POS1386 ANGIOGRAPHIC CHARACTERISTICS OF VASCULOPATHY IN PATIENTS WITH CONNECTIVE TISSUE DISEASES

Background: Vasculopathies are a heterogeneous group of morphologically and pathogenetically distinct vascular diseases. They include both non-inflammatory and inflammatory vasculopathies. Patients with connective tissue disease (CTD), including systemic sclerosis (SSc), dermatomyositis (DM), and polymyositis (PM), can develop a non-thrombotic proliferative vasculopathy (NTPV), a distinctive disease entity characterized by vascular wall proliferation without overt evidence of vascular inflammation and intraluminal thrombosis. Objectives: This study aimed to analyze the angiographic features of NTPV in patients with CTD, including SSc, DM, and PM in comparison to polyarteritis nodosa (PAN). Methods: Angiograms of 47 extremities (24 upper and 23 lower extremities) of 11 patients with CTD (6 SSc, 4 DM, and 1 PM), and 12 patients with PAN who presented with critical digital ischemia between January 2001 and May 2020 were analyzed. The degree and pattern of stenosis, occlusion, aneurysms, and neovascularization in proximal arteries (defined as arteries above the wrist and ankle) and distal arteries (defined as those at or below the wrist and ankle) were compared between CTD-vasculopathy and PAN. Results: Diffuse narrowing was significantly more frequent (66.1% vs. 38.0%; p=0.001), whereas multifocal stenosis was significantly less frequent (6.5% vs. 26.8%, p=0.002) in the CTD group than in the PAN group. All patients with CTD and 72.0% with PAN had diffuse narrowing in the distal arteries (p =0.010). Tapered occlusion was more frequent than abrupt occlusion in patients with CTD (43.5% vs. 11.3%). Abrupt occlusion (11.3% vs. 29.6%, p=0.010) and aneurysm formation (1.6% vs. 11.3%; p=0.037) were significantly less frequent in the CTD than in the PAN group. After 1 year, three patients (27.3%) in the CTD group and seven (58.3%) in the PAN group showed improvements in digital ischemia. Moreover, four patients (36.4%) in the CTD group and two (16.7%) in the PAN group underwent auto- or surgical amputation. Conclusion: Patients with CTD-vasculopathy exhibit more frequently diffuse smooth narrowing, tapered occlusion and delayed distal blood flow on conventional angiograms and worse outcomes than with PAN patients. Larger studies are needed to confirm the current findings. References: [1]Kahaleh MB. Vascular involvement in systemic sclerosis (SSc). Clin Exp Rheumatol. 2004;22(3 Suppl 33):S19-23. [2]Lee JS, Kim H, Lee EB, Song YW, Park JK. Nonthrombotic proliferative vasculopathy associated with antiphospholipid antibodies: A case report and literature review. Mod Rheumatol. 2019;29(2):388-92. Disclosure of Interests: Jina Yeo: None declared, Eun-Ah Park: None declared, Eun Bong Lee Consultant of: Pfizer, Grant/research support from: GC Pharma and Handok Inc., Yeong Wook Song: None declared, Jin Kyun Park: None declared