Long-term outcomes of kidney donors with fibromuscular dysplasia.

BACKGROUND: Fibromuscular dysplasia (FMD) is a non-atherosclerotic systemic arterial disease that is not infrequently discovered during kidney donor evaluation. Current guidelines do not provide recommendations regarding the use of kidneys from donors with FMD and there is a paucity of data on the outcomes of these donors. METHODS: The Renal and Lung Living Donor Evaluation (RELIVE) study addressed long-term outcomes of 8922 kidney donors who donated between 1963 and 2007. We compared the development of hypertension, cardiovascular disease (CVD), proteinuria and reduced estimated glomerular filtration rate (eGFR) in 113 kidney donors with FMD discovered during donor evaluation versus 452 propensity score matched donors without FMD. Outcomes modeling with logistic and Cox regression analysis and Kaplan-Meier statistics were performed. RESULTS: Donors with FMD were older (51 versus 39 years), were more likely to be women (80% versus 56%) and had a higher systolic blood pressure at donation (124.7 versus 121.3 mmHg) (P < 0.05 for all). After a mean ± standard deviation follow-up of 15.5 ± 8.9 years, a similar proportion of donors with and without FMD were alive, and developed hypertension (22.2% versus 19.8%), proteinuria (20.6% versus 13.7%) and CVD (13.3% versus 13.5%). No donor with FMD developed an eGFR <30 mL/min/1.73 m2 or end-stage kidney disease. The multivariable risk of mortality, CVD and renal outcomes in donors with FMD was not elevated. CONCLUSIONS: Kidney donors with FMD appear to do well, do not appear to incur increased risks of hypertension, proteinuria, CVD or reduced eGFR, and perhaps carefully selected candidates with FMD can safely donate as long as involvement of other vascular beds is ruled out.

Displasia fibromuscular en la Argentina. Registro del grupo de trabajo de Hipertensión Secundaria de la Sociedad Argentina de Hipertensión Arterial (SAHARA-DF): proyecto de protocolo y resultados preliminares / Fibromuscular dysplasia in Argentina. A registry from the Secondary Hypertension working group of the Argentine Society of Hypertension (SAHARA-DF): project protocol and preliminary results

En la Argentina no existen datos epidemiológicos sobre displasia fibromuscular. La realización de un registro nacional puede aportar información que conduzca a una actualización de los consensos y recomendaciones para un correcto diagnóstico, evaluación y tratamiento. El Registro Argentino de Displasia Fibromuscular (SAHARA-DF) inició su actividad de recopilación de datos en octubre de 2015. Al año 2019 se confirmaron 49 pacientes (44 mujeres, 38 hipertensos, edad 45,3 ± 17,2 años, 12 con presentación neurológica). Veintidós pacientes tuvieron lesiones vasculares en más de un sitio, a pesar del sesgo diagnóstico por falta de estudios complementarios en casi la mitad de los casos. El sitio afectado más frecuente fue el renovascular, seguido por el carotídeo y el ilíaco, y las lesiones multifocales fueron más frecuentes que las unifocales (35 versus 14, respectivamente). Se constató la presencia de aneurismas asociados en 13 casos y disección arterial en 4 casos. De las 22 angioplastias renales realizadas, 14 fueron con colocación de stent (endoprótesis). En este estudio preliminar de una población argentina se evidencia el carácter sistémico de la enfermedad y se plantea un llamado a actuar en cuanto a la necesidad de debatir el algoritmo diagnóstico y el método de tratamiento. (AU)

In Argentina there are no epidemiological data regarding fibromuscular dysplasia. Building a National Registry may provide information leading to updated consensus and recommendations for a correct diagnosis, assessment and treatment. Data gathering for the Argentine Registry of Fibromuscular Dysplasia (SAHARA-DF) was initiated in October 2015. By 2019, 49 patients were confirmed (44 women, 38 hypertensives, age 45.3 ± 17.2 years, 12 with a neurological presentation). Twenty-two patients had multi-site vascular lesions, in spite of a diagnosis bias due to lack of supporting studies in almost half of the cases. The renovascular site was the most affected, followed by the carotid and iliac sites, and multifocal lesions were more frequent than unifocal (35 versus 14, respectively). Associated aneurysms were found in 13 cases, and arterial dissection in 4. Twenty-two renal angioplasties were performed, 14 with stent placement. In this preliminary study of an Argentinian population, the systemic nature of the disease is evidenced, and a call for action arises regarding the need for discussing the diagnostic algorithm and treatment method. (AU)

Large volume haemorrhage following transoral robotic surgery (TORS) as a result of fibromuscular dysplasia: first reported case.

