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Background Hemoptysis is a major cause of morbidity and mortality in patients with cystic fibrosis (CF). Antifibrinolytic agents have shown efficacy in a broad range of bleeding disorders and conditions. Objectives The goal of this study was to examine the use of antifibrinolytic agents in managing hemoptysis in CF. We developed a clinical treatment pathway for inpatient and outpatient use, and rates of admission for bleeding prior to and following implementation of the pathway are reported. Methods All adult patients with CF treated with systemic antifibrinolytic agents over a 54-month period according to the treatment pathway were analyzed. Data collected included demographic characteristics, baseline CF-related characteristics, and bleeding and treatment parameters. Effectiveness of the pathway was evaluated via comparison of annualized hemoptysis admission rates prior to and following pathway enrollment. Results Seventy-two distinct episodes of hemoptysis treated with antifibrinolytic agents were analyzed in a total of 21 adult patients with CF. Two-thirds of episodes treated involved moderate or massive hemoptysis. Bleeding ceased following a median of 2 days. Outpatient treatment was associated with a 50% reduction in the annualized hemoptysis admission rate following pathway enrollment (2.44 vs 1.23 admissions per year; P = .0024) that was independent of other changes in management. Antifibrinolytic therapy was well tolerated. One central catheter-associated upper extremity DVT was observed in a patient with previous thrombosis in the same vessel. Conclusions A pathway using systemic antifibrinolytic therapy to treat hemoptysis in patients with CF was associated with a reduction in hospital admissions. No serious adverse events were observed. Additional studies are needed to further define the benefits of systemic antifibrinolytic use in patients with CF.

Study objectives: To determine levels of the vascular endothelial growth factor (VEGF) isoform consisting of 165 amino acids (VEGF165) in BAL fluid (BALF) from lung transplant recipients (LTXs). Design: Bronchoscopy with BAL was performed on LTXs and normal volunteers (NVs). Setting: University hospital. Participants: LTXs (n 5 57) and NVs (n 5 15). Measurements and result: VEGF165 concentrations in BALF were higher (mean 6 SEM, 240 6 32 pg/mL) for NVs (n 5 15) vs 133 6 14 pg/mL for LTXs (n 5 37) who were stable without evidence of significant rejection or infection at 6 months after transplantation (p < 0.0001). BALF VEGF concentrations sampled at 24 to 48 h, 2 weeks, 4 weeks, and 6 months after transplantation for 11 LTXs who lacked rejection or infection at any time point were 71 6 8 pg/mL, 80 6 20 pg/mL, 82 6 13 pg/mL, and 167 6 31 pg/mL, respectively. VEGF concentrations in BALF for LTXs with cytomegalovirus (CMV) pneumonia were 55 6 12 pg/mL (n 5 10), 117 6 33 pg/mL for grade A3 acute rejection (n 5 9), and 82 6 17 pg/mL (n 5 14) for active bronchiolitis obliterans syndrome (BOS). Concentrations of VEGF in BALF at 6 months for the 32 stable recipients with bilateral lung transplantation were significantly higher for those with higher values for FEV1, and BALF VEGF concentrations were significantly lower in BALF at 6 months for those recipients who subsequently went on to develop BOS (86 6 19 pg/mL) vs those who did not (158 6 18 pg/mL; p 5 0.03). Serum concentrations of VEGF did not correlate with VEGF concentrations in BALF, but serum VEGF was 291 6 62 pg/mL at 10 to 14 days after transplantation vs 130 6 20 pg/mL at 4 weeks for nine LTXs with paired samples (p < 0.02). Serum VEGF concentrations for NVs (n 5 15) were 102 6 15 pg/mL vs 94 6 17 for stable LTXs (n 5 12) at 24 weeks after transplantation and 123 6 33 pg/mL for LTXs with active BOS (n 5 10). Conclusions: BALF VEGF concentrations are particularly depressed at early time points following lung transplantation, gradually improve in the absence of significant rejection or infection, and are lower with active rejection or CMV pneumonia. (CHEST 2001; 119:137‐143)