Background Respiratory failure is a life-threatening and unpredictable complication of systemic sclerosis (SSc). A study was undertaken to assess the value of alveolar nitric oxide (NO) in predicting the risk of lung function deterioration leading to respiratory failure or death in patients with SSc.
Methods 105 patients with SSc were enrolled in this prospective cohort and were followed longitudinally over a 3-year period during which the risk of occurrence of deleterious events was analysed according to alveolar concentration (CAno), conducting airway output (J′awno) and fractional concentration (FEno0.05) of exhaled NO measured at inclusion. Comparison was made between each NO parameter to predict the occurrence of deleterious events, defined as a 10% decrease in total lung capacity or forced vital capacity from baseline, or death.
Results The area under the receiver operating characteristic curve of CAno to predict the occurrence of the combined events was 0.84 (95% CI 0.76 to 0.92; p<0.001), which was significantly higher than those of J′awno and FEno0.05 (p<0.001). A cut-off of CAno of 5.3 ppb had a sensitivity of 88% and a specificity of 62% for the prediction of the occurrence of combined events during follow-up, and was validated in an independent cohort of patients with SSc. Combined events occurred more frequently in patients whose CAno was >5.3 ppb. The adjusted HR for patients with CAno >5.3 ppb was 6.06 (95% CI 2.36 to 15.53; p<0.001). CAno accurately predicted the occurrence of combined events irrespective of forced vital capacity values or the presence of interstitial lung disease at baseline.
Conclusions Increased CAno accurately identifies patients with SSc with a high risk of developing lung function deterioration and may help to initiate early appropriate treatment.
- Alveolar concentration of nitric oxide
- systemic sclerosis
- interstitial lung disease
- exhaled nitric oxide
- prognostic factor
- exhaled airway markers
- interstitial fibrosis
- lymphocyte biology
- COPD epidemiology
- interstitial fibrosis
- sleep apnoea
- systemic disease and lungs
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Competing interests None.
Patient consent Obtained.
Ethics approval Ethics approval was provided by CPP Ile de France.
Provenance and peer review Not commissioned; externally peer reviewed.
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