Abstract 145: Differences in Presenting Symptoms of Fibromuscular Dysplasia Patients With Family History of Fibromuscular Dysplasia, Arterial Aneurysm, Dissection, or Sudden Death: A Report from the United States Registry for Fibromuscular Dysplasia

Introduction: Fibromuscular dysplasia (FMD) is a non-atherosclerotic, non-inflammatory vascular disorder which may lead to stenosis, tortuosity, dissection, or aneurysm in any arterial bed. Some affected patients have a family history of FMD, arterial aneurysm (AA), arterial dissection (AD) or sudden death (SD), supporting a genetic predisposition. The clinical characteristics of this patient population have not been described. We hypothesized that patients with a family history of FMD, AA, AD, or SD would present differently than those without.Methods: By July 2014, of 880 patients enrolled in the United States Registry for FMD, 295 had a family history of FMD, AA, AD, or SD. These patients were compared to those without family history, analyzing demographics, symptoms at time of diagnosis, and vascular bed involvement. Student’s t-tests and Fisher’s exact tests were used to analyze continuous and categorical variables, respectively.Results: Patients with a family history of FMD, AA, AD, or SD were younger at diagnosis and more likely to experience CNS symptoms (headache, tinnitus, neck pain), post-prandial abdominal pain, as well as stroke and TIA at time of diagnosis. They also exhibited a trend toward more frequent carotid dissection (Table 1).Conclusions: Patients with a family history of FMD, arterial aneurysm (AA), dissection (AD), or sudden death (SD) more commonly had extracranial arterial bed complications, and experienced symptoms related to this vascular territory at time of diagnosis. These results suggest that FMD may present differently in those with this family history. ![][1] [1]: /embed/graphic-1.gif