We present a case of a 61-year-old woman who suffered a haemorrhagic complication of an aneurysmal left lingual artery, secondary to fibromuscular dysplasia, following transoral robotic surgery (TORS). She was admitted through the emergency department 3 days after resection of a central base of tongue tumour. She suffered a massive haemorrhage requiring intensive care admission, blood transfusion, intubation, operative and endovascular intervention. The diagnosis of fibromuscular dysplasia was made at angiography. During attempts to embolise the culprit left lingual artery, the vessel dissected at its origin leading to arrest of bleeding. Repeat angiograms during her admission demonstrated unchanged appearances and ruled out recanalisation of the vessel. She was extubated and remained stable on the ward, discharged home some days later in good health. To our knowledge, this is the first ever reported case of fibromuscular dysplasia affecting the lingual artery leading to aneurysmal dilation and severe haemorrhage following TORS.

First international consensus on the diagnosis and management of fibromuscular dysplasia.

This article is a comprehensive document on the diagnosis and management of fibromuscular dysplasia (FMD) which was commissioned by the Working Group 'Hypertension and the Kidney' of the European Society of Hypertension (ESH) and the Society for Vascular Medicine (SVM). This document updates previous consensus documents/scientific statements on FMD published in 2014 with full harmonization of the position of European and US experts. In addition to practical consensus-based clinical recommendations, including a consensus protocol for catheter-based angiography and percutaneous angioplasty for renal FMD, the document also includes the first analysis of the European/International FMD Registry and provides updated data from the US Registry for FMD. Finally, it provides insights on ongoing research programs and proposes future research directions for understanding this multifaceted arterial disease.

Prevalence of smoking and clinical characteristics in fibromuscular dysplasia. The ARCADIA-POL study.

PURPOSE: Smoking was identified as a potential factor contributing to fibromuscular dysplasia (FMD). To evaluate the prevalence of smoking and clinical characteristics in FMD subjects. MATERIAL AND METHODS: We analysed 190 patients with confirmed FMD in at least one vascular bed. The rate of smokers in FMD patients was compared to that in two control groups selected from a nationwide survey. RESULTS: The rate of smokers in FMD patients was 42.6%. There were no differences in frequency of smokers between FMD patients and: a group of 994 matched control subjects from general population and a group of matched hypertensive subjects. There were no differences in the characteristics of FMD (including rates of multisite FMD and significant renal artery stenosis) and its complications (including rates of dissections and aneurysms) between smokers and non-smokers. Smokers as compared with non-smokers were characterized by higher left ventricle mass index. CONCLUSIONS: There is no difference in the rate of smokers between FMD patients and subjects from the general population. Moreover, we did not find any association between smoking and clinical characteristics of FMD patients nor its extent and vascular complications. Our results do not support the hypothesis that smoking is involved in the pathophysiology of FMD.

Visceral Fibromuscular Dysplasia: From asymptomatic disorder to emergency.

Fibromuscular dysplasia (FMD) is an idiopathic, segmental, non-atherosclerotic and non-inflammatory disease of the musculature of arterial walls, leading to stenosis of small and medium-sized arteries, mostly involving renal and cervical arteries. As a result of better and more systematic screening, it appears that involvement of the splanchnic vascular bed is more frequent than originally assumed. We review epidemiology, pathogenesis, clinical picture as well as diagnosis and treatment of visceral artery (VA) FMD. The clinical picture is very diverse, and diagnosis is based on CT-, MR- or conventional catheter-based angiography. Involvement of VAs generally occurs among patients with multi-vessel FMD. Therefore, screening for VA FMD is advised especially in renal artery (RA) FMD and in case of aneurysms and/or dissections. Treatment depends on the clinical picture. However, the level of evidence is low, and much of the common practice is extrapolated from visceral atherosclerotic disease.

Colonic Adventitial Fibromuscular Dysplasia: A Nonspecific Arteriopathy Associated With Hirschsprung Disease and Other Obstructive Disorders.

