Low-dose mepolizumab is effective as an add-on therapy for treating long-lasting peripheral neuropathy in patients with eosinophilic granulomatosis with polyangiitis

Mod Rheumatol. 2022 Feb 28;32(2):387-395. doi: 10.1093/mr/roab005.

Abstract

Objective: To assess the effectiveness of low-dose mepolizumab as an add-on therapy for treating peripheral neurological symptoms in eosinophilic granulomatosis with polyangiitis (EGPA).

Methods: We prospectively studied 13 EGPA patients with conventional treatment-resistant peripheral neuropathy. Their symptoms (pain, numbness, and muscle weakness) were assessed on a visual analogue scale (VAS) before and after 12 months of mepolizumab therapy (100 mg every 4 weeks). Peripheral eosinophil levels and several biomarkers including urinary levels of eosinophil-derived neurotoxin (EDN) were measured before and after therapy.

Results: VAS scores for pain and numbness significantly improved after 12 months of mepolizumab therapy (from 67.0 to 48.0, P = 0.012, and from 67.0 to 51.0, P = 0.017, respectively). However, the VAS score for muscle weakness did not improve (P = 0.36). There were significant correlations between treatment-related changes in urinary EDN levels from baseline to 6 months later and percent changes in the VAS scores of pain and numbness (r = 0.75, P = 0.020; r = 0.88, P = 0.002).

Conclusions: Treatment-resistant peripheral neuropathy in EGPA was significantly improved by low-dose mepolizumab, and effectiveness was correlated with decreased urinary EDN. Because the possibility of a placebo effect cannot be formally excluded, placebo-controlled studies will be required in the future.

Keywords: Eosinophil-derived neurotoxin; eosinophilic granulomatosis with polyangiitis; mepolizumab; peripheral neuropathy; prospective observational study.

MeSH terms

  • Antibodies, Monoclonal, Humanized / therapeutic use
  • Churg-Strauss Syndrome* / drug therapy
  • Granulomatosis with Polyangiitis* / drug therapy
  • Humans
  • Peripheral Nervous System Diseases* / complications
  • Peripheral Nervous System Diseases* / drug therapy

Substances

  • Antibodies, Monoclonal, Humanized
  • mepolizumab