[PDF][PDF] Prevalence of anti-neutrophil cytoplasmic antibodies and associated vasculitis in COPD associated with alpha-1 antitrypsin deficiency: an ancillary study to a …
S Deshayes, NM Silva, K Khoy, D Mariotte, B Le Mauff… - Chest, 2020 - Elsevier
S Deshayes, NM Silva, K Khoy, D Mariotte, B Le Mauff, JF Mornex, C Pison, A Cuvelier…
Chest, 2020•ElsevierMethods This was an ancillary study from the French cohort of AAT deficiency-related COPD
(CONEDAT 9), the protocol of which has already been published. 10 Briefly, CONEDAT is a
prospective cohort aiming to include all French patients aged≥ 18 with pulmonary
emphysema confirmed by CT scan associated with an obstructive ventilatory disorder and a
serum AAT level below 11 μM or a PI∗ ZZ phenotype. Clinical data were prospectively
collected every 6 months during a 5-year period. All patients gave their Results Among the …
(CONEDAT 9), the protocol of which has already been published. 10 Briefly, CONEDAT is a
prospective cohort aiming to include all French patients aged≥ 18 with pulmonary
emphysema confirmed by CT scan associated with an obstructive ventilatory disorder and a
serum AAT level below 11 μM or a PI∗ ZZ phenotype. Clinical data were prospectively
collected every 6 months during a 5-year period. All patients gave their Results Among the …
Methods
This was an ancillary study from the French cohort of AAT deficiency-related COPD (CONEDAT 9), the protocol of which has already been published. 10 Briefly, CONEDAT is a prospective cohort aiming to include all French patients aged≥ 18 with pulmonary emphysema confirmed by CT scan associated with an obstructive ventilatory disorder and a serum AAT level below 11 μM or a PI∗ ZZ phenotype. Clinical data were prospectively collected every 6 months during a 5-year period. All patients gave their
Results
Among the 416 patients included in the CONEDAT study, sera were available for this ancillary study only for 185 AAT-deficient patients with COPD; these sera were tested for ANCAs and anti-PR3 and anti-MPO antibodies. Five patients were excluded because genotyping had not been performed. The characteristics of the population are detailed in Table 1. Among the patients, 157 (87%) had the PI∗ ZZ phenotype and 14 (8%) had the PI∗ SZ phenotype. No patient had been diagnosed with ANCA-associated
Discussion
A cohort of 180 patients with AAT deficiency-related COPD did not exhibit an increased prevalence of ANCAs, anti-PR3 antibodies, anti-MPO antibodies, or ANCA-associated vasculitis. Therefore, although AAT deficiency is statistically overrepresented among patients with ANCA-associated vasculitis, this deficiency is likely to be involved, but not as a sufficient or necessary factor, in its pathophysiology. Using previous studies that assessed the prevalence of these different conditions, the null
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