Intravenous cyclophosphamide and immunoglobulin ameliorated visual field defects in a patient with eosinophilic granulomatosis with polyangiitis

Abstract

Treating ocular involvement in eosinophilic granulomatosis with polyangiitis (EGPA) can be challenging. We present the case of a 37-year-old woman with EGPA who had severe bilateral visual field defects. Laboratory results showed leukocytosis (17,500 WBC/μL, 25.8 % eosinophils), negative MPO-ANCA titer, and elevated PR3-ANCA level (33.2 IU/mL). Diffusion-weighted MRI revealed bilateral hyperintense occipital lesions, which were more prominent on the left. Her therapy initially included a steroid pulse, followed by daily prednisolone, but her visual field defects remained refractory. The addition of intravenous cyclophosphamide (5 courses) and intravenous immunoglobulin decreased her optic neuropathy and resolved her visual field defects.

Keywords: ANCA-Associated vasculitis; Cyclophosphamide; Eosinophilic granulomatosis with polyangiitis; IVIG; Optic neuritis; Visual field defects.

Fig. 1

Axial brain MRI images before, during, and after treatment with PSL, IVCY, and IVIG. Pretreatment images (a) show high-intensity lesions (red arrows) in the optic nerve region of the occipital lobes on FLAIR (A, B, C), DWI (D, E, F), and T2WI (G, H, I), confirming acute cerebral infarction at diagnosis. Axial MRI images 3 months after starting PSL (b). The high-intensity lesions (red arrows) in the occipital lobe optic nerve region decreased in size on FLAIR (J, K, L) and DWI (M, N, O), whereas the T2WI images (P, Q, R) indicated an old infarction at the same site. Seven months after starting PSL, 1 month after the 5th IVCY dose, and 2 months after IVIG (c), the high-intensity lesions were barely visible (red arrow) on FLAIR (S, T, U) and DWI (V, W, X) and decreased (red arrows) on T2WI (Y, Z, AA) images. Abbreviations: DWI, diffusion-weighted image; FLAIR, fluid-attenuated inversion recovery; IVCY, intravenous cyclophosphamide; IVIG, intravenous immunoglobulin; PSL, prednisolone; T2WI, T2-weighted image. (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)

Fig. 1

Axial brain MRI images before, during, and after treatment with PSL, IVCY, and IVIG. Pretreatment images (a) show high-intensity lesions (red arrows) in the optic nerve region of the occipital lobes on FLAIR (A, B, C), DWI (D, E, F), and T2WI (G, H, I), confirming acute cerebral infarction at diagnosis. Axial MRI images 3 months after starting PSL (b). The high-intensity lesions (red arrows) in the occipital lobe optic nerve region decreased in size on FLAIR (J, K, L) and DWI (M, N, O), whereas the T2WI images (P, Q, R) indicated an old infarction at the same site. Seven months after starting PSL, 1 month after the 5th IVCY dose, and 2 months after IVIG (c), the high-intensity lesions were barely visible (red arrow) on FLAIR (S, T, U) and DWI (V, W, X) and decreased (red arrows) on T2WI (Y, Z, AA) images. Abbreviations: DWI, diffusion-weighted image; FLAIR, fluid-attenuated inversion recovery; IVCY, intravenous cyclophosphamide; IVIG, intravenous immunoglobulin; PSL, prednisolone; T2WI, T2-weighted image. (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)

Fig. 1

Axial brain MRI images before, during, and after treatment with PSL, IVCY, and IVIG. Pretreatment images (a) show high-intensity lesions (red arrows) in the optic nerve region of the occipital lobes on FLAIR (A, B, C), DWI (D, E, F), and T2WI (G, H, I), confirming acute cerebral infarction at diagnosis. Axial MRI images 3 months after starting PSL (b). The high-intensity lesions (red arrows) in the occipital lobe optic nerve region decreased in size on FLAIR (J, K, L) and DWI (M, N, O), whereas the T2WI images (P, Q, R) indicated an old infarction at the same site. Seven months after starting PSL, 1 month after the 5th IVCY dose, and 2 months after IVIG (c), the high-intensity lesions were barely visible (red arrow) on FLAIR (S, T, U) and DWI (V, W, X) and decreased (red arrows) on T2WI (Y, Z, AA) images. Abbreviations: DWI, diffusion-weighted image; FLAIR, fluid-attenuated inversion recovery; IVCY, intravenous cyclophosphamide; IVIG, intravenous immunoglobulin; PSL, prednisolone; T2WI, T2-weighted image. (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)

Fig. 2

Visual field tests by Optical Coherence Tomography of the Peripapillary Retina and ON-Hogging Vessels in Glaucoma Suspects (OCTOPUS) at 3 and 7 months after treatment initiation. The visual field test at 3 months coincides with the brain MRI in Fig. 1(b). The left half of the visual field was severely deficient in both the right (a) and left (b) eyes, particularly in the left lower quadrant of the left eye (b). The second visual field test (c, right; d, left), performed 4 months after the first, coincides with the brain MRI in Fig. 1(c). After the second IVCY course, the 2 hyporeflective lesions in the lower left quadrant were decreased in size, and after IVIG therapy, all but one dark spot had faded and regressed, and many scotomas had disappeared. Abbreviations: IVCY, intravenous cyclophosphamide; IVIG, intravenous immunoglobulin; PSL, prednisolone.

Fig. 3

Clinical course of our patient from diagnosis of eosinophilic granulomatosis with polyangiitis until clinical remission. Abbreviations: IVCY, intravenous cyclophosphamide; IVIG, intravenous immunoglobulin; mPSL, methylprednisolone; PSL, prednisolone.