Peripheral Ulcerative Keratitis Associated with Granulomatosis with Polyangiitis Emerging Despite Cyclophosphamide, Successfully Treated with Rituximab

Yuya Fujita; Shoichi Fukui; Yushiro Endo; Sosuke Tsuji; Ayuko Takatani; Toshimasa Shimizu; Masataka Umeda; Ayako Nishino; Tomohiro Koga; Shin-ya Kawashiri; Naoki Iwamoto; Kunihiro Ichinose; Mami Tamai; Hideki Nakamura; Tomoki Origuchi; Ryotaro Ueki; Masafumi Uematsu; Kaori Ishida; Kuniko Abe; Atsushi Kawakami

doi:10.2169/internalmedicine.0215-17

CASE REPORTS

Peripheral Ulcerative Keratitis Associated with Granulomatosis with Polyangiitis Emerging Despite Cyclophosphamide, Successfully Treated with Rituximab

Yuya Fujita, Shoichi Fukui, Yushiro Endo, Sosuke Tsuji, Ayuko Takatani, Toshimasa Shimizu, Masataka Umeda, Ayako Nishino, Tomohiro Koga, Shin-ya Kawashiri, Naoki Iwamoto, Kunihiro Ichinose, Mami Tamai, Hideki Nakamura, Tomoki Origuchi, Ryotaro Ueki, Masafumi Uematsu, Kaori Ishida, Kuniko Abe, Atsushi Kawakami

Author information

Yuya Fujita
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Shoichi Fukui
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Department of Community Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
Yushiro Endo
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Sosuke Tsuji
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Ayuko Takatani
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Toshimasa Shimizu
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Masataka Umeda
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Medical Education Development Center, Nagasaki University Hospital, Japan
Ayako Nishino
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Center for Comprehensive Community Care Education Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
Tomohiro Koga
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Center for Bioinformatics and Molecular Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
Shin-ya Kawashiri
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Department of Community Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan
Naoki Iwamoto
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Kunihiro Ichinose
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Mami Tamai
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Hideki Nakamura
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Tomoki Origuchi
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan
Department of Rehabilitation Sciences, Nagasaki University Graduate School of Biomedical Sciences, Japan
Ryotaro Ueki
Department of Ophthalmology and Visual Sciences, Nagasaki University Graduate School of Biomedical Sciences, Japan
Masafumi Uematsu
Department of Ophthalmology and Visual Sciences, Nagasaki University Graduate School of Biomedical Sciences, Japan
Kaori Ishida
Department of Pathology, Nagasaki University Hospital, Japan
Kuniko Abe
Department of Pathology, Nagasaki University Hospital, Japan
Atsushi Kawakami
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan

Keywords: ANCA-associated vasculitis, granulomatosis with polyangiitis, peripheral ulcerative keratitis, rituximab, scleritis, Wegener's granulomatosis

JOURNAL OPEN ACCESS

2018 Volume 57 Issue 12 Pages 1783-1788

DOI https://doi.org/10.2169/internalmedicine.0215-17

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Abstract

A 67-year-old Japanese man was diagnosed with granulomatosis with polyangiitis based on the presence of right maxillary sinusitis, proteinase 3 antineutrophil cytoplasmic antibody positivity, and right scleritis. A conjunctival biopsy specimen showed neutrophil-predominant infiltration around the vessels without granuloma. Because there was a risk of blindness, pulsed methylprednisolone and intravenous cyclophosphamide pulse therapy (IVCY) were started. However, it was ineffective, and peripheral ulcerative keratitis newly emerged. We promptly switched the treatment from IVCY to rituximab, and ophthalmologists performed amniotic membrane transplantation, which avoided blindness. The close and effective working relationship between physicians and ophthalmologists improved our patient's ocular prognosis.

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