Systematic and Multidisciplinary Evaluation of Fibromuscular Dysplasia Patients Reveals High Prevalence of Previously Undetected Fibromuscular Dysplasia Lesions and Affects Clinical Decisions

Author:

Warchol-Celinska Ewa1,Prejbisz Aleksander1,Dobrowolski Piotr1,Klisiewicz Anna2,Kadziela Jacek3,Florczak Elzbieta1,Michalowska Ilona4,Jozwik-Plebanek Katarzyna1,Kabat Marek1,Kwiatek Pawel4,Nazarewski Slawomir5,Madej Krzysztof5,Rowinski Olgierd6,Swiatlowski Lukasz7,Peczkowska Mariola1,Hanus Katarzyna1,Talarowska Paulina1,Smolski Mikołaj1,Kowalczyk Katarzyna1,Kurkowska-Jastrzebska Iwona8,Stefanczyk Ludomir9,Wiecek Andrzej3,Widecka Krystyna10,Tykarski Andrzej11,Stryczynski Lukasz11,Litwin Mieczyslaw12,Hoffman Piotr2,Witkowski Adam13,Szczerbo-Trojanowska Malgorzata7,Januszewicz Magdalena6,Januszewicz Andrzej1

Affiliation:

1. From the Departments of Hypertension (E.W.-C., A.P., P.D., E.F., K.J.-P., M.K., M.P., K.H., P.T., M.S., K.K., A.J.), Institute of Cardiology, Warsaw, Poland

2. Congenital Heart Diseases (A.K., P.H.), Institute of Cardiology, Warsaw, Poland

3. Interventional Cardiology and Angiology (J.K., A.W.), Institute of Cardiology, Warsaw, Poland

4. Radiology (I.M., P.K.), Institute of Cardiology, Warsaw, Poland

5. Department of General, Vascular and Transplant Surgery (S.N., K.M.), Medical University of Warsaw, Poland

6. 2nd Department of Radiology (O.R., M.J.), Medical University of Warsaw, Poland

7. Department of Interventional Radiology and Neuroradiology, Medical University of Lublin, Poland (L.S., M.S.-T.)

8. 2nd Department of Neurology, Institute of Psychiatry and Neurology, Warsaw, Poland (I.K.-J.)

9. Department of Radiology and Imaging Diagnostics, Medical University of Lodz, Poland (L.S.)

10. Department of Hypertensiology and Internal Medicine, Pomeranian Medical University in Szczecin, Poland (K.W.)

11. Department of Internal Medicine Metabolic Disorders and Hypertension, Poznan University of Medical Science, Poland (A.T., L.S.)

12. Department of Nephrology and Arterial Hypertension, The Children’s Memorial Health Institute in Warsaw, Poland (M.L.).

13. Department of Nephrology, Tranplantology and Internal Medecine, Medical University of Silesia, Katowice, Poland (A.W.)

Abstract

Fibromuscular dysplasia (FMD), regarded as a generalized vascular disease, may affect all vascular beds and may result in arterial stenosis, occlusion, aneurysm, or dissection. It has been proposed to systematically evaluate all vascular beds in patients with FMD, regardless of initial FMD involvement. However, the impact of this approach on clinical decisions and on management is unknown. Within the prospective ARCADIA-POL study (Assessment of Renal and Cervical Artery Dysplasia–Poland), we evaluated 232 patients with FMD lesions confirmed in at least one vascular bed, out of 343 patients included in the registry. All patients underwent a detailed clinical evaluation including computed tomography angiography of intracranial and cervical arteries, as well as computed tomography angiography of the abdominal aorta, its branches, and upper and lower extremity arteries. In the study group, FMD lesions were most frequently found in renal arteries (87.5%). FMD was also found in cerebrovascular (24.6%), mesenteric (13.8%), and upper (3.0%) and lower extremity (9.9 %) arteries. Newly diagnosed FMD lesions were found in 34.1% of the patients, and previously undetected vascular complications were found in 25% of the patients. Among all FMD patients included in the study, one out of every 4 evaluated patients qualified for interventional treatment due to newly diagnosed FMD lesions or vascular complications. The ARCADIA-POL study shows for the first time that the systematic and multidisciplinary evaluation of patients with FMD based on a whole-body computed tomography angiography scan has an impact on their clinical management. This proved the necessity of the systematic evaluation of all vascular beds in patients with FMD, regardless of initial FMD involvement.

Publisher

Ovid Technologies (Wolters Kluwer Health)

Subject

Internal Medicine

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