Research Article
BPI-ANCA Provides Additional Clinical Information to Anti-Pseudomonas Serology: Results from a Cohort of 117 Swedish Cystic Fibrosis Patients
| | Chronic PsA Leeds 1 | Intermittent PsA Leeds 2 | Free from PsA Leeds 3 | Never had PsA Leeds 4 | Total |
| Alkaline protease Median (IQR) ELISA units | 2056 (596–3372) | 327 (130–661) | 325 (162–593) | 194 (46–381) | 450 (167–1299) |
| Elastase Median (IQR) ELISA units | 1696 (439–5660) | 421 (170–2205) | 284 (197–383) | 239 (152–383) | 389 (217–1750) |
| Exotoxin A Median (IQR) ELISA units | 1985 (890–3925) | 451 (99–839) | 224 (56–517) | 117 (0–225) | 358 (103–1410) |
| BPI-ANCA IgA Median (IQR) ELISA units | 93 (28–2225) | 8 (4–22) | 7 (1–18) | 8 (1–30) | 21 (5–89) |
| Change to Leeds 1 within 3 years | na | 3 | 3 | 6 | 12 |
| Lung transplantation or death within 10 years (%) | 20 (33%) | 1 (9%) | 1 (6%) | 3 (7%)1 | 25 (18%) |
| Lung function at 10 years follow-up Median (IQR) FEV1% pred. | 61,5% (50–76) | 86% (76–101) | 69% (47–83) | 88% (71–101) | 76% (60–94) |
|
|
1One of these patients died due to a non-CF related cause.
|