Angiographic characteristics of vasculopathy in patients with idiopathic inflammatory myopathies and systemic sclerosis

To describe the peripheral angiographic features of vasculopathy in idiopathic inflammatory myopathies (IIM) and systemic sclerosis (SSc) in comparison to polyarteritis nodosa (PAN). Angiograms of 47 extremities (24 upper and 23 lower) of 11 patients with IIM (n = 5) and SSc (n = 6), and 12 patients with PAN who presented with critical limb ischemia were retrospectively analyzed with regards to the presence of stenosis, occlusion, aneurysms and delayed distal flow, and degree of neovascularization. Diffuse narrowing was more frequent (66.1 vs. 38.0%, p = 0.001), whereas multifocal stenosis (6.5% vs. 26.8%, p = 0.002), abrupt occlusion (11.3% vs. 29.6%, p = 0.010) and aneurysm formation (1.6% vs. 11.3%, p = 0.037) were less frequent in IIM/SSc than PAN. In distal arteries, tapered occlusion (95.5% vs. 76.0%, p = ns) and delayed flow (77.3% vs. 48.0%, p = 0.039) were more common in IIM/SSc than PAN. After 1 year, auto- or surgical amputation tended to be more frequent in IIM/SSc than PAN (36.4% vs. 16.7%, p = ns). In conclusion, diffuse narrowing, tapered occlusion and delayed distal flow on conventional angiograms tend to be more frequent in IIM/SSc than PAN. Further studies are needed to verify these findings in a larger prospective cohort.