2026 Volume 43 Issue 2 Pages 125-132
Objective
Eosinophilic granulomatosis with polyangiitis (EGPA) is frequently complicated by neuropathy, requiring both remission induction and maintenance therapies. This study describes the clinical manifestations and treatment of 10 patients with EGPA presenting with mononeuritis multiplex.
Methods
We examined 10 patients treated at our institution who met the Ministry of Health, Labour and Welfare diagnostic criteria for EGPA, and presented with mononeuritis multiplex. Clinical manifestations, laboratory findings, treatment, and outcomes were evaluated.
Results
The cohort included four men and six women. The mean age at the onset of neurological symptoms was 60.5 years (range, 44–79 years). All patients had preceding asthma or sinusitis ; in nine patients, EGPA was diagnosed based on neurological symptoms. The mean disease duration of mononeuritis multiplex was 6.2 years. Eight patients presented with sensorimotor impairment, of whom two had sensory impairment only. Foot drop was observed in five patients, and pain in seven.
Blood tests revealed myeloperoxidase antineutrophil cytoplasmic antibody (MPO–ANCA) positivity in four patients, who commonly had additional organ lesions. Neurological symptoms frequently extended to the upper limbs. For remission induction therapy, glucocorticoids (GCs) were used in all cases. Further, nine patients received steroid pulse therapy, and four received concomitant immunosuppressants. Patients treated with GCs alone had a higher risk of relapse. Intravenous immunoglobulin (IVIg) was administered in seven patients, with multiple courses required in six cases with residual neurological symptoms. Mepolizumab (MEP) was combined in six patients ― in four cases for relapse and in two cases during steroid taper. Patients receiving azathioprine (AZA) or MEP in combination were able to taper prednisolone (PSL) to <5 mg/day.
The mean modified Rankin Scale (mRS) score before treatment was 3.8 (range, 2–4) ; after treatment, nine patients improved to a score of 1 and one patient to a score of 2.
Conclusion
In EGPA, mononeuritis multiplex was commonly characterized by sensory impairment with superimposed motor deficits in severe cases, and there were many cases accompanied by foot drop and pain. Steroid pulse therapy was effective for remission induction, while the addition of MEP facilitated glucocorticoid tapering, and was also effective for relapse. For residual neurological symptoms, repeated administration of IVIg should be considered.
