| Abstract | Fibromuskularna displazija (FMD) je idiopatska, neupalna i neaterosklerotska vaskularna bolest koja može rezultirati stenozom, okluzijom, aneurizmom ili disekcijom, a najčešće zahvaća bubrežne arterije. Cilj ovog rada bio je prikazati karakteristike pacijenata s dijagnozom FMD-a, koji su uključeni u hrvatsku kohortu europskog FMD registra. Također, htjeli smo ispitati postoje li razlike naše kohorte u odnosu na postojeće registre. U ovo istraživanje uključeno je 22 ispitanika koji su svi pacijenti KBC-a Zagreb, a dijagnoza FMD-a postavljena im je patohistološki i/ili angiografski. Svi ispitanici su bili bijele rase, 59,1% su bile žene, a 54,5% ispitanika pušači. Najčešća klinička manifestacija bila je arterijska hipertenzija s medijanom dobi od 36,5 godina kod postavljanja dijagnoze. Medijan dobi prilikom postavljanja dijagnoze FMD-a bio je 41,5 godina što upućuje na prosječni pomak od 5 godina u postavljanju dijagnoze FMD-a od pojave prvih znakova i simptoma bolesti. Teže i ranije kliničke manifestacije, uključujući neurološke, zamijećene su u muškaraca. Svi ispitanici imali su bolest bubrežnih arterija, a u 31,8% bile su zahvaćene i cerebralne arterije. Unilateralna bolest bubrežnih arterija dijagnosticirana je u 77,3% ispitanika, od čega je u 70,6% bila desnostrana. U 63,6% ispitanika tip lezije bio je fokalni i nije bilo značajne razlike u spolu, unilateralnoj zahvaćenosti bubrežne arterije te tipu interevencije (P>0,05). Medijan eGFR-a svih ispitanika iznosio je 91,5 mL/min/1,73m2. Više vrijednosti eGFR-a zabilježene su u skupini fokalnog tipa bolesti. Najčešća intervencija bila je PTA s balon kateterom. Medijan broja antihipertenziva
unatoč intervenciji bio je 3. Možemo zaključiti kako je više žena nego muškaraca imalo FMD, ali teže manifestacije i više intervencija je zabilježeno kod muškaraca. Pri tumačenju rezultata ovog istraživanja treba uzeti u obzir činjenicu da je naš centar referentni centar za hipertenziju te u njemu postoji velika koncentracija pacijenata oboljelih od FMD-a sa zahvaćenom bubrežnom arterijom i posljedičnom renovaskularnom hipertenzijom. Daljnje analize su potrebne za bolje razumijevanje ove bolesti. |
| Abstract (english) | Fibromuscular dysplasia is an idiopathic, noninflammatory and nonatherosclerotic vascular disease that can result in stenosis, occlusion, aneurysm or dissection and most commonly affects renal arteries. Our goal was to report characteristics of patients diagnosed with fibromuscular dysplasia who were included in the Croatian registry, a cohort for the European FMD registry. Also, we wanted to see if there are any differences between our cohort and the already existing
registries. The registry included 22 patients from University Hospital Centre Zagreb in whom the diagnose of FMD was made by histopathology and/or angiography. All patients were Caucasian, 59.1% of them being women and 54.5% of them being smokers. The median age at diagnosis of hypertension, which was also the most common clinical manifestation, was 36.5 years. The median of age at diagnosis of FMD was 41.5 years, indicating that the average delay from the time of first symptom or sign to diagnosis of FMD was 5 years. Worse and earlier clinical manifestations including neurological ones, were observed in men. All patients had renal artery disease and in 31.8% of cases cerebral arteries were affected. Unilateral disease of the renal arteries was diagnosed in 77.3% of cases and in 70.6% of patients it was right-sided. 63.6% of the patients had a focal type of the disease and there was no significant difference in sex, unilateral involvement of the renal artery and type of intervention (P-value >0.05). The median eGFR was 91.5 mL/min/1.73m2. Additionally, higher values of eGFR were noticed in the focal type of disease. The most common intervention was PTA with the balloon catheter
(28.6%). The median of antihypertensive drugs used was 3 despite intervention and only two patients weren't on antihypertensive therapy. In conclusion, more women than men had FMD, but more severe clinical manifestations and more interventions were registered in men. When interpreting this study, potential bias should be considered because our center is a hypertension referral center and there is a big concentration of the patients with FMD, with renal artery involvement and consequent renovascular hypertension. Further analysis is needed for a better understanding of FMD. |
