Le Tourneau T, Millaire A, Asseman P, De Groote P, Théry C, Ducloux G
Service de Cardiologie C, Hôpital Cardiologique, CHRU, Lille.
Ann Med Interne (Paris). 1996;147(5):361-8.
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.
巨细胞性主动脉炎(与巨细胞动脉炎相关的主动脉炎)于1937年首次被描述,在20%至40%的巨细胞动脉炎患者中出现,且通常在临床上无明显症状。颞动脉受累通常先于主动脉受累。病变过程可能累及整个主动脉,但并发症通常与胸段主动脉受累有关。巨细胞性主动脉炎患者可能无症状,或表现为主动脉弓综合征、主动脉扩张、主动脉瘤、主动脉夹层、主动脉突然破裂或主动脉瓣关闭不全。胸段动脉瘤通常为梭形,高达50%的患者可能并发夹层。主动脉受累可能是巨细胞动脉炎的首发特征:也可能发生在已有颞动脉炎的患者中,通常在糖皮质激素治疗减量或停药时出现。主动脉炎并发的主动脉破裂可能是3%至12%的巨细胞动脉炎患者的死亡原因。巨细胞动脉炎患者应每年进行临床随访,通过胸部X线和炎症生物标志物评估疾病活动度。如果心脏或血管超声多普勒显示有主动脉弓综合征、主动脉扩张、动脉瘤或主动脉瓣关闭不全的证据,应怀疑有主动脉受累。以1mg/kg/天的剂量开始的糖皮质激素治疗仍然是治疗的关键。随后根据临床病程和辅助检查结果调整剂量。这种治疗可能预防主动脉破裂导致的致命后果。对所有巨细胞动脉炎或风湿性多肌痛患者进行长期随访至关重要,因为并发症可能在疾病后期出现。