Pulmonary Arteries Involvement in Takayasu's Arteritis: Two Cases and Literature Review

• Published in: Seminars in arthritis and rheumatism Vol. 41; no. 3; pp. 461 - 470
• Main Authors: Toledano, Kohava, MD, Guralnik, Ludmila, MD, Lorber, Avraham, MD, Ofer, Amos, MD, Yigla, Mordechai, MD, Rozin, Alexander, MD, Markovits, Doron, MD, Braun-Moscovici, Yolanda, MD, Balbir-Gurman, Alexandra, MD
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 01-12-2011; Elsevier
• Subjects: Adult; Biological and medical sciences; Diseases of the osteoarticular system; Familial Primary Pulmonary Hypertension; Female; Humans; Hypertension, Pulmonary - complications; Hypertension, Pulmonary - diagnostic imaging; Hypertension, Pulmonary - physiopathology; Male; Medical sciences; Pneumology; pulmonary arterial hypertension; pulmonary arteritis; Pulmonary Artery - diagnostic imaging; Pulmonary Artery - physiopathology; Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases; Radiography; Retrospective Studies; Rheumatology; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Takayasu Arteritis - complications; Takayasu Arteritis - diagnostic imaging; Takayasu Arteritis - physiopathology; Takayasu's arteritis; Takayasu's arteritis; pulmonary arterial hypertension; pulmonary arteritis; Respiratory disease; Lung; Rheumatology; Cardiovascular disease; Review; Pulmonary artery; Pulmonary hypertension; Vascular disease; Vasculitis; Systemic disease; Bibliographic review; Takayashu arteritis
• ISSN: 0049-0172; 1532-866X
• DOI: 10.1016/j.semarthrit.2011.06.001

Objectives 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.