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CASE REPORT
A challenging diagnosis of MPO-C-ANCA EGPA
  1. Gareth Lim1,
  2. Sheryl Lim1,
  3. Shang-Ian Tee2 and
  4. Chai Yiing Ling3
  1. 1 Internal Medicine, Khoo Teck Puat Hospital, National Healthcare Group, Singapore
  2. 2 Dermatology, National Skin Centre, Singapore, Singapore
  3. 3 Rheumatology, Khoo Teck Puat Hospital, Singapore
  1. Correspondence to Dr Gareth Lim, gareth.lim{at}mohh.com.sg

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystemic small-vessel vasculitic disease that can present with positive MPO-P-ANCA (myeloperoxidase–perinuclear–anti-neutrophil cytoplasmic antibody). It is a rare condition that is difficult to diagnose. We present the case of a 64-year-old man with late-onset adult asthma and treated nasopharyngeal carcinoma who initially presented to us with proximal myopathy. Thereafter, he developed a constellation of fleeting symptoms which included rhinosinusitis, mononeuritis multiplex, skin vasculitis and arthritis. Blood investigations showed that he had eosinophilia, and skin biopsy demonstrated dermal vasculitis with eosinophils. He was found to be MPO-C-ANCA positive, and although initially thought to have granulomatosis with polyangiitis, the diagnosis was later revised to EGPA. This case highlights the diagnostic challenges with atypical presentations of EGPA and also presents a rare case of positive MPO-C-ANCA that has never been described in EGPA before.

  • rheumatology
  • vasculitis
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Footnotes

  • Contributors GL, SL and CYL contributed to the care and eventual diagnosis of the patient in the case report. SL performed the literature review and wrote the background section of the paper. GL wrote the case summary and discussion sections, and was responsible for the overall consolidation of the article. CYL contributed ideas, performed the literature review and made additions to the final manuscript. S-IT interpreted and reported the biopsy results of this case and contributed to writing and editing both the background and the case summary sections.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Patient consent for publication Obtained.

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