Severe Pulmonary Involvement In Takayasu Disease - The Case-Reports And A Review Of The Literature

Three cases of pulmonary arterial disease were identified out of a cohort of 75 cases of Takayasu's disease. In the first case, pulmonary hypertension considered to be idiopathic caused massive haemoptysis and the death of a 48 year old Spanish woman; autopsy revealed characteristic stenoses of the common carotid arteries. In the second case, haemoptysis led to pneumonectomy in a 23 year old West Indian woman with a diagnosis of agenesis of the right pulmonary artery. Disease of the supraaortic vessels occurred several months later and a retrospective diagnosis of Takayasu's disease of the pulmonary artery was made. In the third case, systematic pulmonary angiography in a 41 year old French woman referred for Takayasu's disease of the supraaortic arteries showed severe stenosis of the right pulmonary artery. She developed severe haemoptysis four years later which led to an attempted balloon angioplasty of the right pulmonary artery and embolisation of a branch of the right coronary artery thought to be the cause of the haemoptysis. This was complicated by posterior wall myocardial infarction but the haemoptysis did not recur thereafter.These three cases and a review of the literature show that the pulmonary lesions of Takayasu's disease occur mostly on the main right pulmonary artery but they may be more distal and involve the pulmonary arterioles. A coronaro-broncho-pulmonary collateral circulation may develop distal to the stenosed segments.The main complications are pulmonary hypertension and massive haemoptysis. Surgery is possible in cases of stenosis of the right main pulmonary artery. The potential role of endoluminal angioplasty is discussed.