Background Smooth muscle differentiation ("adventitial fibromuscular dysplasia," AFD) was purported as specific to arteries in the transition zone of Hirschsprung disease (HSCR) patients. We investigated AFD in an HSCR population and controls and consider the pathogenesis and significance of the vascular pathology. Design Vascular histology in sections from colonic HSCR resections (n = 55) was compared with age- and site-matched controls with (n = 19) and without (n = 28) non-HSCR obstructive conditions. Vascular pathology was mapped, and correlations were sought between the vascular findings and bowel distension, inflammation, neuromuscular anatomy, preoperative clinical variables, and postsurgical complications. Results One of 2 forms of AFD was identified in 42% (23/55) of the HSCR resections: the previously described "mature" form with adventitial bundles of differentiated smooth muscle cells (7/23, all submucosal) and a newly described "immature" AFD characterized by densely packed myofibroblasts in the arterial adventitia (16/23, 3 submucosal, 3 serosal, and 10 both). Adventitial inflammation and/or medial necrosis was present in the immature form (6/16). Mature submucosal AFD was present in 2/28 (7.1%) nonobstructive and 5/19 (26%) obstructive non-HSCR controls ( P = .10). Immature AFD was only found in less than 1-month-olds, and mature AFD only in older patients, including the 7 affected controls. AFD did not correlate with sex, syndromic status, length of the aganglionic segment, or postoperative complications. AFD was present in grossly dilated (17/23) and narrowed (10/23) regions and in the aganglionic (2/23), ganglionic (14/23), or both (7/23) segments. In several cases, AFD existed proximal to the histological transition zone. Conclusion AFD occurs in HSCR and other obstructive conditions but is significantly less common in the colons of patients with no history of dysmotility. The pathology likely progresses from a reversible accumulation of myofibroblasts in neonates to a stable population of mature smooth muscle cells. The distribution of vascular lesions does not correlate with neuropathological findings and suggests a nonspecific form of vascular injury, possibly related to bowel distension. AFD in HSCR resections has not been shown to be clinically significant and should not influence management.

Coronary Tortuosity: A Clue to the Diagnosis of Fibromuscular Dysplasia?

BACKGROUND: Fibromuscular dysplasia (FMD) is a systemic, nonatherosclerotic, noninflammatory vasculopathy that is often overlooked by clinicians. Clinical clues could help in selecting patients for further evaluation for the presence of FMD. Recently, it was observed that tortuosity of the coronary arteries is often present in patients with FMD-related abnormalities of the coronary artery. Therefore, we wondered if the presence of coronary tortuosity might provide a clinical clue to the diagnosis of extracoronary FMD. CASES: We describe 5 cases of FMD in whom diagnostic studies for FMD were initiated because of the presence of coronary tortuosity. FMD was found in all 5 patients in the renal and/or cervical arteries. CONCLUSIONS: Our 5 cases suggest that exertional chest pain in the presence of coronary tortuosity may be helpful in selecting patients for further evaluation for the presence of FMD. Further research should focus on the prevalence of FMD among patients with coronary tortuosity and whether the presence of additional clinical clues (such as the presence of hypertension at young age or pulsatile tinnitus) next to coronary tortuosity can predict the risk for FMD in individual patients.

Displasia fibromuscular: causa infrecuente de dolor abdominal / Fibromuscular dysplasia: unusual cause of abdominal pain

La disección espontánea de la arteria renal es una entidad rara, más frecuente en hombres. Es causa infrecuente de dolor abdominal2, por lo que el diagnóstico suele ser tardío. Se trata de un paciente de 45 años que presenta dolor súbito en fosa renal izquierda de 12 horas de evolución, sin hallazgos en las pruebas complementarias básicas (analítica, sedimento urinario y ecografía), por lo que se determina la realización de TAC abdominal, que demuestra áreas de infarto renal, y arteriografía urgente, con hallazgos sugerentes de Displasia Fibromuscular de arteria intrarrenal izquierda como primera posibilidad diagnóstica con disección focal parcialmente trombosada (AU)

Spontaneous dissection of the renal artery is a rare phenomenon, and is more common amongst men. It is not a frequent cause of abdominal pain2, which is why diagnosis is often late. The case under study is a 45 year old patient that presented sudden pain in the left renal fossa of 12 hours evolution, with no findings from the basic laboratory tests (lab testing, urinary sediment and ultrasound), an abdominal CAT was therefore carried out, which showed areas of renal infarction, as well as an emergency arteriogram, which gave findings of a possible Fibromuscular Dysplasia of the left intrarenal artery as the first diagnostic probability with a partially thrombosed focal dissection (AU)

Fibromuscular dysplasia: Advances in understanding and management.

