Scleritis Caused by Eosinophilic Granulomatosis with Polyangiitis: A Case Report
DOI:
https://doi.org/10.71204/7agtqd88Keywords:
Eosinophilic Granulomatosis with Polyangiitis, ANCA, Scleritis, Autoimmune Disease, ANCA-associated VasculitisAbstract
To report a case of an 81-year-old female with scleritis due to eosinophilic granulomatosis with polyangiitis (EGPA) and emphasize the importance of accurate diagnosis and appropriate management. The patient's initial symptoms, including scleritis and systemic manifestations such as recurrent fever, were observed. Laboratory tests, imaging studies, ANCA testing, and bone marrow biopsy were performed for diagnosis. Treatment with methylprednisolone and cyclophosphamide was administered, and the response was monitored by observing the resolution of scleritis and normalization of blood parameters. The patient was initially misdiagnosed. However, through comprehensive examinations, the EGPA diagnosis was confirmed. The treatment was effective, with the scleritis being resolved and blood parameters returning to normal. EGPA is a rare ANCA-associated vasculitis with complex and non-specific manifestations. ANCA is a significant biomarker, and comprehensive laboratory testing is crucial for accurate diagnosis. Treatment consists of induction and maintenance phases. Ophthalmologists should be aware of the possibility of systemic autoimmune diseases in scleritis patients, especially those with eosinophilia and systemic symptoms, and cooperate with internal medicine specialists for optimal patient management.
References
Berti, A., Boukhlal, S., Groh, M., & Cornec, D. (2020). Eosinophilic granulomatosis with polyangiitis: the multifaceted spectrum of clinical manifestations at different stages of the disease. Expert Review of Clinical Immunology, 16(1), 51-61. DOI: https://doi.org/10.1080/1744666X.2019.1697678
Churg, J., & Strauss, L. (1951). Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. The American journal of pathology, 27(2), 277.
Comarmond, C., Pagnoux, C., Khellaf, M., Cordier, J. F., Hamidou, M., Viallard, J. F., ... & French Vasculitis Study Group. (2013). Eosinophilic granulomatosis with polyangiitis (Churg‐Strauss): clinical characteristics and long‐term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort. Arthritis & Rheumatism, 65(1), 270-281. DOI: https://doi.org/10.1002/art.37721
Cottin, V., Bel, E., Bottero, P., Dalhoff, K., Humbert, M., Lazor, R., ... & Cordier, J. F. (2016). Respiratory manifestations of eosinophilic granulomatosis with polyangiitis (Churg–Strauss). European Respiratory Journal, 48(5), 1429-1441. DOI: https://doi.org/10.1183/13993003.00097-2016
Durel, C. A., Berthiller, J., Caboni, S., Jayne, D., Ninet, J., & Hot, A. (2016). Long‐term followup of a multicenter cohort of 101 patients with eosinophilic granulomatosis with polyangiitis (Churg‐Strauss). Arthritis care & research, 68(3), 374-387. DOI: https://doi.org/10.1002/acr.22686
Fujimoto, S., Watts, R. A., Kobayashi, S., Suzuki, K., Jayne, D. R., Scott, D. G., ... & Nunoi, H. (2011). Comparison of the epidemiology of anti-neutrophil cytoplasmic antibody-associated vasculitis between Japan and the UK. Rheumatology, 50(10), 1916-1920. DOI: https://doi.org/10.1093/rheumatology/ker205
Furuta, S., Iwamoto, T., & Nakajima, H. (2019). Update on eosinophilic granulomatosis with polyangiitis. Allergology International, 68(4), 430-436. DOI: https://doi.org/10.1016/j.alit.2019.06.004
Hazebroek, M. R., Kemna, M. J., Schalla, S., Sanders-van Wijk, S., Gerretsen, S. C., Dennert, R., ... & Heymans, S. (2015). Prevalence and prognostic relevance of cardiac involvement in ANCA-associated vasculitis: eosinophilic granulomatosis with polyangiitis and granulomatosis with polyangiitis. International journal of cardiology, 199, 170-179. DOI: https://doi.org/10.1016/j.ijcard.2015.06.087
Hinojosa-Azaola, A., García-Castro, A., Juárez-Flores, A., & Recillas-Gispert, C. (2019). Clinical significance of ocular manifestations in granulomatosis with polyangiitis: association with sinonasal involvement and damage. Rheumatology international, 39(3), 489-495. DOI: https://doi.org/10.1007/s00296-019-04242-7
Jennette, J. C., Falk, R. J., Bacon, P. A. (2013). 2012 revised international chapel hill consensus conference nomenclature of vasculitides. Arthritis Rheum, 65, 1-11. DOI: https://doi.org/10.1007/s10157-013-0869-6
