Anti-Inflammatory and/or Anti-Fibrotic Treatment of MPO-ANCA-Positive Interstitial Lung Disease: A Short Review

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.

Usefulness of transbronchial lung cryobiopsy when choosing treatment for chronic fibrosing interstitial lung disease: Descriptive research

“…Thus, if we could intervene more quickly by administering an anti-brotic agent early to patients with worsened pulmonary function, the patients with PF-ILD or PPF in the present study who underwent TBLC might have bene tted from early introduction of an anti-brotic agent. Expansion of brotic change in patients with both a radiological and pathological UIP pattern can lead to a high risk of PPF or PF-ILD, which means a poorer prognosis for any of the ILDs [5,23,24]. Therefore, the descriptive research undertaken in the present study suggested that additional TBLC pathological information can contribute to early medical intervention particular in terms of anti-brotic agent administration in clinical practice, although a comparative study to assess this might be di cult.…”

Presentation and progression of MPO-ANCA interstitial lung disease

“…Avacopan might hold promise if MPO-ANCA ILD is complement-mediated. Empirical management approach to patients with MPO-ANCA ILD has been proposed [ 8 , 77 , 78 ] and there are no guidelines, apart from patients with overt MPA [ 79 ].

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Usefulness of Transbronchial Lung Cryobiopsy When Starting Antifibrotic Treatment and Predicting Progressive Fibrosing Interstitial Lung Disease: Descriptive Research

“…Thus, if we could intervene more quickly by administering an antifibrotic agent early to patients with worsened pulmonary function, the patients with PF‐ILD or PPF in the present study who underwent TBLC might have benefitted from early introduction of an antifibrotic agent. Expansion of fibrotic change in patients with both a radiological and pathological UIP pattern can lead to a high risk of PPF or PF‐ILD, which means a poorer prognosis for any of the ILDs [ 5 , 24 , 25 ]. Therefore, the descriptive research undertaken in the present study suggested that additional pathological information obtained from TBLC can contribute to early medical intervention particular in terms of antifibrotic agent administration in clinical practice, although a comparative study to assess this might be difficult.…”