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Anti-Inflammatory and/or Anti-Fibrotic Treatment of MPO-ANCA-Positive Interstitial Lung Disease: A Short Review
Hideaki Yamakawa1,2, Yuko Toyoda3, Tomohisa Baba4
1Department of Respiratory Medicine, Saitama Red Cross Hospital, 1-5 Shintoshin, Chuo-ku, Saitama 330-8553, Japan.
Abstract:
The presence of a lung lesion is common in microscopic polyangiitis (MPA), and interstitial lung disease (ILD) can lead to a poor prognosis. Although myeloperoxidase antineutrophil cytoplasmic antibodies (MPO-ANCA) are often present in patients with MPA, patients with ILD and MPO-ANCA positivity but without other manifestations of systemic vasculitis have also been reported. Therefore, the possible association between MPO-ANCA, MPA, and idiopathic ILD remains unclear. This problematic matter has influenced the treatment strategy of MPO-ANCA-positive ILD patients without systemic vasculitis. Clinicians should undertake treatment with careful consideration of the four major causes of death in MPO-ANCA-positive ILD: acute exacerbation of ILD, progressive lung fibrosis, infectious comorbidities, and diffuse alveolar hemorrhage. Further, clinicians need to carefully judge whether inflammation or fibrosis is the dominant condition with reference to the patient's clinical domain and radiopathological lung features. Recently, anti-fibrotic agents such as nintedanib and pirfenidone were shown to be effective in treating various etiologies associated with ILD and have thus led to the widening of treatment options. In this review, the clinical characteristics, radiopathology, prognosis, and therapeutic options in patients with MPO-ANCA-positive ILD are summarized using limited information from previous studies.
Insights
Myeloperoxidase antineutrophil cytoplasmic antibodies (MPO-ANCA) are linked to interstitial lung disease (ILD), but their precise association with microscopic polyangiitis (MPA) is unclear. This review examines MPO-ANCA-positive ILD, focusing on prognosis and treatment strategies.
Area of Science:
- Rheumatology
- Pulmonology
- Immunology
Background:
- Microscopic polyangiitis (MPA) commonly presents with lung lesions, and interstitial lung disease (ILD) is a known poor prognostic factor.
- Myeloperoxidase antineutrophil cytoplasmic antibodies (MPO-ANCA) are frequently observed in MPA patients, but also in patients with ILD without systemic vasculitis, creating diagnostic ambiguity.
Purpose of the Study:
- To clarify the association between MPO-ANCA, MPA, and idiopathic ILD.
- To review the clinical characteristics, radiopathology, prognosis, and therapeutic options for MPO-ANCA-positive ILD patients without systemic vasculitis.
Main Methods:
- Literature review of existing studies on MPO-ANCA-positive ILD.
- Analysis of clinical features, imaging findings, and treatment outcomes.
Main Results:
- The relationship between MPO-ANCA, MPA, and ILD requires further elucidation.
- Treatment decisions for MPO-ANCA-positive ILD without systemic vasculitis must consider risks like acute exacerbation, fibrosis, infection, and hemorrhage.
- Distinguishing between inflammation and fibrosis is crucial for guiding treatment, with anti-fibrotic agents offering new options.
Conclusions:
- MPO-ANCA-positive ILD presents unique challenges in diagnosis and management.
- Careful assessment of clinical and radiopathological features is essential for effective treatment strategies.
- Emerging therapies like nintedanib and pirfenidone show promise for managing ILD in this patient group.
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