More Than a Cu-bit of Vision Loss

The patient was treated with five days of high dose intravenous steroids without improvement in his vision or strabismus. ANCA (160; <20 dils) returned elevated, with positive anti-proteinase 3 (PR3) antibodies (>8; <0.4). While the dural biopsy showed nonspecific vascular injury, and the elbow lesion biopsy suggested granulomatous inflammation, the clinical presentation of multiple cranial neuropathies with acute onset of symptoms, particularly the optic neuropathy, suggested an ischemic insult. This was supported by the lack of response to steroids and the MRI imaging. With support from the skin biopsy, a diagnosis of ANCA positive vasculitis, most consistent with polyangiitis with granulomatosis was given. He was transitioned from steroids to cyclophosphamide without progression (at two months when last seen). Repeat MRI at two weeks showed a significant decrease in the dural enhancement, with an increase in the abnormal T2 signal in the apical portion of the left optic nerve; there was still no enhancement of the nerve. He is followed by rheumatology with a plan for long-term immunosuppression. Additionally, the patient is followed by nephrology and pulmonology, despite no evidence at present for lung or renal disease. Polyangiitis with granulomatosis is an uncommon autoimmune disease that often affects the head and neck; neurologic manifestations are uncommon, and meningitis has only rarely been described in the past1 . In a large review, about a third of patients with ANCA-positive vasculitis presented with ophthalmic manifestations; only a small percent of these (<1%) present with oculomotor nerve palsy and/or optic neuropathy 2 Data are sparse, however there is some literature that suggests a poorer prognosis when anti-PR3 positive patients are compared with ANCA positive patients with positive anti-myeloperoxidase antibodies,3 and thus it is important to follow these patients closely and treat their disease aggressively.