Mepolizumab administration for eosinophilic granulomatosis with polyangiitis in an elderly patient with an abrupt onset of limb ischaemia and peripheral neuropathy: A case-based literature review

Mod Rheumatol Case Rep. 2023 Jan 3;7(1):142-147. doi: 10.1093/mrcr/rxac073.

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a type of anti-neutrophil cytoplasmic antibody-associated vasculitis characterised by small- to medium-sized vessel vasculitis and is typically associated with eosinophilic granulomatous inflammation. EGPA can affect any organ system, most commonly the lungs, skin, and the nervous system. However, limb ulcers are rare complications and have only been described in few case reports. Furthermore, no documented cases of EGPA have been treated with mepolizumab. Herein, we report a case of an 86-year-old Japanese woman with anti-neutrophil cytoplasmic antibody-negative EGPA, who had an abrupt onset of upper limb ulcers and bilateral foot drop due to multiple mononeuropathy. Clinicopathological sural nerve biopsy showed eosinophil-associated vascular damage. The patient was administered steroids, intravenous immunoglobulin, vasodilators, and mepolizumab; this resulted in clinical improvement of her finger ulcers and peripheral neuropathy without any adverse effects. In cases of an abrupt onset of limb ischaemia and peripheral neuropathy, physicians should consider the possibility of EGPA as a differential diagnosis. Furthermore, the early administration of mepolizumab might yield better outcomes in terms of improving limb ischaemia and peripheral neuropathy.

Keywords: Eosinophilic granulomatosis with polyangiitis; anti-neutrophil cytoplasmic antibody-associated vasculitis; limb ulcer; mepolizumab; peripheral neuropathy.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Aged, 80 and over
  • Churg-Strauss Syndrome* / diagnosis
  • Eosinophilia*
  • Female
  • Granulomatosis with Polyangiitis* / diagnosis
  • Humans
  • Ischemia / diagnosis
  • Ischemia / drug therapy
  • Ischemia / etiology
  • Peripheral Nervous System Diseases* / diagnosis
  • Peripheral Nervous System Diseases* / drug therapy
  • Peripheral Nervous System Diseases* / etiology
  • Ulcer

Substances

  • mepolizumab

Supplementary concepts

  • digital ulcers