Anti-neutrophil cytoplasmatic antibodies and lung disease in cystic fibrosis.

BACKGROUND: Bactericidal-permeability-increasing protein (BPI) is a potent anti-microbial protein produced by neutrophil granulocytes. Anti-neutrophil cytoplasmatic antibodies (ANCA) directed against BPI have been detected in up to 91% in patients with cystic fibrosis (CF). We aimed to evaluate the prevalence of BPI-ANCA in our CF patients and to determine whether presence of BPI-ANCA is correlated with organ damage. METHODS: Twenty-four patients performed respiratory function testing and pulmonary high-resolution computed tomography (HRCT). HRCT was scored by using a modified Bhalla method. Serum samples were analysed by direct binding enzyme-linked immunosorbent assay for BPI-ANCA. RESULTS: The prevalence of anti-BPI-IgG was 71% and anti-BPI-IgA 33%. Twenty-nine percent of our patients were positive for both BPI-ANCA isotypes. Mean HRCT score was 8.0 ranging from 0 to 22, bronchiectasis presented the most common finding (79%). There was a significant correlation between BPI-ANCA and both HRCT score and FEV(1) (p < 0.01). High levels of BPI-ANCA were correlated to chronic Pseudomonas aeruginosa lung infection (p < 0.01). CONCLUSIONS: BPI-ANCA was common in our study group. Highly significant correlations between BPI-ANCA and parameters to evaluate lung disease in CF may be a consequence of the inflammation process, or it may indicate a pathogenic role of BPI-ANCA levels in the development of lung disease. More research is needed and the clinical significance of our findings needs further evaluation.

High resolution CT in cystic fibrosis--the contribution of expiratory scans.

INTRODUCTION: The use of high-resolution computed tomography (HRCT) is well accepted as an accurate method for evaluation of lung parenchyma in cystic fibrosis (CF). Several scoring methods exist and, in common, all are based on HRCT findings during inspiration alone. OBJECTIVE: To examine whether expiratory HRCT scans could add information about the degree of mosaic perfusion in patients with CF. METHODS AND PATIENTS: Pulmonary HRCT was performed in 17 CF patients (median age of 12 years) with 1-mm thin sections and 10-mm intervals during inspiration, followed by 1-mm thin sections with 20-mm intervals during expiration. HRCT was scored by using a modified Bhalla method. RESULTS: The mean HRCT score was 8.2. Out of 17 patients, 11 (65%) demonstrated a pathological mosaic perfusion in expiration, while only three patients showed mosaic perfusion in inspiration. The degree of expiratory mosaic perfusion was graded as severe in nine patients and moderate in two patients. There was a significant correlation between our modified HRCT score and lung function, as measured by forced expiratory volume in 1 s (FEV1% predicted, P<0.01). CONCLUSION: Mosaic perfusion in expiration was a common pathological HRCT finding in our study group. The clinical significance of this finding needs further evaluation.

Shwachman-Kulczycki score and resting energy expenditure in cystic fibrosis.

BACKGROUND: Disease severity assessed by clinical scores in cystic fibrosis (CF) has been a topic of investigation for many years, although a correlation of clinical scores with resting energy expenditure (REE) has not been described yet. We aimed to assess disease severity as evaluated by the Shwachman-Kulczycki (SK) score and to correlate these findings with REE and forced expiratory volume in 1 s (FEV1). METHODS: Twenty-eight patients performed respiratory function testing (FEV1), and assessment of REE with open circuit indirect calorimetry. The SK score was evaluated according to general activity, physical examination, nutrition and conventional chest X-ray findings. RESULTS: Mean SK score was 75.3 +/- 15.7. Mean REE was 109.1% of predicted vs. 96.5% predicted in 16 healthy subjects (P = 0.002). There was a significant correlation between the SK score and REE (P = 0.001), the SK score and FEV1 (P < 0.001) and REE and FEV1 (P = 0.034). CONCLUSIONS: The correlations between the SK score, REE and FEV1 demonstrate a close connection between disease severity, caloric requirement and lung damage. They confirm the clinical value of the SK score, which is easy to assess in a clinical setting.

Faecal elastase-1 and fat-soluble vitamin profiles in patients with cystic fibrosis in Western Norway.

