PMID- 27127514
OWN - NLM
STAT- PubMed-not-MEDLINE
DCOM- 20160429
LR  - 20201001
IS  - 1687-9260 (Print)
IS  - 1687-9279 (Electronic)
IS  - 1687-9260 (Linking)
VI  - 2016
DP  - 2016
TI  - The Diagnostic Value of Alpha-1-Antitrypsin Phenotype in Patients with 
      Granulomatosis with Polyangiitis.
PG  - 7831410
LID - 10.1155/2016/7831410 [doi]
LID - 7831410
AB  - 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.
FAU - Pervakova, M Y
AU  - Pervakova MY
AUID- ORCID: 0000-0001-9630-257X
AD  - First Pavlov State Medical University of St. Petersburg, Saint Petersburg 197022, 
      Russia.
FAU - Emanuel, V L
AU  - Emanuel VL
AUID- ORCID: 0000-0002-2079-0439
AD  - First Pavlov State Medical University of St. Petersburg, Saint Petersburg 197022, 
      Russia.
FAU - Titova, O N
AU  - Titova ON
AUID- ORCID: 0000-0003-4678-3904
AD  - First Pavlov State Medical University of St. Petersburg, Saint Petersburg 197022, 
      Russia.
FAU - Lapin, S V
AU  - Lapin SV
AUID- ORCID: 0000-0002-4998-3699
AD  - First Pavlov State Medical University of St. Petersburg, Saint Petersburg 197022, 
      Russia.
FAU - Mazurov, V I
AU  - Mazurov VI
AUID- ORCID: 0000-0001-8367-3144
AD  - North-Western Mechnikov State Medical University, Saint Petersburg 191015, 
      Russia.
FAU - Belyaeva, I B
AU  - Belyaeva IB
AUID- ORCID: 0000-0002-7981-6349
AD  - North-Western Mechnikov State Medical University, Saint Petersburg 191015, 
      Russia.
FAU - Chudinov, A L
AU  - Chudinov AL
AUID- ORCID: 0000-0002-7675-5683
AD  - North-Western Mechnikov State Medical University, Saint Petersburg 191015, 
      Russia.
FAU - Blinova, T V
AU  - Blinova TV
AUID- ORCID: 0000-0003-4896-3319
AD  - First Pavlov State Medical University of St. Petersburg, Saint Petersburg 197022, 
      Russia.
FAU - Surkova, E A
AU  - Surkova EA
AUID- ORCID: 0000-0001-5191-0221
AD  - First Pavlov State Medical University of St. Petersburg, Saint Petersburg 197022, 
      Russia.
LA  - eng
PT  - Journal Article
DEP - 20160410
PL  - United States
TA  - Int J Rheumatol
JT  - International journal of rheumatology
JID - 101519204
PMC - PMC4835640
EDAT- 2016/04/30 06:00
MHDA- 2016/04/30 06:01
PMCR- 2016/04/10
CRDT- 2016/04/30 06:00
PHST- 2016/01/28 00:00 [received]
PHST- 2016/03/09 00:00 [revised]
PHST- 2016/03/27 00:00 [accepted]
PHST- 2016/04/30 06:00 [entrez]
PHST- 2016/04/30 06:00 [pubmed]
PHST- 2016/04/30 06:01 [medline]
PHST- 2016/04/10 00:00 [pmc-release]
AID - 10.1155/2016/7831410 [doi]
PST - ppublish
SO  - Int J Rheumatol. 2016;2016:7831410. doi: 10.1155/2016/7831410. Epub 2016 Apr 10.