Pulmonary arterial hypertension (PAH) is a devastating disease associated with progressive elevation in pulmonary pressures that eventually leads to chronic right heart failure and death. At present, agents with vasod...Pulmonary arterial hypertension (PAH) is a devastating disease associated with progressive elevation in pulmonary pressures that eventually leads to chronic right heart failure and death. At present, agents with vasodilatory properties are being used to palliate the symptoms associated with PAH but there is a need for therapies that can prevent or even reverse established disease. Several lines of evidence have suggested that tyrosine kinase inhibitors like imatinib may have a role in reducing progression and improving outcomes in these patients, but their side effect profile is unclear. We present a case of a 55-year-old female with PAH secondary to connective tissue disease treated with triple PAH specific therapy and compassionate-use imatinib who developed a massive right pleural effusion. Despite multiple therapeutic thoracentesis and aggressive diuresis, the pleural effusion continued to re-accumulate necessitating chest tube placement. Resolution of the pleural effusion was finally achieved after imatinib was held, arguing that the patient’s presentation likely was a drug-related event. We believe that our case highlights a serious adverse reaction to imatinib therapy and stresses the need for more studies to evaluate the safety profile of this medication in patients with PAH.展开更多
文摘Pulmonary arterial hypertension (PAH) is a devastating disease associated with progressive elevation in pulmonary pressures that eventually leads to chronic right heart failure and death. At present, agents with vasodilatory properties are being used to palliate the symptoms associated with PAH but there is a need for therapies that can prevent or even reverse established disease. Several lines of evidence have suggested that tyrosine kinase inhibitors like imatinib may have a role in reducing progression and improving outcomes in these patients, but their side effect profile is unclear. We present a case of a 55-year-old female with PAH secondary to connective tissue disease treated with triple PAH specific therapy and compassionate-use imatinib who developed a massive right pleural effusion. Despite multiple therapeutic thoracentesis and aggressive diuresis, the pleural effusion continued to re-accumulate necessitating chest tube placement. Resolution of the pleural effusion was finally achieved after imatinib was held, arguing that the patient’s presentation likely was a drug-related event. We believe that our case highlights a serious adverse reaction to imatinib therapy and stresses the need for more studies to evaluate the safety profile of this medication in patients with PAH.
