Optic neuropathy secondary to granulomatosis with polyangiitis in a patient with Graves' disease: a case report

Abstract

Background: Dysthyroid 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 presentation: A 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.

Conclusion: We 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.

Keywords: Antineutrophil cytoplasmic antibody-associated vasculitis; Drug-induced ANCA-associated vasculitis; Graves’ disease; Hypertrophic pachymeningitis; Myeloperoxidase-antineutrophil cytoplasmic antibody.

Fig. 1

Fundus photograph of the left eye at the first visit. There were no findings that demonstrated that the best-corrected visual acuity of this eye was “light perception” in the fundus. No swelling was seen in the optic disc

Fig. 2

MRI and CT images. A shows a plain MRI image with mild enlargement of the external eye muscle in both eyes, with no findings of direct compression to the optic nerve by enlarged extraocular muscles nor stretching of the optic nerve by proptosis. B shows a coronal section of a plain head CT scan demonstrating the left ethmoid sinus filled with soft tissue and bone destructions in anterior cranial base (white arrow head). C shows contrast-enhanced MRI demonstrating the left optic nerve surrounded by enhancement (white arrow), suggesting inflammation at the optic canal. D shows a coronal section of contrast-enhanced MRI demonstrating thickened mucous membranes in the left ethmoid sinus and enhanced thicken dura of the bottom of left frontal cortex (white arrows)

Fig. 3

Results of Goldman visual field in the left eye with clinical course. A central scotoma is seen at 6 days after administering systemic steroids (A). The central scotoma disappeared in the 9 weeks after starting systemic steroids (B). Scotomas are seen when optic neuropathy relapse occurred at 6 months after starting treatment (C)