. Campath-1H is a humanised lymphocytotoxic monoclonal antibody (mAb) that targets the CD52 antigen on T lymphocytes. Successful mAb therapy using campath-1H has been reported in serious ophthalmic inflammatory conditions that were unresponsive to maximum conventional immunosuppression.1–5

Case report

A 36 year old man presented to the eye department with a severe alkaline burn in both eyes. The right eye had severe stromal opacity and 360 degree limbal ischaemia, and was enucleated 2.5 years later after multiple surgical treatments. The left eye had partial epithelial loss and inferior limbal ischaemia (four clock hours), healing completely. Visual acuity was 20/30 a year after injury, when the patient developed an inferior-nasal gutter of 160 degrees with deep and superficial vascularisation, with progressive discomfort and photophobia, characteristic of Mooren’s ulcer. Autoantibodies, hepatitis titres, and tests for anti-filarial serology were negative. The patient’s HLA (DR) profile was DR15(2), DR17(3), DQ6(1), DQ 2. Serum anti-calgranulin C determined by western blot was not elevated.

Initial systemic and topical prednisolone with cyclosporin A failed to control the process. In spite of conjunctival recession, maximum dose triple oral immunosuppression with prednisolone, cyclosporin A and mycophenolate mofetil, topical prednisolone and cyclosporin A, and oral doxycycline, the inflammation and corneal melting continued to advance. Corneal microperforations were treated with two consecutive applications of histoacrylic glue (see Fig 1).

Figure 1

External photograph of the left eye before treatment with campath-1H. Note the severe injection and the glue applied nasally.

A 5 day course of campath intravenously was then administered. After the first week, there was marked decrease of the corneal inflammation, with epithelial healing and improvement of pain and photophobia. Immunosuppression was reduced to a low dose of oral and topical prednisolone. Two months later, a minor recurrence was treated with oral mycophenolate mofetil and topical cyclosporin A. The cornea healed within a week with no further corneal inflammation and loss of stroma.

The patient had a transient anaemia, reduction in both T and B cell counts and reversal of CD4:CD8 ratio. A posterior subcapsular cataract developed, operated on 10 months after campath-1H treatment. Fourteen months later, the eye remained quiet with a stable ocular surface and corneal stroma, and visual acuity of 20/25. His immunosuppression was being tapered gradually.


In this patient with severe Mooren’s ulcer standard treatments including conjunctival recession and aggressive triple immunosuppression did not control the disease. Treatment with campath-1H was successful. Although medium term tolerance appears to be excellent, long term risks of infection and malignancy are still to be determined. Campath-1H may be considered a last resort drug for use in those patients with Mooren’s ulcer in whom other treatments have failed.

The authors have no commercial interests related to the products described in the article.


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