Keogh, K. A., & Specks, U. (2006). Churg-Strauss syndrome. In Seminars in respiratory and critical care medicine, 27(02), 148-157. DOI: https://doi.org/10.1055/s-2006-939518
Kitching, A. R., Anders, H. J., Basu, N., Brouwer, E., Gordon, J., Jayne, D. R., ... & Kain, R. (2020). ANCA-associated vasculitis. Nature reviews Disease primers, 6(1), 71. DOI: https://doi.org/10.1038/s41572-020-0204-y
Koike, H., Nishi, R., Ohyama, K., Morozumi, S., Kawagashira, Y., Furukawa, S., ... & Katsuno, M. (2022). ANCA-associated vasculitic neuropathies: a review. Neurology and Therapy, 11(1), 21-38. DOI: https://doi.org/10.1007/s40120-021-00315-7
Lanham, J. G., Elkon, K. B., Pusey, C. D., & Hughes, G. R. (1984). Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine, 63(2), 65-81. DOI: https://doi.org/10.1097/00005792-198403000-00001
Lutalo, P. M., & D'Cruz, D. P. (2014). Diagnosis and classification of granulomatosis with polyangiitis (aka Wegener's granulomatosis). Journal of autoimmunity, 48, 94-98. DOI: https://doi.org/10.1016/j.jaut.2014.01.028
Moosig, F., Bremer, J. P., Hellmich, B., Holle, J. U., Holl-Ulrich, K., Laudien, M., ... & Gross, W. L. (2013). A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg–Strauss, EGPA): monocentric experiences in 150 patients. Annals of the rheumatic diseases, 72(6), 1011-1017. DOI: https://doi.org/10.1136/annrheumdis-2012-201531
Nevares, A., Raut, R., Libman, B., & Hajj-Ali, R. (2020). Noninfectious autoimmune scleritis: recognition, systemic associations, and therapy. Current Rheumatology Reports, 22, 1-10. DOI: https://doi.org/10.1007/s11926-020-0885-y
Raffray, L., & Guillevin, L. (2020). Updates for the treatment of EGPA. La Presse Médicale, 49(3), 104036. DOI: https://doi.org/10.1016/j.lpm.2020.104036
Saku, A., Furuta, S., Hiraguri, M., Ikeda, K., Kobayashi, Y., Kagami, S. I., ... & Nakajima, H. (2018). Longterm outcomes of 188 Japanese patients with eosinophilic granulomatosis with polyangiitis. The Journal of Rheumatology, 45(8), 1159-1166. DOI: https://doi.org/10.3899/jrheum.171352
Sinico, R. A., Di Toma, L., Maggiore, U., Bottero, P., Radice, A., Tosoni, C., ... & Buzio, C. (2005). Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg‐Strauss syndrome. Arthritis & Rheumatism, 52(9), 2926-2935. DOI: https://doi.org/10.1002/art.21250
Szczeklik, W., Sokołowska, B. M., Żuk, J., Mastalerz, L., Szczeklik, A., & Musiał, J. (2011). The course of asthma in Churg–Strauss syndrome. Journal of Asthma, 48(2), 183-187. DOI: https://doi.org/10.3109/02770903.2010.551796
Tsurikisawa, N., Oshikata, C., Kinoshita, A., Tsuburai, T., & Saito, H. (2017). Longterm prognosis of 121 patients with eosinophilic granulomatosis with polyangiitis in Japan. The Journal of Rheumatology, 44(8), 1206-1215. DOI: https://doi.org/10.3899/jrheum.161436
Turk, M. A., Hayworth, J. L., Nevskaya, T., & Pope, J. E. (2021). Ocular manifestations in rheumatoid arthritis, connective tissue disease, and vasculitis: a systematic review and metaanalysis. The Journal of Rheumatology, 48(1), 25-34. DOI: https://doi.org/10.3899/jrheum.190768
Vergouwen, D. P. C., Rothová, A., Ten Berge, J. C., Verdijk, R. M., van Laar, J. A. M., Vingerling, J. R., & Schreurs, M. W. J. (2020). Current insights in the pathogenesis of scleritis. Experimental Eye Research, 197, 108078. DOI: https://doi.org/10.1016/j.exer.2020.108078
Watanabe, R., & Hashimoto, M. (2023). Eosinophilic granulomatosis with polyangiitis: latest findings and updated treatment recommendations. Journal of Clinical Medicine, 12(18), 5996. DOI: https://doi.org/10.3390/jcm12185996
Wechsler, M. E., Akuthota, P., Jayne, D., Khoury, P., Klion, A., Langford, C. A., ... & Gleich, G. J. (2017). Mepolizumab or placebo for eosinophilic granulomatosis with polyangiitis. New England Journal of Medicine, 376(20), 1921-1932. DOI: https://doi.org/10.1056/NEJMoa1702079
Downloads
Published
Issue
Section
License
Copyright (c) 2025 Wenbo Hou, Xin Li, Xuguang Sun (Author)

This work is licensed under a Creative Commons Attribution 4.0 International License.
All articles published in this journal are licensed under the Creative Commons Attribution 4.0 International License (CC BY 4.0). This license permits unrestricted use, distribution, and reproduction in any medium, provided the original author(s) and source are properly credited. Authors retain copyright of their work, and readers are free to copy, share, adapt, and build upon the material for any purpose, including commercial use, as long as appropriate attribution is given.
