Ogawa A, et al. – The physicians aimed to assess the efficacy and safety of long–term imatinib therapy for patients with pulmonary veno–occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH). They observed that imatinib improved exercise capacity, hemodynamics and survival in patients with PVOD/PCH. They suggested that in patients with PVOD/PCH, who have no effective medical therapy available, imatinib might function as a bridge to lung transplantation, and may become a potential therapeutic option to improve their survival.

Methods

• Nine patients with PVOD/PCH received imatinib in the present observational study.
• After that, clinical information including exercise capacity and hemodynamics at baseline and at follow-up were compared.
• Finally, survival rate of patients treated with imatinib was compared to those of seven patients who did not treated with imatinib.

Results

• The present study showed that imatinib was prescribed at doses of 100–400 mg/day and was well-tolerated.
• World Health Organization functional class and brain natriuretic peptide levels significantly improved at follow-up.
• It was observed in the findings that mean pulmonary arterial pressure was significantly lessened (from 56.8 ± 8.3 to 43.7 ± 9.0 mmHg) with preserved cardiac index.
• It was noted that patients were treated with imatinib for 797.2 ± 487.0 days.
• 7 patients (77.8%) died and two patients (22.2%) underwent lung transplantation.
• The findings demonstrated that mean survival time in patients treated with imatinib therapy was 1493.7 ± 196.3 days (95% confidence interval, 1108.9–1878.5 days), significantly longer than those without imatinib treatment (713.0 ± 258.1 days, log-rank test, P = 0.04).