Several key findings in recent years have reshaped our understanding of fibromuscular dysplasia (FMD), an uncommon nonatherosclerotic disease of medium-sized arteries that affects mainly women. While the true prevalence of this disease remains unknown, studies suggest that more people may be affected than previously reported. Better understanding of the clinical manifestations and natural history of FMD and advances in diagnostic imaging have altered the clinical approach to managing patients with this uncommon vascular disease. Although there are a multitude of unanswered questions regarding FMD, this review highlights recent insights and how this information has modified clinical care for those affected.

Isquemia mesentérica crónica / Chronic mesenteric ischemia

La isquemia mesentérica crónica (IMC) es una entidad poco frecuente. Se presenta con síntomas inespecíficos como dolor abdominal posprandial, miedo a comer, disminución de peso y diarrea. Los pacientes sintomáticos no tratados evolucionan hacia una desnutrición severa y muerte debido a las complicaciones sépticas de la isquemia mesentérica. La causa más frecuente es la aterosclerosis, aunque se han descrito otras causas como la displasia fibromuscular, traumatismos, disección, rotura de aneurisma mesentérico, poliarteritis nodosa y la enfermedad de Takayasu. El diagnóstico de IMC se basa en la sospecha clínica y el diagnóstico diferencial con otras causas de dolor abdominal. El ecodoppler es el estudio inicial de elección para la evaluación de la enfermedad arterial oclusiva del tronco celíaco (TC) y arteria mesentérica superior (AMS), con sensibilidad y especificidad superiores al 80% en equipos expertos. La arteriografía, sin embargo, es el estudio definitivo para detallar las lesiones y planificar la cirugía. Se ha descrito estenosis significativa en 2 vasos mesentéricos en el 91% de los pacientes y en 3 arterias en el 55%. La angiotomografía axial computarizada ha demostrado ser efectiva en describir la anatomía de los vasos digestivos. Además, es útil para detectar otras enfermedades abdominales, causas extravasculares compresivas y para el control de la permeabilidad después del tratamiento quirúrgico abierto o endovascular. Los objetivos del tratamiento son aliviar los síntomas de forma permanente, modificar los factores de riesgo cardiovascular, mejorar el estado nutricional y prevenir el infarto mesentérico, lo cual se logra mediante revascularización mesentérica (cirugía abierta o endovascular) y apoyo multidisciplinario

Chronic mesenteric ischemia (CMI) is a rare condition that presents with non-specific symptoms such as postprandial abdominal pain, fear of eating, weight loss and diarrhea. Untreated symptomatic patients progress to severe malnutrition, and death due to septic complications of acute mesenteric ischemia. The most common cause is atherosclerosis, although other causes have been described, such as fibromuscular dysplasia, trauma, dissection, ruptured mesenteric aneurysm, polyarteritis nodosa, and Takayasu's disease. The diagnosis of CMI is based on clinical suspicion and differential diagnosis of other causes of abdominal pain. Doppler ultrasound is the initial method of study for evaluation of arterial occlusive disease of the celiac trunk (CT) and superior mesenteric artery (SMA), with sensitivity and specificity greater than 80% by expert teams. Angiography is the definitive study to show the lesions and for surgical planning. Significant stenosis has been described in two mesenteric vessels in 91% of patients, and of three arteries in 55%. The computed tomography angiography has shown to be effective in describing the anatomy of the SMA and CT. In addition, it is useful to detect other abdominal conditions, extravascular compressive causes and monitoring of patency after open or endovascular surgery

Abordagem endovascular de paciente com fibrodisplasia de artéria renal bilateral associada a volumoso aneurisma renal / Endovascular approach of a patient with bilateral renal artery fibrodysplasia associated with a massive renal aneurysm

A fibrodisplasia muscular renal é de etiologia desconhecida, de origem não inflamatória e não aterosclerótica, estando associada ao desenvolvimento de estenoses e de aneurismas. Relatamos um caso de fibrodisplasia muscular renal bilateral associada a aneurisma volumoso de artéria renal tratado com angioplastia com balão e stent multicamadas.

Renal artery fibromuscular dysplasia is a condition of unknown etiology, with non-inflammatory, nonatheroscleroticorigin, associated to the development of stenosis and aneurysms. The authors report a case of bilateral renal artery fibromuscular dysplasia associated with a large renal artery aneurysm, treatedwith balloon angioplasty and a multilayer stent.

