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.
|
