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Eosinophilic Granulomatosis with Polyangiitis Presenting as Unilateral Acute Anterior Ischaemic Optic Neuropathy.

ABSTRACT: Eosinophilic granulomatosis with polyangiitis (eGPA) is a rare vasculitis of small-medium sized vessels that can cause both anterior and posterior ischaemic optic neuropathies. Herein, the authors present a rare case of eGPA presenting initially as an acute unilateral anterior ischaemic optic neuropathy from short posterior ciliary artery vasculitis. The diagnosis presented a challenge as clinical and histopathological evidence suggested allergic rhinosinusitis, and no invasive fungal sinusitis was found. The high serum eosinophilia, asthma, optic neuropathy and paranasal sinus abnormalities fulfilled the criteria for a diagnosis of eGPA. Furthermore serum was positive for myeloperoxidase antibodies. Subsequently the case was successfully treated with oral glucocorticoids and intravenous rituximab.

SUBMITTER: Fong A

PROVIDER: S-EPMC8158048 | biostudies-literature | 2021

REPOSITORIES: biostudies-literature

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Eosinophilic Granulomatosis with Polyangiitis Presenting as Unilateral Acute Anterior Ischaemic Optic Neuropathy.

Fong Anthony A Ahmed Shahzada S Ramalingam Satheesh S Brown Rachel M RM Harper Lorraine L Mollan Susan P SP

Neuro-ophthalmology (Aeolus Press) 20200625 2

Eosinophilic granulomatosis with polyangiitis (eGPA) is a rare vasculitis of small-medium sized vessels that can cause both anterior and posterior ischaemic optic neuropathies. Herein, the authors present a rare case of eGPA presenting initially as an acute unilateral anterior ischaemic optic neuropathy from short posterior ciliary artery vasculitis. The diagnosis presented a challenge as clinical and histopathological evidence suggested allergic rhinosinusitis, and no invasive fungal sinusitis ...[more]

PMID: 34108782

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Project description:BackgroundEosinophilic granulomatosis with polyangiitis is an eosinophilic vasculitis. Mepolizumab, an anti-interleukin-5 monoclonal antibody, reduces blood eosinophil counts and may have value in the treatment of eosinophilic granulomatosis with polyangiitis.MethodsIn this multicenter, double-blind, parallel-group, phase 3 trial, we randomly assigned participants with relapsing or refractory eosinophilic granulomatosis with polyangiitis who had received treatment for at least 4 weeks and were taking a stable prednisolone or prednisone dose to receive 300 mg of mepolizumab or placebo, administered subcutaneously every 4 weeks, plus standard care, for 52 weeks. The two primary end points were the accrued weeks of remission over a 52-week period, according to categorical quantification, and the proportion of participants in remission at both week 36 and week 48. Secondary end points included the time to first relapse and the average daily glucocorticoid dose (during weeks 48 through 52). The annualized relapse rate and safety were assessed.ResultsA total of 136 participants underwent randomization, with 68 participants assigned to receive mepolizumab and 68 to receive placebo. Mepolizumab treatment led to significantly more accrued weeks of remission than placebo (28% vs. 3% of the participants had ≥24 weeks of accrued remission; odds ratio, 5.91; 95% confidence interval [CI], 2.68 to 13.03; P<0.001) and a higher percentage of participants in remission at both week 36 and week 48 (32% vs. 3%; odds ratio, 16.74; 95% CI, 3.61 to 77.56; P<0.001). Remission did not occur in 47% of the participants in the mepolizumab group versus 81% of those in the placebo group. The annualized relapse rate was 1.14 in the mepolizumab group, as compared with 2.27 in the placebo group (rate ratio, 0.50; 95% CI, 0.36 to 0.70; P<0.001). A total of 44% of the participants in the mepolizumab group, as compared with 7% of those in the placebo group, had an average daily dose of prednisolone or prednisone of 4.0 mg or less per day during weeks 48 through 52 (odds ratio, 0.20; 95% CI, 0.09 to 0.41; P<0.001). The safety profile of mepolizumab was similar to that observed in previous studies.ConclusionsIn participants with eosinophilic granulomatosis with polyangiitis, mepolizumab resulted in significantly more weeks in remission and a higher proportion of participants in remission than did placebo, thus allowing for reduced glucocorticoid use. Even so, only approximately half the participants treated with mepolizumab had protocol-defined remission. (Funded by GlaxoSmithKline and the National Institute of Allergy and Infectious Diseases; ClinicalTrials.gov number, NCT02020889 .).

Project description:BackgroundDysthyroid optic neuropathy is the most commonly suspected diagnosis of optic neuropathy in Graves' patients; however, other causes need to be ruled out. We present a unique case of optic neuropathy secondary to hypertrophic pachymeningitis with antineutrophil cytoplasmic antibody-associated vasculitis, which was suspected to be antithyroid drug related.Case presentationA 79-year-old Japanese male presented with acute visual loss in the left eye. He had a 24-year history of Graves' disease and was taking methimazole. Best-corrected visual acuity was 0.8 in the right eye and light perception in the left eye, and relative afferent pupillary defect in the left eye was seen. Ocular movement was normal, and there were no findings explaining visual loss in intermediate optic media and fundus in the left eye. Contrast-enhanced magnetic resonance imaging demonstrated thickened dura mater. Tests for myeloperoxidase-antineutrophil cytoplasmic antibody, proteinuria, and hematuria were positive; pulmonary nodule lesions and a blood clot in the left lower leg were also found. After excluding the presence of diseases that could lead to hypertrophic pachymeningitis, we diagnosed optic neuropathy due to hypertrophic pachymeningitis with granulomatosis with polyangiitis-a subtype of antineutrophil cytoplasmic antibody-associated vasculitis. Since he had history of using methimazole, antineutrophil cytoplasmic antibody-associated vasculitis was considered as drug related. We started high-dosage steroid pulse therapy followed by 1 mg/kg body weight daily of oral prednisolone, and subsequently tapered. Methimazole was stopped. Best-corrected visual acuity recovered to 0.9, 2 weeks after starting treatment. Though myeloperoxidase-antineutrophil cytoplasmic antibody remained negative, the symptom relapsed 6 months after treatment initiation. We gave a second high-dose steroid pulse therapy followed by prednisolone tapered together with methotrexate. Remission remained, and using 4 mg/week methotrexate without prednisolone, myeloperoxidase-antineutrophil cytoplasmic antibody was kept within the normal limit until now, 4 years after onset.ConclusionWe present a case of optic neuropathy with hypertrophic pachymeningitis related to antineutrophil cytoplasmic antibody-associated vasculitis, which was suspected to be drug related. The patient had good visual recovery after quitting the drug and receiving immunosuppressive therapy with systemic steroids. Hypertrophic pachymeningitis with antineutrophil cytoplasmic antibody-associated vasculitis related to antithyroid drugs should be considered as a differential diagnosis for optic neuropathy in Graves' patients in whom optic nerve compression is not obvious.

Dataset Information

Eosinophilic Granulomatosis with Polyangiitis Presenting as Unilateral Acute Anterior Ischaemic Optic Neuropathy.

Publications

Eosinophilic Granulomatosis with Polyangiitis Presenting as Unilateral Acute Anterior Ischaemic Optic Neuropathy.

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