Possible familial presentation in two siblings with carotid fibromuscular dysplasia.

Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory vascular disease which can affect the cervical, renal and visceral arteries. Here we report on two sisters diagnosed with carotid FMD at the same age, presenting with similar symptoms of pulsating tinnitus. The familial presentation of this rare disorder attracted our attention and was suggestive of a genetic etiology. Conservative treatment with aspirin was initiated. Carotid FMD is a rare disorder of which the exact pathophysiology is not known. A review of the literature on the clinical presentation, diagnosis and management is presented. In addition possible etiological factors and the genetic nature of the disease are discussed.

[Surgical management of patients with pathological deformities of carotid arteries].

Surgical management of pathological deformities of the internal carotid arteries, a cause of chronic brain ischemia, is discussed. This pathology is very common and is found in 25% of all individuals who underwent preventive medical examination according to the ultrasonography data. Most deformities do not pose any threat to patients, while some of them may cause ischemic stroke and chronic brain ischemia. The study included 165 patients with the known follow-up history who had been operated on at the N.N. Burdenko Neurosurgical Institute since 2001. A total of 196 reconstructive interventions of carotid arteries were analyzed. The indications for surgical management of pathological deformities based on clinical symptoms and identification of the signs of vascular wall dysplasia are thoroughly discussed. The local and cerebral hemodynamics during pre- and postoperative period are analyzed. The results of pathomorphological examination of the resected fragments of the deformed arteries are presented; they show that the changes are identical to those in patients with fibromuscular dysplasia. The follow-up history of the patients was recorded; it showed a sustained regression of transitory ischemic strokes and cerebral symptoms in most cases (69%). For proper indications for surgical management, reconstructive surgical interventions are a reliable and effective method for treating chronic brain ischemia and preventing recurrent ischemic strokes in patients with deformities of carotid arteries.

Small bowel adenocarcinomas complicating Crohn's disease are associated with dysplasia: a pathological and molecular study.

BACKGROUND: Crohn's disease (CD) is associated with an increased risk of small bowel adenocarcinoma (SBA). However, there are no guidelines for the screening and early diagnosis of SBA. Colorectal cancer associated with chronic colitis arises from dysplasia. High-risk patients benefit from surveillance colonoscopies aimed to detect dysplasia. The dysplasia-carcinoma sequence remains poorly documented in CD-associated SBA. Moreover, molecular data about SBA complicating CD and associated dysplasia are very limited. We therefore assessed dysplasia and several key molecular markers of carcinogenesis in SBA and dysplasia developed in patients with CD. METHODS: Forty-five SBA complicating CD and 4 specimens with dysplasia without SBA were screened. In SBA, we looked for dysplasia and determined their pathological characteristics (type, grade, distribution). We also stained for mismatch repair proteins (MLH1, MSH2, MSH6, PMS2), p53, ß-catenin, and p16 and looked for KRAS, BRAF and PIK3CA mutations. RESULTS: All neoplastic lesions, except 1 lesion, were found in inflamed mucosal areas. Dysplasia was found in 20 of 41 patients with SBA (49%). Dysplasia was flat or raised, low grade or high grade, and adjacent or distant to concomitant SBA. Molecular markers of SBA carcinogenesis complicating CD were similar to those observed in chronic colitis-related colorectal cancer (KRAS, BRAF, p53, MSI), although differences were observed for ß-catenin and p16. No PIK3CA mutations were observed. CONCLUSIONS: These results suggest that there is an inflammation-dysplasia-adenocarcinoma sequence in at least half of CD-related SBA, similar to what is observed in chronic colitis-related colorectal cancer and may have implications for the prevention and treatment of this cancer.

Fibromuscular dysplasia unraveled: the pulsation-induced microtrauma and reactive hyperplasia theory.

