Objective: To summarize the clinical features of Takayasu arteritis (TA) patients with pulmonary hypertension due to pulmonary artery involvement.
Methods: Ninety-four TA patients with pulmonary artery involvement treated in Fuwai Hospital from Jun 1988 to Jun 2014 were retrospectively summarized. Patients were divided into two groups according to whether aorta and its main branches affected (APTA, n=48) or not (PTA, n=46). Clinical features and angiographic data were further analyzed.
Results: Of all the patients, male to female rate was 1∶3.3, age ranged from 8 to 60 years with median age of 33.5 years when symptom onset. Main symptoms included dyspnea on exertion. Heart murmur and mid-systolic murmur in pulmonic area were commonly found by physical examination. All patients had pulmonary hypertension by echocardiographic examination. Angiographic data showed that multi-lobular and multi-segmental pulmonary branches were predominantly affected, followed by sub-segmental and peripheral branches. Both sides of pulmonary artery involvement were more common than one side involvement, while the right pulmonary arteries were more often affected than the left. Most of the clinical symptoms and signs between APTA group and PTA group were comparable. However, erythrocyte sedimentation rate and N-terminal pro-natriuretic peptide were significant higher in APTA group than that in PTA group when admission [14.0(5.0, 28.0) vs 8.5(3.0, 19.3) mm/1 h and (1 936±1 769) vs (1 627±1 153) ng/L, both P<0.05].
Conclusions: Dyspnea on exertion may be the main manifestation of TA with pulmonary artery involvement. All patients present with pulmonary hypertension and moderate to severe heart failure when symptom onset.