Exhaled nitric oxide is elevated in patients with progressive systemic sclerosis without interstitial lung disease

Abstract

Background: Progressive systemic sclerosis (PSS) is a multisystem disorder of unknown etiology. Interstitial lung disease (ILD) is a major cause of mortality in this condition, and a major challenge in this regard is to identify parameters that would predict the onset or progression of ILD in patients with PSS. Abnormal cellularity of BAL fluid (BALF) has been demonstrated in patients with PSS without ILD.

Study objectives: We investigated exhaled nitric oxide (NO) as a noninvasive marker of pulmonary inflammation in patients with PSS with and without clinical and radiologic evidence of ILD. This was compared with the cellularity of BALF. Our hypothesis was that exhaled NO was elevated in patients with PSS without ILD who had abnormal BALF cellularity.

Setting: Pulmonology and rheumatology units of a university-based, tertiary referral hospital in Durban, South AFRICA:

Study methods: Exhaled NO was measured using a chemiluminescence analyzer in 12 patients with PSS and ILD and in 12 patients without clinical or radiologic evidence of ILD and in 30 healthy control subjects. BAL was performed in patients with PSS with and without the presence of ILD and in six healthy control subjects.

Results: Subclinical inflammation was confirmed by increased inflammatory cell counts in BALF from patients with PSS without ILD. Exhaled NO (mean [SEM]) was elevated in patients with PSS without ILD at 9.6 (0.7) parts per billion (ppb) compared to patients with PSS and ILD at 6.2 (0.6) ppb (p < 0.001) and healthy control subjects at 6.3 (0.2) ppb (p < 0.001).

Conclusion: Exhaled NO may therefore be an important noninvasive surrogate marker of inflammation in patients with PSS without ILD.