POS1386 ANGIOGRAPHIC CHARACTERISTICS OF VASCULOPATHY IN PATIENTS WITH CONNECTIVE TISSUE DISEASES
Authors: J. Yeo, E. A. Park, E. B. Lee, Y. W. Song, J. K. Park
Journal: Annals of the Rheumatic Diseases (2021)

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. Figure 1. Angiographic features of CTD-vasculopathy and PAN. Arteries in the (A) upper extremities and (B) lower extremities of patients with CTD-vasculopathy and PAN. Diffuse narrowing is indicated by white arrowheads; tapered occlusion by white arrows; multifocal stenosis by black arrowheads; abrupt occlusion by black arrows; aneurysmal changes by empty arrows; grade 2 tortuosity by white stars; and grade 3 tortuosity by black stars. CTD, connective tissue disease; PAN, polyarteritis nodosa. Table 1. Comparison of angiographic parameters between CTD-vasculopathy and PAN. CTD (upper 14, lower 8 ) PAN (upper 10, lower 15 ) p -value Total number of images 62 71 Shoulder/elbow/wrist and hand 11/14/14 8/10/10 Femoral/knee/ankle and foot 7/8/8 13/15/15 Stenosis Diffuse narrowing 41/62 (66.1%) 27/71 (38.0%) 0.001 Focal stenosis 13/62 (21.0%) 10/71 (14.1%) 0.295 Multifocal stenosis 4/62 (6.5%) 19/71 (26.8%) 0.002 Occlusion Tapered occlusion 27/62 (43.5%) 23/71 (32.4%) 0.185 Abrupt occlusion 7/62 (11.3%) 21/71 (29.6%) 0.010 Aneurysm 1/62 (1.6%) 8/71 (11.3%) 0.037 Neovascularization in muscular branch Tortuosity Grade 1 45/62 (72.6%) 30/68 (39.1%) 0.002 Grade 2 11/62 (17.7%) 14/68 (23.9%) Grade 3 6/62 (9.7%) 24/68 (37.0%) Tortuosity grade 1, normal; grade 2, mild to moderate; grade 3, severe (hypertortuosity). CTD, connective tissue disease; PAN, polyarteritis nodosa. 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