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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
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Interstital lung disease in ANCA vasculitis
Marco A Alba1, Luis Felipe Flores-Suárez2, Ashley G Henderson3
1Department of Pathology and Laboratory Medicine, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.
Autoimmunity Reviews
|May 9, 2017
Summary
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides can involve the lungs, often with interstitial lung disease (ILD) and pulmonary fibrosis. This condition negatively impacts patient prognosis.
Area of Science:
- Rheumatology
- Pulmonology
- Immunology
Background:
- Anti-neutrophil cytoplasmic antibodies (ANCA) vasculitides are systemic autoimmune diseases affecting small blood vessels.
- Lung involvement is a significant complication, increasing morbidity and mortality in conditions like microscopic polyangiitis and granulomatosis with polyangiitis.
- Recent reports highlight a link between interstitial lung disease (ILD) and ANCA vasculitis, particularly myeloperoxidase-ANCA positive cases.
Purpose of the Study:
- To review the clinical and radiologic features of pulmonary fibrosis in ANCA vasculitis.
- To summarize histopathology, prognosis, and therapeutic strategies for this condition.
Main Methods:
- Literature review of retrospective series and case reports.
- Focus on studies describing the association between ILD and ANCA vasculitis.
- Synthesis of information on clinical presentation, imaging, pathology, and treatment.
Main Results:
- Pulmonary fibrosis often co-occurs with or precedes ANCA vasculitis diagnosis.
- Myeloperoxidase-ANCA positivity is frequently observed in patients with ILD and vasculitis.
- ILD is associated with a poorer long-term prognosis for ANCA vasculitis patients.
Conclusions:
- Pulmonary fibrosis is an important manifestation of ANCA vasculitis with significant prognostic implications.
- Understanding the interplay between ILD and ANCA vasculitis is crucial for patient management.
- Further research into therapeutic options is warranted.
