Pseudomonas-induced lung damage in cystic fibrosis correlates to bactericidal-permeability increasing protein (BPI)-autoantibodies.

OBJECTIVE: Lung damage is the most common cause of death in cystic fibrosis (CF). It is induced by bacterial colonization and inflammatory activity perpetuates its course. Autoantibodies directed against BPI (bactericidal permeability increasing protein), called BPI-ANCA, have recently been associated with cystic fibrosis. Here we confirm this association and evaluate the relation between ANCA and total IgG level as they relate to bacterial colonization, pulmonary function, and musculoskeletal symptoms. METHODS: BPI-ANCA, MPO-ANCA, and PR3-ANCA were measured with ELISA in 46 adult patients with CF. Total IgG was determined by immunoturbidimetry. Results were correlated to bacterial colonization, lung function and musculoskeletal symptoms. RESULTS: BPI-ANCA was found in 33 patients. In the whole group, both BPI-ANCA and total IgG were inversely correlated to lung function, but in patients chronically colonized with Pseudomonas aeruginosa (P. aeruginosa), BPI-ANCA alone was correlated to lung damage (p = 0.01). Median lung function, measured as forced expiratory volume in 1 second, in P. aeruginosa colonized patients with high levels of BPI-ANCA was 43% of the predicted value. In BPI-ANCA negative, the corresponding figure was 83%. In patients not colonized with P. aeruginosa, this relation was less evident. No correlation between ANCA and musculoskeletal symptoms was seen. CONCLUSION: P. aeruginosa induced lung damage in CF patients is associated with the presence of BPI-ANCA. P. aeruginosa colonized patients without BPI-ANCA have almost normal lung function. We suggest that BPI-ANCA discriminate P. aeruginosa colonized CF patients with severe lung damage from those whose disease is less destructive. Vasculitis like symptoms in CF are not ANCA associated.

Pharmaceutical intervention in the care of cystic fibrosis patients.

In a prospective, randomised, cross-over study including cystic fibrosis patients with indications for HIVAT (home intravenous antibiotic treatment) the prospect of pharmaceutical intervention was investigated. A comparison between the use of disposable infusion devices with antibiotics from the pharmacy and when the patients prepared the drugs themselves was performed. During a first treatment course the patients received either infusion devices during 5 days or reconstituted the drugs themselves during 5 days, or vice versa. During a second treatment course the order was the reversed. Eight patients were included, out of which six completed the original design as a cross-over study, yielding a total of 550 doses of antibiotics. The patients preferred infusion devices from the pharmacy prepared according to GMP (Good Manufacturing Practice) as opposed to reconstituting the antibiotics themselves. Points of view presented included no anxiety over the correct dosage of drugs and less disruption of family and social life. In a practical sense, portable devices are more expensive than the preparation of the drugs by the patients themselves. However, when comparing with in-hospital treatment the direct costs for a hospital stay exceed that of the devices. Another part of the study evaluated the quality of life using a modified form of SEIQoL-DW (Schedule for the Evaluation of Individual Quality of Life - Direct Weighting). Twenty patients took part in the study and the overall quality of life scores increased significantly when patients received infusion devices compared to reconstituting the drugs themselves.