BACKGROUND: Exocrine pancreatic insufficiency is a major clinical manifestation of cystic fibrosis (CF). Almost nine of ten patients develop signs and symptoms of maldigestion and malabsorption, which often deteriorates nutritional status and therefore worsens the prognosis. Human faecal elastase-1 (FE-1) has shown promising results to assess exocrine pancreatic insufficiency, and this test has been used at Haukeland University Hospital since 1996. AIM OF THE STUDY: To evaluate FE-1 values and fat-soluble vitamin profiles in patients with CF and to correlate exocrine pancreatic function as measured as FE-1 to fat-soluble vitamin profiles. Moreover, we wanted to assess if there are differences between fat-soluble vitamin profiles in patients with impaired versus patent exocrine pancreatic function, and thirdly, if fat-soluble vitamin deficiency at diagnosis is effectively treated by supplementation. METHODS: Consecutive analyses (N = 212) of fat-soluble vitamin profiles and 35 analyses of FE-1 were investigated in 35 patients with CF. In 17 out of 35 patients fat-soluble vitamin profiles were also assessed at diagnosis. Results Mean value of FE-1 for all CF patients was 256.9 microg/g faeces (median 24.1 microg/g faeces). CF patients considered to have maldigestion (N=24) showed a mean value of 19.9 microg/g faeces (median 18.7 microg/g faeces), those without pancreas affection had a mean value of 773.9 microg/g faeces (median 728.9 microg/g faeces, p < 0.01). There was no difference in fat-soluble vitamin profiles among patients with or without exocrine pancreatic insufficiency while on appropriate supplementation. Median value for vitamin E in patients with exocrine pancreatic insufficiency at diagnosis was low (3.6 mg/L). Supplementation of pancreatic enzymes and vitamins normalised profiles in this group at follow-up. There was no significant correlation between exocrine pancreatic function as measured as FE-1 and fat-soluble vitamin profiles, neither in patients with impaired nor in those with patent pancreatic function. CONCLUSIONS: Severe degree of exocrine pancreatic insufficiency is common in patients with cystic fibrosis. There was no correlation of faecal elastase-1 levels to fat-soluble vitamin status. Fat-soluble vitamins (A, D, E) given in appropriate dosages combined with pancreatic enzymes ensured normal profiles in our patients with CF and malabsorption. Officially recommended supplementation of vitamin A and D in Norway during infancy and childhood may explain why so few patients had vitamin deficiencies at diagnosis.

[High-resolution computer tomography in cystic fibrosis]. / Høyoppløsningscomputertomografi av lungene ved cystisk fibrose.

BACKGROUND: Worsening lung disease is the most important factor concerning quality of life and survival rate in the approximately 250 patients with cystic fibrosis in Norway. Pulmonary high-resolution CT has been advocated as a precise diagnostic method, that offers the opportunity to detect slight disease progression. MATERIAL AND METHODS: We examined 21 patients (age 6-34) with high-resolution CT. The findings were scored using a modified Bhalla method, where score 0 is normal and score 27 reflects a maximal degree of e.g. bronchiectasis and mucous plugging. RESULTS: Mean high-resolution CT score was 8.0 (range 0-22). Bronchiectasis was found in 17 out of 21 patients, peribronchial wall thickening in 15 patients and mucous plugging in 14 patients. The Bhalla score showed a close correlation with forced expiratory volume in one second (r = -0.844, p < 0.01). INTERPRETATION: High-resolution CT characterises very well the lung pathology in patients with cystic fibrosis. The degree of pathology assessed with a modified Bhalla score correlates well with forced expiratory volume in one second.

Resting energy expenditure and lung disease in cystic fibrosis.

Optimal nutritional support is considered to be an integral part in the management of cystic fibrosis (CF). Several factors contribute to increased resting energy expenditure (REE), which itself can lead to energy imbalance and thus contribute to deterioration of the nutritional status. We aimed to assess the impact of lung parenchyma damage on REE and correlated these findings with forced expiratory volume in 1 s (FEV(1)). Twenty patients performed respiratory function testing (FEV(1)), pulmonary high-resolution computed tomography (HRCT) and assessment of REE with open circuit indirect calorimetry. HRCT was scored by using a modified Bhalla method. Mean HRCT score was 8.4 and mean REE value was 108.4% predicted vs. 96.5% predicted of 16 healthy subjects (P<0.01). There was a significant correlation between HRCT score and REE (P<0.01), HRCT score and FEV(1) (P<0.001) and REE and FEV(1) (P<0.05). The correlations demonstrate a close correlation between lung damage and elevated REE in people with CF. Prevention of negative energy balance is an important part in follow-up of patients with CF. Any increase in REE should raise suspicion of progress in lung impairment.