Scleritis Caused by Eosinophilic Granulomatosis with Polyangiitis: A Case Reporte
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.
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.
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.
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.
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.
Furuta, S., Iwamoto, T., & Nakajima, H. (2019). Update on eosinophilic granulomatosis with polyangiitis. Allergology International, 68(4), 430-436.
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.
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.
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.
Keogh, K. A., & Specks, U. (2006). Churg-Strauss syndrome. In Seminars in respiratory and critical care medicine, 27(02), 148-157.
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.
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.
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.
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.
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.
Nevares, A., Raut, R., Libman, B., & Hajj-Ali, R. (2020). Noninfectious autoimmune scleritis: recognition, systemic associations, and therapy. Current Rheumatology Reports, 22, 1-10.
Raffray, L., & Guillevin, L. (2020). Updates for the treatment of EGPA. La Presse Médicale, 49(3), 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.
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.
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.
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.
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.
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.
Watanabe, R., & Hashimoto, M. (2023). Eosinophilic granulomatosis with polyangiitis: latest findings and updated treatment recommendations. Journal of Clinical Medicine, 12(18), 5996.
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.
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