Dissecting visceral fibromuscular dysplasia reveals a new vascular phenotype of the disease: a report from the ARCADIA-POL study.
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Warchoł-Celińska Ewa, Pieluszczak Krzysztof, Pappaccogli Marco, Soplińska Aleksandra, Prejbisz Aleksander, Dobrowolski Piotr, Klisiewicz Anna, Kądziela Jacek, Falkowski Aleksander, Śmigielski Witold, Florczak Elżbieta, Jóźwik-Plebanek Katarzyna, Michałowska Ilona, Kabat Marek, Zgorzelski Cezary, Madej Krzysztof, Nazarewski Sławomir, Smólski Mikołaj, Olewnik Łukasz, Litwin Mieczysław, Szczerbo-Trojanowska Małgorzata, Zieniewicz Krzysztof, Drygas Wojciech, Rowiński Olgierd, Witkowski Adam, Adlam David, Van der Niepen Patricia, Persu Alexandre, Januszewicz Andrzej, Januszewicz Magdalena
Аннотация
METHODS: A total of 232 FMD patients enrolled into ongoing ARCADIA-POL study were included in this analysis. All patients underwent detailed clinical evaluation including ambulatory blood pressure monitoring, biobanking, duplex Doppler of carotid and abdominal arteries and whole body angio-computed tomography. Three control groups (patients with renal FMD without visceral involvement, healthy normotensive patients and resistant hypertensive patients) matched for age and sex were included.
RESULTS: VA FMD was present in 32 patients (13.8%). Among these patients (women: 84.4%), FMD lesions were more frequent in celiac trunk (83.1%), 62.5% of patients showed at least one visceral aneurysm, and five patients presented with severe complications related to VA FMD. No demographic differences were found between patients with VA FMD and individuals from the three control groups, with the exception of lower weight (P < 0.001) and BMI (P < 0.001) in VA FMD patients. Patients with FMD (with or without visceral artery involvement) showed significantly smaller visceral arterial diameters compared with controls without FMD.
CONCLUSION: Patients with FMD showed smaller visceral arterial diameters when compared with patients without FMD. This may reflect a new phenotype of FMD, as a generalized arteriopathy, what needs further investigation. Lower BMI in patients with VA FMD might be explained by chronic mesenteric ischemia resulting from FMD lesions. FMD visceral involvement and visceral arterial aneurysms in patients with renal FMD are far to be rare. This strengthens the need for a systematic evaluation of all vascular beds, including visceral arteries, regardless of initial FMD involvement.