INTRODUCTION: Fibromuscular dysplasia (FMD) is a relatively uncommon, non-inflammatory, non-atherosclerotic vascular condition of unknown etiology. There have been no major advancements in understanding FMD in the past 30-40yrs. We hypothesize that the vascular changes seen in FMD are a direct result of recurrent mechanical trauma in susceptible vessels and reflect a compensatory response to recurrent pulsation-induced mechanical trauma. METHODS: Radiology records at a large tertiary care medical center were queried for cases of cervical carotid FMD. Cases with viewable sequential angiographic evidence of FMD were selected and analyzed for degree of vessel tortuosity, FMD type, degree of vessel movement, and association with the FMD lesion. Images were independently analyzed by 1 neurologist and 2 neuroradiologists. RESULTS: Twenty-four carotid vessels in fourteen patients were identified to have FMD, 100% of patients were female, average age was 58.43±8.72yrs (range 45-73), 35.7% were black, 35.7% had HTN, 7.14% had CKD and 7.14% used tobacco in the past year. Associated findings included aneurysms and dissections. All but 1 patient had at least mild vessel tortuosity, and 28.6% were moderate or severe. All but 1 patient had at least "some" pulsation-like vessel movement, 16.7% had "significant" movement, and all movement was focally associated with the FMD abnormality. CONCLUSION: Pulsatile vascular movements were observed in high frequency and in high association with FMD. We propose a theoretical connection between the pulsation-induced movement and a process of reactive vascular hyperplasia and fibrosis responsible for the FMD phenotype and is consistent with previous findings.

The United States registry for fibromuscular dysplasia: new findings and breaking myths.

Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory arterial disease predominantly seen in women. Most FMD cases are classified as medial fibroplasia, which has the appearance of a string of beads on angiography. Until recently, FMD was thought to be seen in women in their 20s and 30s, with more than 60%-75% of cases occurring in the renal artery and 25% of cases in the carotid artery. Hypertension was regarded as the primary symptom seen in these patients followed by a small number of patients presenting with intracranial aneurysms with subarachnoid hemorrhage. The United States Registry of FMD, a patient registry, has broken many preconceived notions as well as provided more in-depth knowledge of this uncommon disorder. In this review, we discuss the findings of this registry and its use in understanding this disorder. In addition to the registry, we review other recent studies and future directions in the diagnosis and management of this disorder.

Renal artery stenosis due to fibromuscular dysplasia in a transplanted kidney from a deceased donor: a difficult diagnosis at color Doppler ultrasonography.

Atherosclerotic renal artery stenosis is a frequent cause of arterial hypertension and/or allograft dysfunction after kidney transplantation and is usually located at the iliac artery anastomosis. Fibromuscular dysplasia is a less frequent, nonatherosclerotic, vascular disease, inducing stenosis at the proximal/mid-distal part of the renal artery. We report the case of a 44-year-old woman, in whom serum creatinine concentration increased and arterial hypertension developed 3 months after renal transplantation. Color Doppler ultrasonography showed a low arterial resistance index and prolonged acceleration time in the interlobar arteries, and a significantly increased peak systolic velocity at the mid third of the renal artery, demonstrating hemodynamically significant stenosis. Percutaneous transluminal angioplasty allowed stenosis correction and was followed by creatinine concentration and arterial blood pressure normalization.

Evaluation of characteristics, associations and clinical course of isolated spontaneous renal artery dissection.

BACKGROUND: Spontaneous renal artery dissection (SRAD) is a rare entity of unknown etiology. We aimed to study the clinical course and outcomes and compare the characteristics of patients with SRAD with those of the general population. METHODS: All cases of isolated renal artery dissection diagnosed at the University of Michigan Hospitals between January 2000 and July 2012 were identified by the ICD-9 code. Cases were matched by age, gender and race with individuals from the 2009-2010 National Health and Nutrition Examination Survey (NHANES). Characteristics and awareness of comorbid conditions were compared. Information about the clinical course after diagnosis was retrieved from the case group to ascertain their outcomes. RESULTS: Overall, 17 patients with SRAD with a mean age of 38.6 years (SD = 8.3) were identified. Eleven patients were male and 14 were white. The most common presenting symptom was excruciating sudden-onset flank pain ipsilateral to the site of dissection. Fibromuscular dysplasia, Ehlers-Danlos and polyarteritis nodosa were present in 4, 4 and 1 patients, respectively. After adjusting in a multivariable model, the case group was more likely to report history of hypertension, cancer and connective tissue disorders (P < 0.001), and less likely to have obesity (BMI ≥30 kg/m(2)) compared with the general population. Supportive medical treatment, endovascular intervention and surgery were required in 8, 5 and 4 cases, respectively. After discharge from the hospital, hypertension was adequately controlled in all the patients but one. CONCLUSION: SRAD may be part of a syndrome having multi-organ involvement. With appropriate medical or surgical management, long-term clinical outcome appears favorable.