Background/Purpose .Takayasu arteritis (TA) is a chronic inflammatory disease that primarily affects large vessels, such as the aorta and its main branches. To report clinical features, morphologic findings, treatment and long-term outcome of a large cohort of patients with TA, and to determine risk factors for the occurrence of severe ischemic complications (SIC) during follow-up.
Methods .We performed a retrospective multicenter study of characteristics and outcomes of 182 patients with TA fulfilling the American College of Rheumatology criteria. Characteristics at presentation, SIC, relapses and deaths were recorded.
Results . The median age [interquartile range] at onset of symptoms was 31 [24 ; 45] years and with a predominance of females (85%). The median delay of diagnosis was 10 [0 ; 47] months. The most common clinical findings were vascular bruits (52%), unequal or absent pulses (47%), and upper extremity blood pressure discrepancy >10 mm Hg (44%). Major complications at diagnosis were hypertension (36%), aneuvrysm (24%), and aortic regurgitation (18%). Forty six percent of patients had extensive disease at diagnosis according to Numano type V. Twenty percent of patients had another inflammatory or auto-immune disease associated to TA, mostly ankylosing spondylitis, crohn disease and sarcoidosis. Stenotic lesions were 3.1-fold more common than were aneuvrysms (78% versus 25%, respectively). Revascularization procedure was required for 49% of patients. The median delay between diagnosis and first surgery or endovascular intervention was 5 [0 ; 17.5] months. Glucocorticoids were prescribed in 151 (83%) patients. The median delay in initiation of corticosteroids was 1 [0 ; 5] months. Fifty eight percent of patients required additional immunosuppressive agent. The median delay in initiation of the first immunosuppressor was 11 [2 ; 29.5] months. Twenty six percent of patients required three or more antihypertensive drugs. SIC occurred in 38 (21%) patients after TA diagnosis. SIC-free survival at 10 years was 82% in patients without refractory hypertension versus 34% in patients with refractory hypertension (i.e. requiring ≥ 3 antihypertensive drugs) (P = 0.003). SIC-free survival at 5 years was 89% in patients with immunosuppressive agent versus 78% without immunosuppressive agent (P = 0.175). In multivariable analysis, predictive variables for the occurrence of SIC after TA diagnosis were refractory hypertension (OR 12.03 [95%CI 2.78-52.07], P = 0.001), relapse (OR 7.11 [95%CI 1.82-27.78], P = 0.005) and SIC at time of TA diagnosis (OR 4.65 [95%CI 1.10-19.67], P = 0.036). Forty four percent relapsed after a median follow-up of 69 [31 ; 145] months. The median relapse-free survival was 16 [7 ; 62] months. The mortality rate was 12%. Five of 7 (71%) deaths were related to cardiovascular complication.
Conclusion . In this cohort of TA patients, risk factors for late-developing SIC were refractory hypertension, relapse and SIC at time of TA diagnosis. Immunosuppressive agents may reduce late-developing SIC. Despite prolonged and intensive therapy, TA remains a major cause of cardiovascular morbi-mortality.
Disclosure:
C. Comarmond,
None;
T. Mirault,
None;
J. E. Kahn,
None;
P. Cluzel,
None;
F. Koskas,
None;
L. Chiche,
None;
J. Gaudric,
None;
E. Messas,
None;
P. Cacoub,
Astra Zeneca, Bayer, Boehringer Ingelheim, Gilead, Glaxo Smith Kline, Janssen, Merck Sharp Dohme, Roche, Servier, Vifor.,
5;
D. Saadoun,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/risk-factors-for-severe-ischemic-complications-in-takayasu-arteritis-a-french-multicenter-retrospective-cohort-of-182-patients/