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The Diagnostic Value of Alpha-1-Antitrypsin Phenotype in Patients with Granulomatosis with Polyangiitis.

Pervakova MY, Emanuel VL, Titova ON, Lapin SV, Mazurov VI, Belyaeva IB, Chudinov AL, Blinova TV, Surkova EA - Int J Rheumatol (2016)

Bottom Line: Abnormal A1AT variants were found in 18.4% (7/38) of cases: 1 ZZ, 4 MZ, 2 MF, and only 1 MZ in control group (2%).We found that patients with abnormal A1AT phenotypes had significantly higher vasculitis activity (BVAS) as well as anti-PR3 antibodies concentration.We conclude that A1AT deficiency should be considered in all patients with GPA.

View Article: PubMed Central - PubMed

Affiliation: First Pavlov State Medical University of St. Petersburg, Saint Petersburg 197022, Russia.

ABSTRACT
The deficiency of alpha-1 protease inhibitor, or alpha-1-antitrypsin (A1AT), predisposes to chronic lung diseases and extrapulmonary pathology. Besides classical manifestations, such as pulmonary emphysema and liver disease, alpha-1-antitrypsin deficiency (A1ATD) is also known to be associated with granulomatosis with polyangiitis (GPA or Wegener's granulomatosis). The aim of our study was to evaluate the frequency of allelic isoforms of A1AT and their clinical significance among GPA patients. Detailed clinical information, including Birmingham Vasculitis Activity Score (BVAS), incidence of lung involvement, anti-proteinase 3 (PR3) antibodies concentrations, and other laboratory data were collected in 38 GPA patients. We also studied serum samples obtained from 46 healthy donors. In all collected samples A1AT phenotyping by isoelectrofocusing (IEF) and turbidimetric A1AT measurement were performed. Abnormal A1AT variants were found in 18.4% (7/38) of cases: 1 ZZ, 4 MZ, 2 MF, and only 1 MZ in control group (2%). The mean A1AT concentration in samples with atypical A1AT phenotypes was significantly lower (P = 0.0038) than in normal A1AT phenotype. We found that patients with abnormal A1AT phenotypes had significantly higher vasculitis activity (BVAS) as well as anti-PR3 antibodies concentration. We conclude that A1AT deficiency should be considered in all patients with GPA.

No MeSH data available.


Related in: MedlinePlus

A1AT concentration, anti-PR3 antibodies concentration, and GPA activity in patients with normal and abnormal A1AT phenotypes; ∗P < 0.1. NF-patients with normal A1AT phenotype; PF-patients with pathological A1AT phenotype; A1AT: alpha-1-antitrypsin; PR3: Proteinase 3, anti-PR3: anti-proteinase 3 antibodies; GPA: Granulomatosis with polyangiitis; BVAS: Birmingham Vasculitis Activity Score.
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fig3: A1AT concentration, anti-PR3 antibodies concentration, and GPA activity in patients with normal and abnormal A1AT phenotypes; ∗P < 0.1. NF-patients with normal A1AT phenotype; PF-patients with pathological A1AT phenotype; A1AT: alpha-1-antitrypsin; PR3: Proteinase 3, anti-PR3: anti-proteinase 3 antibodies; GPA: Granulomatosis with polyangiitis; BVAS: Birmingham Vasculitis Activity Score.

Mentions: Comparing A1AT phenotypes and clinical data, we found that the mean vasculitis activity, measured with BVAS, in the group with normal phenotypes was 16.42 ± 1.498 that was significantly lower than 24.00 ± 2.828, the mean BVAS score in GPA patients with abnormal A1AT (see Figure 3).


The Diagnostic Value of Alpha-1-Antitrypsin Phenotype in Patients with Granulomatosis with Polyangiitis.

Pervakova MY, Emanuel VL, Titova ON, Lapin SV, Mazurov VI, Belyaeva IB, Chudinov AL, Blinova TV, Surkova EA - Int J Rheumatol (2016)

A1AT concentration, anti-PR3 antibodies concentration, and GPA activity in patients with normal and abnormal A1AT phenotypes; ∗P < 0.1. NF-patients with normal A1AT phenotype; PF-patients with pathological A1AT phenotype; A1AT: alpha-1-antitrypsin; PR3: Proteinase 3, anti-PR3: anti-proteinase 3 antibodies; GPA: Granulomatosis with polyangiitis; BVAS: Birmingham Vasculitis Activity Score.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4835640&req=5

fig3: A1AT concentration, anti-PR3 antibodies concentration, and GPA activity in patients with normal and abnormal A1AT phenotypes; ∗P < 0.1. NF-patients with normal A1AT phenotype; PF-patients with pathological A1AT phenotype; A1AT: alpha-1-antitrypsin; PR3: Proteinase 3, anti-PR3: anti-proteinase 3 antibodies; GPA: Granulomatosis with polyangiitis; BVAS: Birmingham Vasculitis Activity Score.
Mentions: Comparing A1AT phenotypes and clinical data, we found that the mean vasculitis activity, measured with BVAS, in the group with normal phenotypes was 16.42 ± 1.498 that was significantly lower than 24.00 ± 2.828, the mean BVAS score in GPA patients with abnormal A1AT (see Figure 3).

Bottom Line: Abnormal A1AT variants were found in 18.4% (7/38) of cases: 1 ZZ, 4 MZ, 2 MF, and only 1 MZ in control group (2%).We found that patients with abnormal A1AT phenotypes had significantly higher vasculitis activity (BVAS) as well as anti-PR3 antibodies concentration.We conclude that A1AT deficiency should be considered in all patients with GPA.

View Article: PubMed Central - PubMed

Affiliation: First Pavlov State Medical University of St. Petersburg, Saint Petersburg 197022, Russia.

ABSTRACT
The deficiency of alpha-1 protease inhibitor, or alpha-1-antitrypsin (A1AT), predisposes to chronic lung diseases and extrapulmonary pathology. Besides classical manifestations, such as pulmonary emphysema and liver disease, alpha-1-antitrypsin deficiency (A1ATD) is also known to be associated with granulomatosis with polyangiitis (GPA or Wegener's granulomatosis). The aim of our study was to evaluate the frequency of allelic isoforms of A1AT and their clinical significance among GPA patients. Detailed clinical information, including Birmingham Vasculitis Activity Score (BVAS), incidence of lung involvement, anti-proteinase 3 (PR3) antibodies concentrations, and other laboratory data were collected in 38 GPA patients. We also studied serum samples obtained from 46 healthy donors. In all collected samples A1AT phenotyping by isoelectrofocusing (IEF) and turbidimetric A1AT measurement were performed. Abnormal A1AT variants were found in 18.4% (7/38) of cases: 1 ZZ, 4 MZ, 2 MF, and only 1 MZ in control group (2%). The mean A1AT concentration in samples with atypical A1AT phenotypes was significantly lower (P = 0.0038) than in normal A1AT phenotype. We found that patients with abnormal A1AT phenotypes had significantly higher vasculitis activity (BVAS) as well as anti-PR3 antibodies concentration. We conclude that A1AT deficiency should be considered in all patients with GPA.

No MeSH data available.


Related in: MedlinePlus