Objective: Bronchoalveolar lavage (BAL) is a procedure for sampling the terminal airspace cell population to diagnose alveolitis, a condition that predicts changes in lung function in scleroderma patients. Cyclophosphamide (CYC) stabilizes the progression of lung disease in some, but not all, patients with active alveolitis. However, it is unknown whether the BAL fluid cell count obtained after CYC treatment of alveolitis predicts long-term lung function outcomes and can therefore be used to assist in therapeutic decision-making. The purpose of this study was to determine whether CYC therapy for active lung disease alters BAL fluid neutrophil and eosinophil counts and whether the persistence of abnormal BAL findings after CYC therapy predicts a decline in lung function in patients with scleroderma and interstitial lung disease (ILD).
Methods: We systematically reviewed the records of scleroderma patients who had active ILD, as evidenced by neutrophilia or eosinophilia on BAL fluid analysis before CYC therapy and on repeat analysis after completion of CYC. Pulmonary function tests (PFTs) were performed before initiation of therapy, at completion of therapy, and during long-term followup (mean +/- SD 3.6 +/- 1.94 years).
Results: Of the 25 study patients, in only 6 did the BAL fluid cell counts normalize after CYC therapy. No significant differences were observed between the proportions of patients who had a > or =10% decline in the % predicted DLCO or FVC on the second set of PFTs and had abnormal findings on followup BAL fluid analysis. During long-term followup, patients with persistent alveolitis had a decline in lung function (mean +/- SD change in % predicted FVC -0.6 +/- 10.8 liters and mean +/- SD change in % predicted DLCO -4.7 +/- 21.43 ml/minute/mm Hg), but their lung function did not significantly differ from that in patients whose BAL fluid cell counts had normalized (P = 0.70 and P = 0.62, respectively).
Conclusion: Persistently abnormal results on BAL fluid analysis following CYC treatment is a common finding and does not predict a subsequent decline in lung function.