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Interstitial Lung Disease in ANCA-Associated Vasculitis: Pathogenic Considerations and Impact for Patients’ Outcomes

  • Vasculitis (C Dejaco and C Duftner, Section Editors)
  • Published:
Current Rheumatology Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

This review provides an update on recent advances in the diagnosis, pathogenesis, clinical presentation, histopathological findings, and treatment approaches for antineutrophil cytoplasmic antibody (ANCA) vasculitis–associated interstitial lung disease (AAV-ILD) with a focus on literature published in the last 3 years.

Recent Findings

Although there is no validated definition of AAV-ILD, which contributes to some of the heterogeneity seen in study results, there has been an increasing number of publications in recent years on this topic. Most patients with AAV-ILD have MPO-ANCA vasculitis, and this association appears to reduce their 5-year-survival to 60–66% (Sun et al. BMC Pulm Med 21(1), 2021, Maillet et al. J Autoimmun 106, 2020). Median age of diagnosis ranges from mid-60 s to mid-70 s (Ando et al. Respir Med 107(4), 2013), Kagiyama et al. BMJ Open Respir Res 2(1):1–9, 2015, Hozumi et al. Lung 194(2):235–42, 2016, Liu et al. Chest 156(4):715–23, 2019, Maillet et al. J Autoimmun 106, 2020, Wurmann et al. Sarcoidosis Vasc Diffuse Lung Dis 37(1):37–42, 2020, Watanabe et al. BMC Pulm Med 19(1), 2019). Computed tomography (CT) chest imaging for patients with AAV-ILD often shows a usual interstitial pneumonia (UIP) or nonspecific interstitial pneumonia (NSIP) pattern (12–58% and 13–61%, respectively) (Sun et al. BMC Pulm Med 21(1), 2021, Maillet et al. J Autoimmun 106, 2020, Wurmann et al. Sarcoidosis Vasc Diffuse Lung Dis 37(1):37–42, 2020, Watanabe et al. BMC Pulm Med 19(1), 2019, Baqir at al. Sarcoidosis Vasc Diffuse Lung Dis Off J WASOG 36(3):195–201, 2019). Additionally, lung biopsies typically do not demonstrate active inflammation, or capillaritis, questioning whether these patients should be treated with either immunotherapy or anti-fibrotic therapy, or both (Hozumi et al. Lung 194(2):235–42, 2016, Liu et al. Chest 156(4):715–23, 2019, Kitching at al. Nat Rev Dis Prim 6(1):71, 2020, Tanaka et al. Respir Med 106(12):1765–70, 2012).

Summary

Besides immunosuppressive treatments, recent advances in anti-fibrotic therapy may offer patients with progressive AAV-ILD an alternative and/or more effective and individualized treatment option.

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Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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Authors and Affiliations

  1. Division of Respirology, Department of Medicine, University of Toronto, Toronto, Canada

    Kelly Sun & Jolene H. Fisher

  2. Vasculitis Clinic, Division of Rheumatology, Department of Medicine, Mount Sinai Hospital, University of Toronto, 60 Murray Street, Ste 2-220, Box 8, Toronto, ON, M5T 3L9, Canada

    Christian Pagnoux

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Correspondence to Christian Pagnoux.

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Conflict of Interest

Dr. Sun reports grants from the Canadian Pulmonary Fibrosis Foundation. Dr. Fisher reports grants from the Canadian Pulmonary Fibrosis Foundation, and personal fees from Boehringer- Ingelheim and AstraZeneca, outside the submitted work. Dr. Pagnoux reports grants and honoraria (consultancy boards) or speaker fees from Roche, GSK, Pfizer, TEVA, and personal fees (consultancy boards or speaker fees) from InflaRx, Otsuka, Chemo Centryx, AstraZeneca, and Sanofi.

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Sun, K., Fisher, J.H. & Pagnoux, C. Interstitial Lung Disease in ANCA-Associated Vasculitis: Pathogenic Considerations and Impact for Patients’ Outcomes. Curr Rheumatol Rep 24, 259–267 (2022). https://doi.org/10.1007/s11926-022-01078-2

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