Article

Fibromuscular dysplasia (FMD) is a noninflammatory nonatherosclerotic
disease of small- to medium-sized arteries. The frequency of multisite
involvement and its influence on prognosis has not been systematically assessed
in patients with cervicocephalic FMD, and little is known about their mid-term
clinical and arterial prognosis. The aim of our study was to assess the
prevalence of renal involvement and clinical and arterial prognosis in patients
with cervicocephalic FMD. METHODS: We reviewed clinical and radiological data of
consecutive patients with a diagnosis of cervicocephalic FMD, admitted to our
hospital between January 2000 and March 2010. Patients were identified
retrospectively until December 2008, and prospectively from January 2009. For
each cervical and intracranial artery, we recorded the presence and type
(unifocal or multifocal) of FMD. We classified each FMD-related stenosis into
four categories:<50%, 50-80%,>80% and occlusion. During the first six months of
2012, patients were scheduled for follow-up visit, including cervicocephalic
follow-up imaging, and renal artery imaging, if not already available. On
follow-up imaging, FMD-related stenosis was classified according to the same
method used at baseline. Renal artery FMD was defined as the presence of the
typical string of beads appearance, or as the presence of a unique stenosis of
renal artery. Primary endpoints were stroke (ischemic or hemorrhagic), death, and
progression of FMD lesions, defined by any increase in category of stenosis on
follow-up imaging. RESULTS: Out of the 36 patients included (32 women), all with
carotid artery involvement and 17 with associated vertebral artery involvement,
28 (78%) had ischemic symptoms and/or cervical artery dissection at the time of
the diagnosis of FMD. Among the 30 patients who had renal artery imaging, 13
(43%) had renal FMD. Patients with renal artery disease did not differ from those
without renal artery disease. After a median follow-up of 3.5years, three
patients had four strokes, one recurrent cervical dissection, one brain
hemorrhage, and one fatal cardiac arrhythmia. Among the 31 patients who had
follow-up imaging, two showed progression of cervicocephalic FMD (occlusion of
carotid artery). Patients with renal involvement showed a non-significant trend
toward a higher rate of stroke (P=0.17). CONCLUSIONS: In patients with
cervicocephalic FMD, renal involvement is common. The risk of stroke, death or
FMD progression was high in our cohort, suggesting that prognosis may not be as
good as expected. This underlines the need for larger prospective studies to
define the best treatment options.
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Langue : ANGLAIS