SP059FIBROMUSCULAR DYSPLASIA: 40 YEARS OF EXPERIENCE IN A TERTIARY REFERRAL HOSPITAL

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INTRODUCTION: Fibromuscular dysplasia (FMD) is evolving from a rare disease of renal arteries that causes secondary hypertension in young women to a systemic vascular disease affecting renal but also other arteries. Hypertension is the most common clinical presentation of FMD. Recent works suggest a genetic susceptibility and genetic studies will soon lead to a better understanding of the disease. The aim of our study was to describe the characteristics and outcomes of the patients diagnosed with FMD and followed in our center during the years 1979-2019. METHODS: Retrospective review of medical records of patients diagnosed with FMD and followed in our center during the years 1979-2019. The following variables were gathered at diagnosis: clinical and radiologic findings, number of antihypertensive drugs, creatinine and type and number of interventions on renal or other arteries. Creatinine and number of antihypertensive drugs were also gathered at last follow-up. Median follow-up was 164 (22-306) months. RESULTS: 41 patients (63.4% female) were analysed, with a mean age of 37,5± 17,6 years. 41% were smokers or ex-smokers at diagnosis and only one patient had a positive family history for FMD. 8% of women had a history of oral contraception at diagnosis and 8% had a spontaneous abortion during follow-up. 97% of the patients were diagnosed during the study of hypertension and 3 patients had neurological symptoms related to FMD (subarachnoid hemorrhage, transient ischemic attack or stroke). No spontaneous coronary artery dissection was found in our cohort. The mean creatinine value at diagnosis was 1.13± 0.56 mg/dL and the mean number of antihypertensive drugs was 2.2. In the angiography, stenosis was found in 17% and 34% of all cases for left and right renal artery, respectively. In 34% patients, stenosis was bilateral. 44% of the stenosis were multifocal and 6 patients had an associated aneurysm. Stenotic lesions in abdominal arteries were found incidentally during arteriography in 2 patients. Intervention on renal arteries was done in 85% of the patients (54% angioplasty, 20% angioplasty + stenting, 26% surgery). 24% of the cohort required a second intervention, 12% a third and in only 7% a fourth intervention was done. Regarding to the surgical cases, 4 autotransplants were done, 2 bypass, 1 nephrectomy and 6 aneurysm resections. Mean creatinine at last follow-up was 1.03 ± 0.46 mg/dLand mean number of antihypertensive drugs was 1.4. CONCLUSIONS: FMD is usually diagnosed in young women but a significant percentage can be diagnosed in men and beyond the fourth decade. Smoking cessation must be encouraged and close follow-up with directed anamnesis is recommended in order to perceive alarm symptoms and promptly detect abnormalities of other vascular beds.

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