A case of idiopathic necrotizing scleritis with secondary glaucoma treated successfully with golimumab and Ahmed valve implantation.

A 52-year-old woman presented with idiopathic active scleritis not responding to oral cyclophosphamide, azathioprine, and oral steroid. Her intraocular pressure in the left eye was 45 mm of Hg in spite of using combination of brinzolamide 1% and brimonidine 0.2% (thrice a day), timolol maleate 0.5% (twice a day) eye drops in both eyes and oral acetazolamide. She was administered subcutaneous golimumab 50 mg injection every 4 weeks along with oral methotrexate 15 mg/week. The scleral inflammation responded and she underwent Ahmed glaucoma valve implantation after two months of initiation of golimumab therapy. After one week of surgery her IOP in left eye was the reduced to 8 mm of Hg. The index case showed that Golimumab can be a useful drug in the management of necrotizing scleritis refractory to the conventional therapy.

The management of glaucoma in patients with scleritis always remains a challenge. The surgical intervention to control the intraocular pressure (IOP) in necrotizing scleritis, the most common subtypes of scleritis associated with secondary glaucoma further adds to the challenge.[1] The higher chances of globe-perforation because of extensive scleral thinning are often the main limiting factor in such cases. Also, reactivation of scleral inflammation following surgery is a major concern for the treating ophthalmologist. Literature on surgical management of glaucoma associated with necrotizing scleritis is sparse. There has been only two reports on Ahmed glaucoma valve (AGV) implantation in patients with necrotizing scleritis available in literature.[23]

Golimumab (GLM) is a fully human anti-tumour necrosis factor (TNF)-α monoclonal antibody. Since its first reported use in the treatment of non-infectious uveitis in 2011,[4] various reports on efficacy of the drug in the management of ocular inflammatory diseases have been published. We present a refractory case of necrotizing scleritis with secondary glaucoma, where subcutaneous injection of GLM with oral methotrexate helped to attain resolution of scleral inflammation and IOP control was achieved with an AGV implantation. To the best of our knowledge, the index case is the first report on potential use of GLM in patients with scleritis.

Case Report

A 52-year-old woman presented to our clinic with complaints of recurrent attacks of ocular pain and redness in both eyes for last 6 years. She received a diagnosis of necrotising scleritis, and was extensively investigated. A thorough systemic evaluation for syphilis, tuberculosis, and sarcoidosis was unrevealing. Results of laboratory investigations for vasculitis and rheumatologic disorders were within reference limits. She had undergone phacoemulsification with intraocular lens implantation. She was treated with topical and oral corticosteroid and various immunosuppressives – methotrexate, azathioprine, cyclophosphamide, cyclosporine in these years; but failed to achieve a steroid-free remission. Her best corrected visual acuity was 6/36 in right eye and 6/9 in left eye. Slit-lamp examination of the right eye revealed 360 degree scleral thinning with peripheral corneal thinning and opacity in right eye with a quiet anterior chamber and pseudophakia [Fig. 1a]. In left eye, diffuse scleral thinning was noted with dilated deeper episcleral vessels, which did not blanch with topical vasoconstrictor [Fig. 1b-d]. Slit-lamp examination of the left eye also revealed cells 1+, flare 1+ in anterior chamber. IOP measured with Goldman applanation tonometry was 26 mm of Hg in right eye and 45 mm of Hg in left eye. Fundus examination showed clear media with no evidence of posterior segment inflammation. Optic nerve examination revealed increased vertical cup: Disc ratios of 0.8 with bipolar thinning in the right eye and 0.9 with near total cupping in left eye. She was on oral corticosteroid (10 mg every day), oral cyclophosphamide (50 mg twice a day), oral azathioprine (50 mg twice a day). She was also using a combination of brinzolamide 1% and brimonidine 0.2% (thrice a day), timolol maleate 0.5% (twice a day) eye drops in her both eyes and was on oral acetazolamide 250 mg four times a day. A decision to insert Ahmed glaucoma valve was suggested after proper control of scleritis. She was subsequently administered subcutaneous golimumab 50 mg and started on oral methotrexate (15 mg/week) under the care of a rheumatologist. It was decided to repeat the subcutaneous golimumab 50 mg injection every 4 weeks. She was advised to continue anti-glaucoma medications. Follow-up examination 2 months later revealed complete resolution of scleritis with a quiet anterior chamber in left eye. IOP in right and left eye was 14 and 28 mm of Hg respectively. Subsequently she underwent Ahmed glaucoma valve implantation in her left eye. One week after the surgery, her IOP in left eye was 8 mm of Hg [Fig. 2]. A third injection of subcutaneous golimumab 50 mg injection was administered and she was advised to continue oral methotrexate 15 mg/week.

Figure 1

(a) Slit-lamp photograph of the right eye showing 360 degree scleral thinning and peripheral corneal thinning and opacity. (b-d) Slit-lamp photograph of the left eye showing diffuse scleral thinning with dilated deeper episcleral vessels, which did not blanch with topical vasoconstrictor

Figure 2

Slit-lamp photograph of the left eye showing Ahmed glaucoma valve in superotemporal quadrant and tube in situ

Discussion

The cause of glaucoma in scleritis patients can be multi-factorial and can be due to obstruction of the trabecular meshwork by inflammatory cells, peripheral anterior synechiae and/or posterior synechiae related or due to steroid response.[5] The concurrent anterior uveitis, presence of synechia, previous cataract surgery and corneal involvements are the other reported risk factors for increased IOP in patients with scleritis.[1]

Over the last few years, drainage implants have become popular and increasingly being advocated for the treatment of uveitic glaucoma with high published success rates. However, the literature on AGV implantation in patients with necrotizing scleritis is sparse. Ranjan and Rao[2] described a case of necrotizing scleritis with extensive staphylomas and secondary glaucoma in a 35-year-old one eyed female. The patient underwent AGV implant and had good control IOP post-operatively. After three and half months, she presented with recurrence of scleral inflammation with scleral melt leading to exposure of the valve for which she required patch graft.[2] Goel et al.[3] described a patient with bilateral idiopathic healed sclerokeratouveitis with ciliary and intercalary staphyloma where phacoemulsification with intraocular lens implantation and AGV implant were performed as a single-stage procedure for complicated cataract and secondary glaucoma in the left eye. A cadaveric scleral graft was used to cover the AGV and the post-operative outcome was good except for severe anterior chamber inflammation on the second day.[3]

Necrotising scleritis, though rare, is the most severe form of the scleral inflammation. Immunosuppressive agents are often considered as first-line of therapy in the management of necrotizing scleritis. Though, reported to have a higher association with systemic rheumatic diseases than other subtypes of scleritis, necrotizing scleritis can be idiopathic.[6] Management of necrotizing scleritis, refractory to the conventional immunosuppressive therapy is challenging. In our case, it was further complicated by the secondary glaucoma. Various biological agents has been successfully used in the management of refractory necrotizing scleritis.[78] The scleral inflammation in our patient was resistant to conventional immunosuppressives, but responded to subcutaneous injection of GLM which helped us to perform AGV implantation. GLM, which requires once a month injection, has been found to be effective in the management of recalcitrant cases of uveitis.[910] Our report suggests that GLM can be a useful drug in the management of necrotizing scleritis refractory to the conventional therapy. However, further research is needed to ensure the safety and efficacy of the drug in patients with necrotizing scleritis.

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The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.