Research Article
The Diagnostic Value of Alpha-1-Antitrypsin Phenotype in Patients with Granulomatosis with Polyangiitis
Table 1
The description of clinical data of GPA patients with pathological A1AT phenotypes.
| | Number | 1 | 2 | 3 | 4 | 5 | 6 | 7 |
| | Gender | Male | Male | Male | Female | Female | Female | Female |
| | Age | 31 | 19 | 49 | 62 | 52 | 58 | 67 |
| | Disease duration (years) | 8 | 1 | 3 | 1 | 1 | 1 | 8 |
| | Lung involvement type | Cavitating infiltrates | Cavitating infiltrates | Interstitial fibrosis | Cavitating infiltrates | Cavitating infiltrates | Cavitating infiltrates | Interstitial fibrosis |
| | BVAS activity | 28 | 26 | 14 | 20 | 28 | 38 | 14 |
| | A1AT phenotype | MZ | ZZ | MZ | MF | MZ | MF | MZ |
| | A1AT concentration (mg/L) | 1041 | 345 | 1033 | 1057 | 430 | 1576 | 1308 |
| | Anti-PR3-antibodies concentration (RU/mL) |
25.0 |
260.5 |
135.1 |
116.0 |
266.0 |
330.9 |
129.4 |
| | ESR (mm/hr) | 54 | 57 | 46 | 54 | 63 | 60 | 55 |
|
|
Note:
A1AT reference values: 900–2000 mg/L.
Anti-PR3 antibodies reference values: <20 RU/mL.
GPA, granulomatosis with polyangiitis; BVAS, Birmingham Vasculitis Activity Score; A1AT, alpha-1-antitrypsin; PR3, proteinase 3; ESR, erythrocyte sedimentation rate.
|
