Peripheral ulcerative keratitis with necrotizing scleritis, manifestation of granulomatosis with polyangiitis
DOI:
https://doi.org/10.5281/zenodo.14617182Keywords:
Granulomatosis with Polyangiitis, Immunotherapy, Corneal Ulcer, Necrotizing Scleritis, RituximabAbstract
Background: Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmic antibody (ANCA) associated autoimmune disorder that typically affects small and/ or medium size vessels. A collaborative management form ophthalmologist and rheumatologist could lead to long term remission of the disease despite being yet a life threatening pathology.
Clinic case: A 61 years old masculine complied of recurrent cases of ocular discomfort managed as a bacterial conjunctivitis for 2 years, also patient with loss of visual acuity and weight loss mainly in the three previous months. After discard infectious diseases such as tuberculosis and syphilis being in a high incidence country GPA investigation were carried out and later diagnosed. He was to start proper treatment with rituximab but as systemic complications advance he could not managed to achieve disease control. Ocular involvement has been show to occur in around 30% percent of cases in several studies. It could cause scleritis as well as conjunctivitis, episcleritis, necrotizing keratitis, corneoscleral perforation, posterior uveitis, and optic neuritis. C-ANCA and high positive PR3 autoantibody have strong association and sensitivity with GPA. Rituximab has been prove to be the most accurate treatment for this disease.
Conclusions: Necrotizing scleritis and peripheral ulcerative keratitis can be manifestation of GPA. An ophthalmic check up may be the key to reach a promptly diagnosis of a tough systemic case.
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References
Jennette JC, Falk Rj, Bacon PA, et al . 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1-11.
Zhan Y, Amin S, Lung Ki, et al. Incidence, prevalence and risk factors of infectious uveitis and scleritis in the United States: A claims-based analysis. PLoS ONE. 2020;15(8):e0237995.
Puéchal X. Granulomatosis with polyangiitis (Wegener’s). Joint Bone Spine. 2020;87(6):572-8. doi: 10.1016/j.jbspin. 2020.06.005.
Junek ML, Zhao L, Garner S, et al. Ocular manifestations of anca-associated vasculitis. Rheumatology. 2022;62(7):2517- 24. doi: 10.1093/rheumatology/keac663.
Tarabishy AB, Schulte M, Papaliodis GN, et al. Wegener’s granulomatosis clinical manifestations, diferential diagnosis, and management of ocular and systematic disease. Surv Ophthalmol 2010;55;429-44.
American Academy of Ophthalmology. Uveitis and Ocular inflammation. AAO; San Francisco CA: 2022.
Whitcup SM. ‘Scleritis’, in Whitcup and Nussenblatt’s uveitis, e-book: Fundamentals and Clinical Practice. 5th edn. 2022; Elsevier.
Gupta Y, Kishore A, Kumari P, et al. Peripheral ulcerative keratitis. Survey of Ophthalmology. 2021;66(6):977–98. doi: 10.1016/j.survophthal.2021.02.013.
Dikshya B, Ranju KS, Sitaula S, et al. Case report peripheral ulcerative keratitis with nodular scleritis, presents as the primary manifestation of granulomatosis with polyangiitis. Annals of Medicine & Surgery. 2023;85(7):3658-3662. doi: 10.1097/ MS9.0000000000000871
Muñoz SA, Garmendia C, Bustuoabad V, et al. Escleritis Necrotizante Asociada a poliangeítis con granulomatosis (granulomatosis de Wegener). Revista Argentina de Reumatología. 2014;25(4):44-7. doi: 10.47196/rar.v25i4.96.
Eshak N, Walterscheid B, Tarbox J, et al. Necrotizing scleritis as the initial presentation of granulomatosis with polyangiitis. European Journal of Rheumatology. 2022;9(1):60-1. doi:10.5152/ eurjrheum.2020.20142.
Lorenzana-Blanco N, Alejandre-Alba N. Necrotizing scleritis. New England Journal of Medicine. 2020;383(19). doi: 10.1056/ nejmicm2004836. http://revistamedica.imss.gob.mx/ 6 Rev Med Inst Mex Seguro Soc. 2025;63(2):e6015
Garlapati P, Qurie A. Granulomatosis with polyangitis. Natl Libr Med; 2022. http://www.ncbi.nlm.mih.gov/books/NBK557827/.
De Groot K, Rasmussen N, Bacon PA, et al. Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineitrophil cytoplasmic Antibody Associated vasculitis. Arthritis Rhehum. 2005;52:2461-9.
Thietart S, Karras A, Augusto JF, et al. Evaluation of Rituximab for induction and maintenance therapy in patient 75 five years and older with antineutrophil cytoplasmic antibody-associated vasculitis. JAMA Network Open. 2022;89:1542.
Merkel PA, Niles JL, Mertz LE, et al. Long-term safety of Rituximab in granulomatosis with polyangiitis and in microscopic polyangiitis. Arthritis Care & Research. 2021;73(9):1372-8. doi: 10.1002/acr.24332.
Furuta S, Nakagomi D, Kobayashi Y, et al. Effect of reduced-dose vs high-dose glucocorticoids added to rituximab on remission induction in ANCA-associated vasculitis. JAMA. 2021; 325(21):2178. doi: 10.1001/jama.2021.6615.
Byszewska A, Skrzypiec I, Rymarz A, et al. Ocular Involvement of Granulomatosis with Polyangiitis. J Clin Med. 2023;12 (13):4448.
Sudharshan S, Nair N, Curl A, et al. Human Immunodeficiency virus and intraocular inflamation in the era of highly active anti retroviral therapy - An update. Indian J Ophthalmol. 2020;68: 1787-98.
Agrawal R, Testi I, Mahajan S, et al. Collaborative ocular tuberculosis study consensus guidelines on the management of tubercular uveitis—report 1. Ophthalmology. 2021;128(2):266– 276. doi: 10.1016/j.ophtha.2020.01.008.
Agrawal R, Testi I, Bodaghi B, et al. Collaborative ocular tuberculosis study consensus guidelines on the management of tubercular uveitis—report 2. Ophthalmology. 2021;128(2):277- 287. doi: 10.1016/j.ophtha.2020.06.052.
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