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We conducted this retrospective study to determine the prevalence of giant cell arteritis (GCA) in patients exhibiting nonatherosclerotic upper and/or lower extremity arterial involvement and to evaluate the clinical features and long-term outcome of those patients.From January 1997 to March 2008, 36 consecutive patients in the Department of Internal Medicine at the University of Rouen medical center received a diagnosis of symptomatic upper/lower extremity vasculitis related to GCA. In the 36 patients, upper/lower extremity vasculitis preceded the initial GCA diagnosis in 7 patients (19.4%), it was identified in association with GCA in 13 patients (36.1%), and it developed after the onset of GCA in the remaining 16 patients (44.4%). GCA clinical manifestations were severe resulting in ischemic complications of the extremities in 10 patients (27.8%). GCA-related large-vessel involvement was located in the upper extremity alone in 21 patients (58.3%), the lower extremity alone in 7 patients (19.4%), and both the upper and lower extremities in 8 patients (22.2%).Arterial involvement in GCA patients with upper extremity vasculitis was distributed in the subclavian (55.6%), axillary (47.2%), and brachial (22.2%) arteries. In patients with lower extremity vasculitis, involvement included the internal iliac artery (11.1%), common femoral artery (13.9%), superficial femoral artery (33.3%), deep femoral artery (5.6%), and popliteal and anterior tibial arteries (5.6%). Aortic localizations were common in GCA patients with upper/lower extremity vasculitis (68.9% of cases).All patients were given steroid therapy at a median daily dose of 1 mg/kg initially. Reconstructive study was performed in 10 patients (27.8%): venous bypass graft (n = 6), angioplasty (n = 1), thromboendarteriectomy (n = 2), or thrombectomy (n = 1); 2 other patients with extremity ischemia underwent amputation. The median observation time was 32 months; the outcome of upper/lower extremity vasculitis was disappearance of clinical symptoms (44.4%), improvement of clinical manifestations (44.4%), and deterioration of clinical manifestations (11.1%). At last follow-up, the median daily dose of prednisone was 6 mg. Steroid therapy could be discontinued in 12 patients (33.3%).We found that upper/lower extremity vasculitis is not uncommon in patients with GCA, and may be present in the early acute phase of GCA. Nevertheless, because upper/lower extremity vasculitis occurs during the course of GCA, yearly clinical vascular examinations may be adequate to screen for upper/lower extremity vasculitis at an early stage in GCA patients. Early diagnosis of GCA-related upper/lower extremity vasculitis is crucial, and can result in decreased severe ischemic complications. Because aortic localizations were common, GCA patients with upper/lower extremity vasculitis should undergo routine investigations for underlying life-threatening aortic complications (aortic ectasia/aneurysm). We also suggest that patients exhibiting aortic complications should undergo routine clinic...

2229 Background: Acquired thrombotic thrombocytopenic purpura (TTP) is still associated with a 10–20% death rate, which did not significantly improve for more than 20 years despite a better awareness about this diagnosis. Usually, death occurs within the first days of management and so far, early prognostic factors of death could not be clearly identified. In this context, the accurate understanding of factors associated with a fatal outcome at the acute phase of the disease would help to better tailor initial treatment and further improve these results. Objective: To identify prognostic factors associated with 1-month death in TTP patients with acquired severe (< 10% of normal activity) ADAMTS13 deficiency. Design: Prospective national cohort of adult (≥ 18 year-old) patients included between October, 2000, and December, 2008. A validation cohort of patients was set up from January, 2009 to March, 2010. Participants: 248 (analysis cohort) and 39 (validation cohort) consecutive adult TTP patients with acquired severe ADAMTS13 deficiency from 39 French centers. Measurements: 30-day mortality after treatment initiation according to characteristics at inclusion. Results: When compared to survivors, non-survivors (11%) were older (54.0 ± 19.4 versus 39.0 ± 15.5 year-old, respectively, P <.001) and had more frequently a past history of arterial hypertension (37% versus 10%, respectively, P <.001) and ischemic heart disease (19% versus 4%, respectively, P =.002). Prognosis was increasingly poor with age (p <.004), particularly in patients ≥ 60 year-old. On presentation, cerebral manifestations were more frequent in non-survivors (81% versus 55%, respectively, P =.009) and serum creatinine level was higher (172 ± 123 versus 117 ± 91 μmol/L, respectively, P =.037). Death occurred after a few days (mean 7 days, interquartile range = [3, 12]), in a context of one or multiple organ failure in relation with an uncontrolled TTP. Platelet count between diagnosis and death did not significantly increase (20 ± 25×109/L versus 28 ± 46×109/L, respectively, P =.44). The most significant independent variables for determining death were age (P <.001), cerebral involvement (stupor and seizure) and LDH level > 10 times normal value (P <.04 for both). After computing the risk scores using these 3 variables (Table), patients were stratified into 3 distinctive risk groups regarding death: low, 0 and 1; intermediate, 2; and high, 3 and 4. The proportion of positive predictive value for 30-day death was 11–13% in the low risk group, 20% in the intermediate group, and 39–50% in the high risk group. This score was confirmed in the validation cohort using these variables, with higher values corresponding to increased risk of early death (P <.01). Conclusions: A risk score for early death was defined in patients with TTP and validated on an independent cohort. This score should help to stratify early treatment and intensify patients with a worse prognosis. Importantly, we provide clear evidence that age is an important prognostic factor of TTP. Consequently, old patients with a diagnosis of TTP should benefit from more intensive supportive care and should be monitored more closely in intensive care units with more aggressive attention to cardiac and renal function. Disclosures: Rottensteiner: Baxter Innovations GmbH: Employment.

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