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Myeloperoxidase and associated lung disease: Review of the latest developments

Hannah Hu1,2, Karuna Keat1,3

  • 1Department of Immunology, Campbelltown Hospital, Sydney, New South Wales, Australia.

International Journal of Rheumatic Diseases
|September 9, 2021
PubMed

Insights

Myeloperoxidase (MPO)-ANCA-positive interstitial lung disease (ILD) presents challenges, differing in pathophysiology from MPO-ANCA-negative ILD. Management strategies are evolving, requiring tailored approaches beyond standard ILD care.

Area of Science:

  • Pulmonology
  • Immunology
  • Rheumatology

Background:

  • Myeloperoxidase (MPO) anti-neutrophil cytoplasmic antibodies (ANCA) are linked to lung diseases, primarily interstitial lung disease (ILD).
  • A distinct group of patients present with MPO-ANCA and ILD, but without ANCA-associated vasculitis.
  • Distinguishing this MPO-ANCA-positive ILD cohort from MPO-ANCA-negative ILD is clinically and radiologically difficult, suggesting different underlying mechanisms.

Purpose of the Study:

  • To summarize the proposed pathophysiology of MPO-ANCA-positive ILD.
  • To review current management strategies for MPO-ANCA-positive ILD.
  • To highlight the need for tailored treatment approaches and novel therapeutic agents.

Main Methods:

  • Literature review focusing on pathophysiology and management of MPO-ANCA-positive ILD.
  • Analysis of current evidence regarding immunosuppression, anti-fibrotic therapies, and emerging treatments.
  • Discussion of monitoring protocols for ANCA-associated vasculitis development.

Main Results:

  • Proposed pathophysiological mechanisms for MPO-ANCA-positive ILD are outlined.
  • Current management includes monitoring for vasculitis, immunosuppression, and anti-fibrotic agents.
  • Novel therapeutic agents are under investigation but not yet trialled in humans.

Conclusions:

  • MPO-ANCA-positive ILD represents a unique clinical entity requiring distinct management strategies.
  • Further research into pathophysiology and novel treatments is crucial for improving patient outcomes.
  • Close monitoring for the development of ANCA-associated vasculitis is essential in this patient cohort.
Keywords:
anti-neutrophil cytoplasmic antibodiesfibrotic lung diseaseinterstitial lung diseasemyeloperoxidase antibodyvasculitis

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