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Regression of necrotizing scleritis in Wegener’s granulomatosis after infliximab treatment

2009; Wiley; Volume: 88; Issue: 3 Linguagem: Inglês

10.1111/j.1755-3768.2009.01546.x

1755-3768

Matti Kontkanen, Leena Paimela, Kai Kaarniranta,

Sarcoidosis and Beryllium Toxicity Research

Editor, Wegener's granulomatosis (WG) is a multisystem granulomatous inflammatory disorder of the autoimmune system. In its complete form, the diagnosis is based on necrotizing granulomas of the respiratory tract, generalized focal necrotizing vasculitis and focal necrotizing glomerulonephritis (Soukiasian et al. 1992). Ocular involvement includes conjunctivitis, keratitis, episcleritis, uveitis, retinal vasculitis, optic neuropathy and orbital infiltrative deposits. Visual loss is to be expected in 85% of individuals with severe necrotizing posterior scleritis, even with aggressive immunosuppression (Foster & Vitale 2004; Pakrou et al. 2006). A 40-year-old man was examined in the ophthalmology unit because of scleritis. He was suffering left-side facial nerve palsy and chronic rhinitis. The WG diagnosis was based on respiratory tract findings in high-resolution computed tomography (CT), elevated serum anti-neutrophil cytoplasmic antibody, inflammatory changes in nasal mucosa biopsy sample and scleral inflammation in magnetic resonance images. A subcutaneous methotrexate injection (7.5 mg) per week and daily oral prednisolone (60 mg dose) were initiated. In addition, prednisolone eyedrops every 1 or 2 hr and dexamethasone ointment for use at night were applied topically. The patient's visual acuity (VA) was 1.0 (−3.50cyl+0.25ax70) in the right eye and 1.0 (−2.75) in the left eye. Two months later, the right eye's VA had decreased to 0.8 (−5.0cyl+1.0ax35) simultaneously with observation of a nodular scleritis. During follow-up, drug therapy included subcutaneus (15 mg) methotrexate injections weekly, oral prednisolone (40 mg) each day and topical non-steroidal anti-inflammatory drops without preservatives to be used 4–6 times each day with dexamethasone ointment at night. Four months after the WG diagnosis, VA had declined further to 0.6 after the best corrected refraction (−7.0cyl+ 6.50ax35) and signs of necrotizing scleritis were seen in the right eye (Fig. 1). In addition, a moderate number of anterior-chamber inflammatory cells were seen in a slit-lamp examination. The cyclophosphamide (CYP) infusion every 2 weeks was supplemented to the prevailing treatments. Seven months after the WG diagnosis, VA had declined to 0.16 (−7.0cyl+6.50ax35) and an abundance of inflammatory cells were seen not only in the anterior chamber but also in vitreous body in the right eye. At this time, there was also scleral thinning and clear early signs of necrotizing scleritis in the left eye. In addition, the presence of the anterior-chamber inflammatory cells was observed in slit-lamp examination. However, the VA in the left eye was normal. Intravenous therapy with infliximab 300 mg, a specific tumour necrosis factor-α inhibitor (Bartolucci et al. 2002; Sahlin et al. 2008), was initiated for every 4 weeks and the CYP treatment was terminated. The inflammatory signs declined rapidly in both eyes in response to the infliximab infusion. Maintenance drug therapy consisted of 600 mg infliximab every month, subcutaneous methotrexate 20 mg per week and oral prednisolone, the dose of which was decreased slowly to 25 mg, as well as topical dexamethasone. Two years after the WG diagnosis, which was associated with necrotizing scleritis, the individual could count fingers with the right eye while VA was 1.2 (−3.0) in the left eye. Photographs of the right eye, which proceeded to blindness despite conventional treatment (upper panels); photographs of the left eye with preserved normal visual acuity after infliximab treatment in necrotizing scleritis of Wegener's granulomatosis (lower panels). One eye proceeded to blindness because of the necrotizing scleritis in spite of the conventional WG treatment. However, a clear response was observed in the fellow eye after initiation of infliximab, a compound that is used widely in a variety of inflammatory diseases (Bartolucci et al. 2002; Sahlin et al. 2008). It can only be speculated whether the right eye would have better visual acuity had there been earlier initiation of the infliximab therapy. It does seem that infliximab represents a more effective therapeutic option in the treatment of WG, is superior to standard immunosuppression therapy and may offer better prospects for patients with a previously poor prognosis.