Research Article

BPI-ANCA and Long-Term Prognosis among 46 Adult CF Patients: A Prospective 10-Year Follow-Up Study

Figure 1

(a) PsA colonization and transplantation free survival. The blue line shows patients who are not chronically colonized with PsA (never had PsA or free from earlier colonization, Leeds class IV or III). The red line shows patients who are chronically or intermittently colonized with PsA (Leeds class I or II) (log rank test ). (b) BPI-ANCA and days of transplantation free survival. The blue line indicates patients without IgA-BPI-ANCA, the dotted line patients with IgA-BPI-ANCA level of 67–200, and the green line patients with a BPI-ANCA above 200 at inclusion. There is a clear difference in transplantation free survival for BPI-negative patients compared to patients with a high BPI-ANCA (log rank test ). (c) Lung function and transplantation free survival. Patients with FEV1 >80% pred, FEV1 between 50 and 80% pred, and FEV1 below 50% pred are indicated by red line, green dotted line, and blue dotted, respectively. None of the patients with a normal lung function at inclusion die or receive a lung transplant during over ten years of followup. The patients who have a low lung function at the start of the study experience a negative outcome to a very high degree (log rank test ).
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