Efficacy and safety of long-term imatinib therapy for patients with pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis

Ogawa, Aiko; Miyaji, Kohei; Matsubara, Hiromi

doi:10.1016/j.rmed.2017.08.032

Cited by 22 publications

(14 citation statements)

References 20 publications

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“…The authors did not observe the development of subdural haematomas, but two patients in the imatinib-treated group died of infective complications. Nevertheless, median survival in the group treated with imatinib was over 4 years, which is unusual for PVOD/PCH patients [49].…”

Section: Therapy and Prognosismentioning

confidence: 93%

“…Imatinib resulted in the significant improvement of the World Health Organisation (WHO) functional class and serum BNP concentration, as well as in the decrease of mean pulmonary artery pressure and prolonged survival [49]. The authors did not observe the development of subdural haematomas, but two patients in the imatinib-treated group died of infective complications.…”

Section: Therapy and Prognosismentioning

confidence: 97%

“…Recently, Ogava et al [49] reported on imatinib as add-on therapy, at a dose of 100-400 mg/ day, in 9 PVOD/PCH patients, and compared the results with 7 PVOD/PCH patients who did not receive the drug [49]. Imatinib resulted in the significant improvement of the World Health Organisation (WHO) functional class and serum BNP concentration, as well as in the decrease of mean pulmonary artery pressure and prolonged survival [49].…”

Section: Therapy and Prognosismentioning

confidence: 99%

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Pulmonary Veno-Occlusive Disease: Pathogenesis, Risk Factors, Clinical Features and Diagnostic Algorithm—State of the Art

Szturmowicz¹,

Kacprzak²,

Szołkowska³

et al. 2018

Advances in Respiratory Medicine

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Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary haemangiomatosis (PCH) are rare disorders, with the estimated prevalence of less than 1 case per million inhabitants. The vascular pathology in PVOD/PCH involves pre-septal and septal veins, alveolar capillaries and small pulmonary arteries. According to the ERS/ESC classification of pulmonary hypertension (PH) from 2015, PVOD/PCH have been included in the subgroup 1' of pulmonary arterial hypertension (PAH). Recent data indicate, however, the possibility of PVOD/PCH pathology in the patients diagnosed in the group 1. The problem may concern PAH associated with scleroderma, drug-induced PAH, PAH due to HIV infection and up to 10% of patients with idiopathic PAH (IPAH). Recently, bi-allelic EIF2AK4 mutations were found in the cases with heritable form of PVOD/PCH and in about 9% of sporadic cases. Moreover, an association between occupational exposure to organic solvents and PVOD/PCH was proved. The present review is an attempt to summarise the current data on pathogenesis, risk factors, clinical features and diagnostic algorithm for PVOD/PCH.

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Section: Therapy and Prognosismentioning

confidence: 93%

Section: Therapy and Prognosismentioning

confidence: 97%

Section: Therapy and Prognosismentioning

confidence: 99%

See 1 more Smart Citation

Pulmonary Veno-Occlusive Disease: Pathogenesis, Risk Factors, Clinical Features and Diagnostic Algorithm—State of the Art

Szturmowicz¹,

Kacprzak²,

Szołkowska³

et al. 2018

Advances in Respiratory Medicine

View full text Add to dashboard Cite

show abstract

“…Pulmonary veno-occlusive disease and/or pulmonary capillary hemangiomatosis (PVOD/PCH) are categorized as group 1 pulmonary hypertension, with no effective medical therapy except for lung transplantation. Recently, a small observational study [96] found that imatinib improved survival time and enhanced exercise capacity in patients with PVOD/PCH and may function as a bridge therapy to lung transplantation.…”

Section: Metabolism Dysfunctionmentioning

confidence: 99%

Pathological Mechanisms and Potential Therapeutic Targets of Pulmonary Arterial Hypertension: A Review

Xiao¹,

Chen²,

Zhou³

et al. 2020

Aging and disease

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Pulmonary arterial hypertension (PAH) is a progressive cardiovascular disease characterized by pulmonary vasculature reconstruction and right ventricular dysfunction. The mortality rate of PAH remains high, although multiple therapeutic strategies have been implemented in clinical practice. These drugs mainly target the endothelin-1, prostacyclin and nitric oxide pathways. Management for PAH treatment includes improving symptoms, enhancing quality of life, and extending survival rate. Existing drugs developed to treat the disease have resulted in enormous economic and healthcare liabilities. The estimated cost for advanced PAH has exceeded $200,000 per year. The pathogenesis of PAH is associated with numerous molecular processes. It mainly includes germline mutation, inflammation, dysfunction of pulmonary arterial endothelial cells, epigenetic modifications, DNA damage, metabolic dysfunction, sex hormone imbalance, and oxidative stress, among others. Findings based on the pathobiology of PAH may have promising therapeutic outcomes. Hence, faced with the challenges of increasing healthcare demands, in this review, we attempted to explore the pathological mechanisms and alternative therapeutic targets, including other auxiliary devices or interventional therapies, in PAH. The article will discuss the potential therapies of PAH in detail, which may require further investigation before implementation.

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“…However, imatinib failed to be a treatment option in the phase III trial because of the adverse events despite its efficacy (Hoeper et al 2013). Recently it has been reported that imatinib could improve functional capacity in PVOD patients who were clinically diagnosed, although in some cases the diagnosis is made only pathologically after autopsy or lung transplantation (Overbeek et al 2008;Ogawa et al 2017).…”

Section: Introductionmentioning

confidence: 99%

Beneficial Effects of Imatinib in a Patient with Suspected Pulmonary Veno-Occlusive Disease

Sato

Sugimura

Miura

et al. 2019

Tohoku J. Exp. Med.

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Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension (PH). The prognosis of PVOD patients remains poor, since no effective medical therapy is yet available. Imatinib is a tyrosine kinase inhibitor specific for platelet-derived growth factor receptor and is expected as a treatment option for pulmonary arterial hypertension (PAH). Recently, it has been reported that imatinib improved functional capacity of a patient with PVOD. We here report a patient with suspected PVOD who has been successfully treated with imatinib and is alive for 6 years after diagnosis. A 57-year-old woman was admitted to a hospital for severe dyspnea. Echocardiography suggested the presence of PH, because tricuspid regurgitation pressure gradient was elevated. The patient was then transferred to our hospital by an ambulance ahead of schedule due to fever and worsening dyspnea. Because the patient had no left heart disease, we diagnosed that she had PAH associated with severe right heart failure. We immediately started treatment with nitric oxide (NO) for her severe hypoxia; however, it caused pulmonary edema. We suspected PVOD from CT characteristics and pulmonary edema after PAH-targeted vasodilator therapy, and then started oral imatinib treatment. In response to imatinib, her pulmonary edema gradually improved. Since then, the patient has been alive for 6 years with imatinib and pulmonary vasodilators. At present, lung transplantation is the only effective therapy for PVOD with limited availability. We therefore propose that imatinib may be a treatment option for PVOD and a bridge to lung transplantation.

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Efficacy and safety of long-term imatinib therapy for patients with pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis

Cited by 22 publications

References 20 publications

Pulmonary Veno-Occlusive Disease: Pathogenesis, Risk Factors, Clinical Features and Diagnostic Algorithm—State of the Art

Pulmonary Veno-Occlusive Disease: Pathogenesis, Risk Factors, Clinical Features and Diagnostic Algorithm—State of the Art

Pathological Mechanisms and Potential Therapeutic Targets of Pulmonary Arterial Hypertension: A Review

Beneficial Effects of Imatinib in a Patient with Suspected Pulmonary Veno-Occlusive Disease

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