%0 Journal Article %1 Olin:2012:Circulation:22615343 %A Olin, J W %A Froehlich, J %A Gu, X %A Bacharach, J M %A Eagle, K %A Gray, B H %A Jaff, M R %A Kim, E S %A Mace, P %A Matsumoto, A H %A McBane, R D %A Kline-Rogers, E %A White, C J %A Gornik, H L %D 2012 %J Circulation %K FMD %R 10.1161/CIRCULATIONAHA.112.091223 %T The United States Registry for Fibromuscular Dysplasia: Results in the First 447 Patients %U http://www.ncbi.nlm.nih.gov/pubmed/22615343?dopt=Abstract %X BACKGROUND: Fibromuscular dysplasia (FMD), a non-inflammatory disease of medium sized arteries, may lead to stenosis, occlusion, dissection and/or aneurysm. There has been little progress in understanding the epidemiology, pathogenesis and outcomes since its first description in 1938. METHODS AND RESULTS: Clinical features, presenting symptoms, and vascular events are reviewed for the first 447 patients enrolled in a national FMD registry from 9 U.S. sites. Vascular beds were imaged selectively based on clinical presentation and local practice. The majority of patients were female (91%) with a mean age at diagnosis of 51.9 (SD 13.4 years, range 5-83 years). Hypertension, headache, and pulsatile tinnitus were the most common presenting symptoms of the disease. Self-reported family history of stroke (53.5%), aneurysm (23.5%) and sudden death (19.8%) were common, but FMD in first or second degree relatives was reported only in 7.3%. FMD was identified in the renal artery in 294 patients, extracranial carotid arteries in 251 patients, vertebral arteries in 82 patients. A past or presenting history of vascular events were common: 19.2% of patients had a transient ischemic attack or stroke, 19.7% had suffered arterial dissection(s), and 17% of patients had an aneurysm(s). The most frequent indications for therapy were hypertension, aneurysm, and dissection. CONCLUSIONS: In this registry, FMD occurred primarily in middle-aged women, though it presents across the lifespan. Cerebrovascular FMD occurred as frequently as renal FMD. While a significant proportion of FMD patients may present with a serious vascular event, many present with nonspecific symptoms and a subsequent delay in diagnosis.

@article{Olin:2012:Circulation:22615343, abstract = {BACKGROUND: Fibromuscular dysplasia (FMD), a non-inflammatory disease of medium sized arteries, may lead to stenosis, occlusion, dissection and/or aneurysm. There has been little progress in understanding the epidemiology, pathogenesis and outcomes since its first description in 1938. METHODS AND RESULTS: Clinical features, presenting symptoms, and vascular events are reviewed for the first 447 patients enrolled in a national FMD registry from 9 U.S. sites. Vascular beds were imaged selectively based on clinical presentation and local practice. The majority of patients were female (91%) with a mean age at diagnosis of 51.9 (SD 13.4 years, range 5-83 years). Hypertension, headache, and pulsatile tinnitus were the most common presenting symptoms of the disease. Self-reported family history of stroke (53.5%), aneurysm (23.5%) and sudden death (19.8%) were common, but FMD in first or second degree relatives was reported only in 7.3%. FMD was identified in the renal artery in 294 patients, extracranial carotid arteries in 251 patients, vertebral arteries in 82 patients. A past or presenting history of vascular events were common: 19.2% of patients had a transient ischemic attack or stroke, 19.7% had suffered arterial dissection(s), and 17% of patients had an aneurysm(s). The most frequent indications for therapy were hypertension, aneurysm, and dissection. CONCLUSIONS: In this registry, FMD occurred primarily in middle-aged women, though it presents across the lifespan. Cerebrovascular FMD occurred as frequently as renal FMD. While a significant proportion of FMD patients may present with a serious vascular event, many present with nonspecific symptoms and a subsequent delay in diagnosis.}, added-at = {2012-05-30T09:21:35.000+0200}, author = {Olin, J W and Froehlich, J and Gu, X and Bacharach, J M and Eagle, K and Gray, B H and Jaff, M R and Kim, E S and Mace, P and Matsumoto, A H and McBane, R D and Kline-Rogers, E and White, C J and Gornik, H L}, biburl = {https://www.bibsonomy.org/bibtex/2d5be1420690bb7935ddd9ed429eecb9b/freefattyacids}, description = {The United States Registry for Fibromuscular Dys... [Circulation. 2012] - PubMed - NCBI}, doi = {10.1161/CIRCULATIONAHA.112.091223}, interhash = {deb35a12937da1d95995704b9603af80}, intrahash = {d5be1420690bb7935ddd9ed429eecb9b}, journal = {Circulation}, keywords = {FMD}, month = may, pmid = {22615343}, timestamp = {2012-05-30T09:21:36.000+0200}, title = {The United States Registry for Fibromuscular Dysplasia: Results in the First 447 Patients}, url = {http://www.ncbi.nlm.nih.gov/pubmed/22615343?dopt=Abstract}, year = 2012 }