THU0556 ARTERIAL AND VENOUS THROMBOTIC EVENTS IN IGG4-RELATED DISEASE: A NATIONAL OBSERVATIONAL RETROSPECTIVE STUDY (2024)

Results: One hundred eighty-nine patients with IgG4-RD (135 men/54 women, median age 61 years) were included. During a 12-months median follow-up, one or more arterial thrombotic events occurred in 10 patients and venous thrombotic events in 16 (5.3 and 8.5 events/100 patient-years, respectively).

Arterial complications (coronary artery disease n=5, lower limb peripheral arterial disease n=2, mesenteric ischemia, transient ischemic attack, and carotid thrombosis: n=1) occurred on average 30 months [0-140] after the first symptoms of IgG4-RD. They were inaugural in 2 patients without any cardiovascular risk factor, and associated with IgG4-RD arterial involvement in 3 (coronary aneurysms n=2, leg arteritis n=1). Among patients with arterial thrombosis, 60% had systemic involvement (≥3 organs involved), 89% elevated serum IgG4 (> 3N in 56%), and 57% CRP >10 mg/l. Only 5/10 were treated with steroids at the time of arterial complication, and 4 had never been exposed to steroid therapy. Renal involvement was associated with the occurrence of an arterial thrombotic event (p = 0.03).

Venous thromboembolic complications (deep venous thrombosis (DVT) n=12, pulmonary embolism n=4) occurred on average 24 months [0-164] after the first symptoms of IgG4-RD, but were inaugural in 6 patients. Usual venous thrombosis risk factors were found in only 3/16. Seven patients had retroperitoneal fibrosis (RPF), 2 had mediastinal fibrosis, 60% had localized IgG4-RD (≤2 organs involved), serum IgG4 level was normal in 67% and CRP <10 mg/l in 79%. Nine patients were on steroids at the time of venous thrombosis. RPF was more frequent in the group of IgG4-RD patients with a venous thrombotic event (p = 0.05), and largely associated with DVT in a multivariate analysis (OR=8.36 [2.25-35.93], p = 0.002).