Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0729233 (Thoracic)
 6,478 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

First described in 1937, giant cell aortitis (aortitis associated with giant cell arteritis) occurs in 20 to 40% of patients with giant cell arteritis and is often clinically silent. Temporal involvement usually precedes aortic involvement. The process may involve the entire aorta, but complications are usually related to thoracic involvement. Patients with giant cell aortitis may be asymptomatic, or present with aortic arch syndrome, dilation of the aorta, aortic aneurysm, aortic dissection, sudden rupture of the aorta, or aortic valve incompetence. Thoracic aneurysms are usually fusiform and can be complicated by dissection in up to 50% of patients. Aortic involvement may be the presenting feature of giant cell arteritis: it may also occur in patients with preexisting temporal arteritis, often when corticosteroid therapy is reduced or discontinued. Aortic rupture complicating aortitis may be the cause of death in 3-12% of patients with giant cell arteritis. Clinical follow-up with assessment of disease activity by chest X-ray and biological markers of inflammation should be performed yearly in giant cell arteritis. Aortic involvement should be suspected if cardiac or vascular echo-Doppler shows evidence of an aortic arch syndrome, aortic dilation, aneurysm, or of aortic valve incompetence. Corticosteroid therapy, beginning with a dose of 1 mg/kg/day remains the key point of therapy. The dose is subsequently adjusted based on the clinical course and the results of ancillary tests. This treatment might prevent fatal outcome with aortic rupture. Long-term follow-up of all patients with giant cell arteritis or polymyalgia rheumatica is essential, as complications may develop late in the course of the disease.
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PMID:[Aortitis in Horton disease. Review of the literature]. 903 41

Pulmonary involvement is considered to be rare in giant cell arteritis (GCA), usually occurring in the course of the disease. We describe the case of a patient who developed left pleural effusion revealing GCA. Thoracic CT scan demonstrated an abundant left pleural effusion and a thickening of the aortic wall. The patient's condition improved rapidly, with regression of pulmonary clinical features and complete clearance of pleural effusion, after institution of steroid therapy. Our case report reinforces the possibility of unusual presentation of GCA; such a diagnosis should, therefore, be considered in elderly patients presenting with pulmonary manifestations, even in the absence of typical clinical features of temporal arteritis (e.g. headache, jaw claudication, blurred vision, scalp tenderness) or polymyalgia rheumatica.
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PMID:Pleural effusion revealing giant cell arteritis. 1517 28

In 4 patients with temporal arteritis or polymyalgia rheumatica, women aged 60, 57, 83 and 73 years respectively, signs of aortic involvement were established. The first patient presented with signs of systemic inflammation without signs of temporal arteritis or aortitis. In the second, an acute symptomatic thoracoabdominal aneurysm developed. In the third, temporal arteritis was associated with chronic progressive dilatation ofthe thoracic aorta. The fourth developed signs of intermittent claudication of the extremities. The clinical manifestations in all patients were attributed to chronic inflammation of the aorta caused by giant cell arteritis. Aortic giant cell arteritis frequently accompanies temporal arteritis, but is rarely diagnosed. Up to 75% of patients with temporal arteritis may have some degree of aortic involvement. Thoracic aneurysms, complicated by rupture or dissection, are the most serious complications. Aortic disease associated with signs of systemic inflammation should trigger the suspicion of giant cell arteritis. Corticosteroids are the most important part of treatment. Three patients recovered following treatment; the first two received an endoprosthesis; in the woman aged 83 years, this was not technically possible; she died after 1.5 years.
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PMID:[Aortic involvement in patients with temporal arteritis and polymyalgia rheumatica]. 1615 30