ANCA-Associated Vasculitis Prevalence Is Low in AAT-Deficient COPD

Illustration of vasculitis, an inflammation of the blood vessels that may cause thickening, weakening, narrowing, and scarring of the blood vessel walls. This condition occurs if the immune system attacks the blood vessels. This may happen as a result of an infection, medicine, or another disease or condition. The blood vessel on the left shows damage as a result of vasculitis, while the one on the right is normal.
A deficiency in alpha-1-antitrypsin does not appear to increase the risk of vasculitis associated with anti-neutrophil cytoplasmic antibodies in patients with COPD.

A deficiency in alpha-1-antitrypsin (AAT), a serine protease inhibitor encoded by the SERPINA1 gene, does not appear to increase the risk of vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCA) in patients with chronic obstructive pulmonary disease (COPD), according to study findings published in CHEST.

In the study, a cohort of 185 patients with AAT deficiency-related COPD were enrolled from a larger study conducted in France. Indirect immunofluorescence staining of ethanol-fixed human peripheral blood neutrophils was performed to test all sera for ANCAs. Additionally, researchers tested for anti-PR3 and anti-MPO antibodies using enzyme-linked immunosorbent assay. Symptoms suggestive of ANCA-associated vasculitis were examined using a clinical database.

Approximately 87% (n=157) of the patients had the PI*ZZ phenotype and 8% (n=14) had the PI*SZ phenotype. None of the patients tested positive for anti-MPO antibodies, but 1 patient was positive for anti-PR3 antibodies. The patient with a positive anti-PR3 antibody test did not have signs suggestive of ANCA-associated vasculitis, according to medical history and 1-year follow-up data. Based on these data, the prevalence of ANCA-associated vasculitis was 0%, which the investigators suggested was not significantly different from the estimated 0.006% prevalence rate of ANCA-associated vasculitis in the general population.

Study limitations included the small sample size as well as the inclusion of patients from a single restricted geographical location in Europe.

In spite of these limitations, the researchers argued that “because of the low prevalence of ANCAs and ANCA-associated vasculitis symptoms in severe AAT-deficient patients with COPD, systematic screening appears not to be appropriate.”

Disclosure: Several study authors declared affiliations with the pharmaceutical industry. Please see the original reference for a full list of authors’ disclosures.


Deshayes S, Silva NM, Khoy K, et al. Prevalence of anti-neutrophil cytoplasmic antibodies and associated vasculitis in chronic obstructive pulmonary disease associated with alpha-1-antitrypsin deficiency: an ancillary study to a prospective study on 180 French patients [published online May 14, 2020]. CHEST. doi:10.1016/j.chest.2020.04.054