Study objectives: Interstitial inflammation is a major aggravating factor in fibrosing lung disease associated with scleroderma (FASSc) and cryptogenic fibrosing alveolitis (CFA). Exhaled nitric oxide (NO) production is increased in asthma and bronchiectasis and reflects the degree of inflammation. We investigated whether measuring levels of exhaled NO is valuable in assessing disease activity in patients with CFA and patients with FASSc.
Measurements and results: NO levels were measured in 11 patients with CFA (mean age +/- SEM, 58 +/- 12 years old; 5 were male) and 17 patients with FASSc (mean age, 48 +/- 9 years old; 5 were male), and they were compared to BAL cell counts and lung function. Patients with CFA and FASSe had elevated NO levels (11.2 +/-1.0 parts per billion [ppb] and 9.8 +/- 1.0 ppb, respectively; p > 0.05), whereas in a group of 13 nonsmoking normal subjects, the NO levels were not elevated (6.9 +/- 0.5 ppb; p < 0.05). Patients with FASSc (n = 8) who had active BAL (defined as either lymphocytes > 14%, neutrophils > 4%, or eosinophils > 3%) had significantly higher NO levels (13.2 +/- 1.8 ppb), and neutrophil (16.5 +/- 4.0%) and lymphocyte (26.8 +/- 3.4%) BAL cell counts than did patients with FASSc who had inactive BAL (6.7 +/- 1.2 ppb; 1.3 +/- 1.0% and 7.5 +/- 1.3%, respectively; p < 0.05). There was a significant correlation between exhaled NO and lymphocyte cell count in patients with FASSc (r = 0.58; p < 0.05). All patients with CFA had active BAL; however, those treated with corticosteroids (12.9 +/- 1.0% ppb, p < 0.05) had lower NO levels (9.0 +/- 1 ppb) and higher BAL lymphocyte cell couits (16.6 +/- 2.0%) than did those not treated with corticosteroids (7.2 +/- 1.7%; p < 0.05).
Conclusions: We conclude that exhaled NO may be a useful addition to BAL cell counts in disease monitoring.