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Rituximab for refractory necrotizing scleritis and peripheral ulcerative keratitis secondary to antineutrophil cytoplasmic antibody-negative granulomatosis with polyangiitis
Jun Yong Chow1, Wan Norliza Wan Muda2, Mae-Lynn Catherine Bastion3
1 Department of Ophthalmology, Universiti Kebangsaan Malaysia Medical Centre, Jalan Yaacob Latif, Bandar Tun Razak, Kuala Lumpur, Wilayah Persekutuan Kuala Lumpur; Department of Ophthalmology, Hospital Tengku Ampuan Afzan, Kuantan, Jalan Tanah Putih, Kuantan, Pahang, Malaysia
2 Department of Ophthalmology, Hospital Tengku Ampuan Afzan, Kuantan, Jalan Tanah Putih, Kuantan, Pahang, Malaysia
3 Department of Ophthalmology, Universiti Kebangsaan Malaysia Medical Centre, Jalan Yaacob Latif, Bandar Tun Razak, Kuala Lumpur, Wilayah Persekutuan Kuala Lumpur, Malaysia
Correspondence Address:
Prof. Mae-Lynn Catherine Bastion
Department of Ophthalmology, Faculty of Medieine, Universiti Kebangsaan Malaysia Medical centre, Jalan Yaacb Latif, 56000 Kuala Lumpur
Malaysia
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/ijo.IJO_3013_20
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A 52-year-old woman was diagnosed with bilateral necrotizing scleritis (NS) and peripheral ulcerative keratitis secondary to the antineutrophil cytoplasmic antibody (ANCA)-negative granulomatosis with polyangiitis (GPA) as evidenced by increased erythrocyte sedimentation rate, bilateral multiple cavitation lung nodules, and persistent microscopic hematuria. Infective workup and autoimmune tests were negative including the ANCA test. She received multiple courses of high dose intravenous methylprednisolone, intravenous cyclophosphamide, and oral methotrexate. Multiple tectonic patching operations were performed. Despite all the treatment, the disease continued to progress. Eventually, few doses of rituximab were given and the disease was stable for at least a year without any reactivation.
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