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Myeloperoxidase-Antibody Positivity and Progression to Microscopic Polyangiitis in Interstitial Lung Disease: A Case
Michael Hoskins1,2, Grace Thompson3,4, Vidya Navaratnam1,2
1Respiratory Department Sir Charles Gairdner Hospital Perth Australia.
Abstract:
Microscopic polyangiitis (MPA) is associated with myeloperoxidase-antibody positivity and pulmonary complications. However, these antibodies may be found incidentally in some people with interstitial lung disease (ILD). As a result, management of people with ILD and myeloperoxidase-antibody positivity remains unclear. Our case series describes the natural history of this group of patients over a mean follow-up period of 4 years. Using data from a public sector pathology provider in Western Australia, we identified 17 people with a positive myeloperoxidase-antibody titre and who attended our tertiary Respiratory outpatient clinic. Eventual or concurrent diagnosis of MPA was more common in those with radiological usual interstitial pneumonia pattern, higher baseline and peak myeloperoxidase-antibody titres, lower gas transfer capacity and lower distances achieved on 6-min walk testing. These features may be used by clinicians during multidisciplinary team discussion to identify those at high-risk of developing MPA.
Insights
Microscopic polyangiitis (MPA) can be associated with myeloperoxidase-antibody positivity in interstitial lung disease (ILD). Certain features like radiological patterns and antibody titres help identify patients at higher risk of developing MPA.
Area of Science:
- Rheumatology
- Pulmonology
- Immunology
Background:
- Microscopic polyangiitis (MPA) is linked to myeloperoxidase-antibody (MPO-ANCA) positivity and lung issues.
- MPO-ANCA positivity can occur incidentally in interstitial lung disease (ILD) patients, complicating management.
- The natural history and risk factors for MPA in ILD patients with MPO-ANCA positivity are not well-defined.
Purpose of the Study:
- To describe the natural history of patients with ILD and MPO-ANCA positivity.
- To identify clinical and radiological features associated with MPA development in this cohort.
Main Methods:
- Retrospective case series analysis.
- Inclusion criteria: positive MPO-ANCA titre and attendance at a tertiary Respiratory outpatient clinic.
- Data collected from a public sector pathology provider in Western Australia.
Main Results:
- A cohort of 17 patients with ILD and MPO-ANCA positivity was studied over a mean 4-year follow-up.
- MPA diagnosis (eventual or concurrent) was more frequent in patients with a usual interstitial pneumonia (UIP) radiological pattern.
- Higher baseline and peak MPO-ANCA titres, reduced gas transfer capacity, and shorter 6-minute walk test distances were associated with MPA development.
Conclusions:
- Specific clinical and radiological features can help identify ILD patients with MPO-ANCA positivity who are at higher risk of developing MPA.
- These identified features can aid clinicians in multidisciplinary team discussions for risk stratification.
- Further research may refine diagnostic and management strategies for this specific patient group.
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