Abstract

Introduction: Fibromuscular dysplasia (FMD) is a polyvasculopathy that may result in aneurysm or dissection in affected arterial beds. A common symptom of FMD is headache (HA). The purpose of this study is to describe and compare characteristics of FMD patients with (w/HA) and without headaches (wo/HA) in the current US Registry for FMD. Method: Data regarding demographics, family history, medical history, and vascular beds affected in patients with FMD were collected from the US Registry for FMD between 2009 and 2016. Patients with complete HA data were included, and data were compared between patients w/HA and wo/HA as reported at time of registry enrollment. Results: Of the 1,433 patients in the registry, 1,251 (87.3%) had complete HA data. Most were female (94.1%), with a mean age of 56.0±12.4. A history of HA was reported in 844 (67.5%) patients and 54.2% reported a HA at least weekly. No difference in gender distribution was seen between HA groups. Patients w/HA were younger at the time of FMD diagnosis (50.6±11.8 years v 58.0±13.8 years, p<0.001) and were less likely to have a history of HTN (66.8% v 74.8%, p=0.004), hyperlipidemia (36.9% v 47.2%, p=0.004), or renal failure (2.6% v 5.7%, p=0.009). Patients w/HA were more likely to have a history of aneurysm or dissection in the extracranial carotid, intracranial, or vertebral arteries (aneurysm: 13.0% v 7.2% p=0.003; dissection: 25.1% v 8.3%, p<0.001). No differences in extracranial carotid, intracranial, or vertebral FMD involvement between patients w/ and w/o HA were seen when those with history of aneurysm or dissection of those arteries were excluded (71.2% v 66.8%, p=0.20). Headache patients more often reported a history of depression (25.1% v 16.5%, p=0.01) and anxiety (28.3% v 18.0%, p=0.003). Conclusion: More than half of FMD patients experience HAs, most on an at least weekly basis. FMD patients with HA tended to be younger, and more likely to have extracranial carotid, intracranial, or vertebral disease as well as aneurysm and dissection. Depression and anxiety was also more common in FMD patients with HA. Further research is needed to classify the types of headaches being experienced as well as effective management strategies.

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