title: Pulmonary arteries involvement in Takayasu's arteritis: two cases and literature review.
abstract: OBJECTIVE
  To review pulmonary arteritis (PA) complicated by pulmonary arterial hypertension (PAH) in Takayasu's arteritis (TA).
  
  
  METHODS
  Two cases of PA and PAH in TA patients and similar cases published in the Medline database from 1975 to 2009 were reviewed.
  
  
  RESULTS
  Forty-six cases (females 89.1%, Asians 65%, mean age 34.6 years) were analyzed, 42.2% of which had PAH. Isolated PA was reported in 31.8%. Respiratory symptoms were presented as dyspnea (75.5%), chest pain (48.9%), hemoptysis (42.2%), and cough (17.7%). Hypertension, vascular bruits, and diminished/absent pulses were reported in 48.9% of patients. A diagnosis of PA was based on abnormal uptake on pulmonary perfusion scan and a finding of stenosis, narrowing, occlusion, and irregularity on computed tomography or magnetic resonance imaging, and/or pulmonary angiography. Patients were treated with glucocorticoids (77.5%), disease-modified antirheumatic drugs (35%), and warfarin (20%); only a few were treated with biological agents. Vascular procedures were performed in 52.5% of cases, on pulmonary arteries in 37.5% with good results. The outcome was death in 20.5% of PA patient and 33.3% in PAH patients.
  
  
  CONCLUSIONS
  TA may be complicated by life-threatening PA and PAH. Clinical signs are not specific and may be masked by involvement of the aorta and its branches. Treatment with glucocorticoids and disease-modified antirheumatic drugs has only partial effect, which may be intensified by biological agents. Invasive procedures on pulmonary arteries may be a complementary option. PA and PAH in TA patients should be recognized early and treated promptly for prevention of irreversible vascular damage.
authors.affiliation: B. Shine Rheumatology Unit, Rambam Health Care Campus, Haifa, Israel.
authors.forename: Kohava
authors.initials: K
authors.lastname: Toledano
authors.forename: Ludmila
authors.initials: L
authors.lastname: Guralnik
authors.forename: Avraham
authors.initials: A
authors.lastname: Lorber
authors.forename: Amos
authors.initials: A
authors.lastname: Ofer
authors.forename: Mordechai
authors.initials: M
authors.lastname: Yigla
authors.forename: Alexander
authors.initials: A
authors.lastname: Rozin
authors.forename: Doron
authors.initials: D
authors.lastname: Markovits
authors.forename: Yolanda
authors.initials: Y
authors.lastname: Braun-Moscovici
authors.forename: Alexandra
authors.initials: A
authors.lastname: Balbir-Gurman
mesh_terms: D000328:Adult; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology; D008297:Male; D011651:Pulmonary Artery / Q000000981:diagnostic imaging / Q000503:physiopathology*; D011859:Radiography; D012189:Retrospective Studies; D013625:Takayasu Arteritis / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology