Воспаление сосудов привело к слепоте: редкий случай EGPA у японской пациентки

A rare, severe initial manifestation of the eosinophilic granulomatosis with polyangiitis (EGPA) subtype of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) has been reported in a Japanese woman, according to a case report published recently in Modern Rheumatology Case Reports. The unusual disease presentation, which was ultimately recognized as being anterior ischemic optic neuropathy (AION), involved sudden, painless vision loss in the patient’s left eye. Patients with AION require immediate, intensive immunosuppressive therapy to experience visual recovery. The patient was a 57-year-old woman with an 11-year history of bronchial asthma. Three days following the onset of her symptoms, she was admitted to the hospital. At the time, visual acuity testing revealed the ability to count fingers with her left eye and 20/20 eyesight in her right eye. Results of a funduscopy examination demonstrated left optic disc edema. Magnetic resonance imaging (MRI) scans of the orbit showed “local contrast enhancement of intraorbital fat around the left optic nerve and left retrobulbar fat at the posterior globe.” Additional MRI scans revealed an intravitreal protrusion of the left optic nerve disc. Other symptoms in this patient included myeloperoxidase-ANCA positivity, peripheral eosinophilia, sinusitis, nasal polyps, pulmonary involvement, and mononeuritis multiplex. Her diagnosis was EGPA with AION. The patient underwent urgent administration of intravenous pulse methylprednisolone 3 days after the onset of her symptoms, which was followed 2 weeks later with mepolizumab. Her eosinophilic inflammation resolved and she had improved imaging results, however, no improvements in her visual acuity were detected. The researchers noted that a literature review identified 14 articles published between 1983 and 2025 in which EGPA-associated AION was identified. Including the current case, 11 cases with available data that involved 14 eyes underwent analysis. Findings from the review showed that visual recovery was reported in 29% (4 of 14) of the patients’ eyes that were analyzed. A median time of 2.5 days from onset of visual symptoms to initiation of treatment was observed. Of 12 eyes in which severe visual impairment was reported prior to treatment, visual recovery was seen only in those eyes that received earlier treatment or those treated with cyclophosphamide except for 1 eye. There was no recovery reported among patients with delayed treatment or those who did not receive cyclophosphamide therapy. “This case highlights the potential of AION as an initial manifestation of EGPA,” the authors wrote. “Both earlier initiation and adequate intensity of immunosuppressive therapy, particularly including cyclophosphamide when clinically appropriate, may be important for visual recovery in EGPA-associated severe AION,” they concluded. This article originally appeared on Rare Disease Advisor