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… Show more
“…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.…”
Background: Although transbronchial lung cryobiopsy (TBLC) is widely used in diagnostic algorithms for various interstitial lung diseases (ILD), its real-world utility in the therapeutic decision-making strategy for ILD patients remains unclear.
Methods: We analyzed medical records of 40 consecutive patients with idiopathic or fibrotic hypersensitivity pneumonitis who underwent TBLC. A TBLC-based usual interstitial pneumonia (UIP) score was used to assess three morphologic descriptors: i) patchy fibrosis, ⅱ) fibroblastic foci, and ⅲ) honeycombing.
Results: In our 40 patients with ILD, the most frequent radiological feature was indeterminate for UIP (45.0%). Final diagnosis included idiopathic pulmonary fibrosis (22.5%), fibrotic nonspecific interstitial pneumonia (5.0%), fibrotic hypersensitivity pneumonitis (35.0%), and unclassifiable ILD (37.5%). Linear mixed-effects analysis showed that declines in the slopes of %FVC and %DLCO in patients with TBLC-based UIP ‘Score ≥2’ were significantly steeper than those of patients with ‘Score ≤1’. During follow-up of patients with ‘Score ≥2’ (n = 24), more than half of them received combination therapy (i.e., anti-inflammatory and anti-fibrotic agents). Moreover, in the groups receiving an anti-fibrotic agent, many patients received anti-fibrotic agent administration within 6 months after the TBLC procedure. Among the groups with combination therapy, most patients received an anti-fibrotic agent within 6 months of the multi-disciplinary discussion-based diagnosis.
Conclusions: TBLC-based UIP score ≥2 indicated the increased possibility of a progressive fibrosis course that may prove helpful in predicting progressive pulmonary fibrosis/progressive fibrosing ILD even if disease is temporarily stabilized due to anti-inflammatory agents. Patients may benefit from early introduction of anti-fibrotic agents by treating clinicians.
“…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.…”
Background: Although transbronchial lung cryobiopsy (TBLC) is widely used in diagnostic algorithms for various interstitial lung diseases (ILD), its real-world utility in the therapeutic decision-making strategy for ILD patients remains unclear.
Methods: We analyzed medical records of 40 consecutive patients with idiopathic or fibrotic hypersensitivity pneumonitis who underwent TBLC. A TBLC-based usual interstitial pneumonia (UIP) score was used to assess three morphologic descriptors: i) patchy fibrosis, ⅱ) fibroblastic foci, and ⅲ) honeycombing.
Results: In our 40 patients with ILD, the most frequent radiological feature was indeterminate for UIP (45.0%). Final diagnosis included idiopathic pulmonary fibrosis (22.5%), fibrotic nonspecific interstitial pneumonia (5.0%), fibrotic hypersensitivity pneumonitis (35.0%), and unclassifiable ILD (37.5%). Linear mixed-effects analysis showed that declines in the slopes of %FVC and %DLCO in patients with TBLC-based UIP ‘Score ≥2’ were significantly steeper than those of patients with ‘Score ≤1’. During follow-up of patients with ‘Score ≥2’ (n = 24), more than half of them received combination therapy (i.e., anti-inflammatory and anti-fibrotic agents). Moreover, in the groups receiving an anti-fibrotic agent, many patients received anti-fibrotic agent administration within 6 months after the TBLC procedure. Among the groups with combination therapy, most patients received an anti-fibrotic agent within 6 months of the multi-disciplinary discussion-based diagnosis.
Conclusions: TBLC-based UIP score ≥2 indicated the increased possibility of a progressive fibrosis course that may prove helpful in predicting progressive pulmonary fibrosis/progressive fibrosing ILD even if disease is temporarily stabilized due to anti-inflammatory agents. Patients may benefit from early introduction of anti-fibrotic agents by treating clinicians.
“…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 ]. Fig.…”
“…Antifibrotic treatment may be considered a therapeutic option for patients with ANCAassociated interstitial lung disease [141]. Although no published studies have specifically evaluated the role of antifibrotic agents in AAV, the French Vasculitis Study Group is currently conducting an open-label study to assess the safety and effectiveness of pirfenidone in patients with anti-MPO positivity and pulmonary fibrosis with or without AAV (NCT03385668).…”
Section: Treatment Of Pulmonary Manifestations In Aavmentioning
Pulmonary manifestations of vasculitis are associated with significant morbidity and mortality in affected individuals. They result from a complex interplay between immune dysregulation, which leads to vascular inflammation and tissue damage. This review explored the underlying pathogenesis of pulmonary involvement in vasculitis, encompassing various forms such as granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), and anti-GBM disease. Mechanisms involving ANCA and anti-GBM autoantibodies, neutrophil activation, and neutrophil extracellular trap (NETs) formation are discussed, along with the role of the complement system in inducing pulmonary injury. Furthermore, the impact of genetic predisposition and environmental factors on disease susceptibility and severity was considered, and the current treatment options were presented. Understanding the mechanisms involved in the pathogenesis of pulmonary vasculitis is crucial for developing targeted therapies and improving clinical outcomes in affected individuals.
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