Fibromuscular Hyperplasia: Extension of the Disease and Therapeutic Outcome

Lüscher, Thomas F.; Keller, H.; Imhof, H; Greminger, P; Kuhlmann, Ulrich; Largiadèr, F; Schneider, Ernst; Schneider, Jakob; Vetter, W

doi:10.1159/000184059

Cited by 101 publications

(16 citation statements)

References 15 publications

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“…FMD is very often seen affecting the renal arteries, 60%–75% cases. [1] The prevalence of FMD is 4.4%–6.6%, most commonly in females. [1] It is most commonly seen in patients younger than 50 years of age.…”

Section: Discussionmentioning

confidence: 99%

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Surgical revascularization of bilateral renal artery stenosis due to fibromuscular dysplasia

et al. 2017

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Fibromuscular dysplasia (FMD) is a noninflammatory disease affecting small- and medium-sized arteries of the renal and the carotids. It affects the renal arteries in nearly 60%–75% cases. The primary clinical manifestation of renal FMD is hypertension. Medial fibroplasia represents the most common dysplastic lesion. We report two cases who presented with hypertension and renal insufficiency and on evaluation was found to have bilateral renal artery stenosis. Stenting of the renal vessels was not possible due to the narrowed caliber of the vessel and inability to cannulate the renal arteries. They underwent renal artery revascularization with a splenorenal end to end anastomosis. The renal parameters and blood pressure of both the patients stabilized subsequently. Renal revascularization can be a good option for patient having failed angioplasty with stenting.

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Section: Discussionmentioning

confidence: 99%

“…[1] The prevalence of FMD is 4.4%–6.6%, most commonly in females. [1] It is most commonly seen in patients younger than 50 years of age. Medial fibroplasia represents the most common dysplastic lesion.…”

Section: Discussionmentioning

confidence: 99%

Surgical revascularization of bilateral renal artery stenosis due to fibromuscular dysplasia

et al. 2017

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“…1 FMD, however, typically presents in young white women, whereas PAN characteristically presents in men in the fifth decade of life. Arterial narrowing and aneurysms in FMD reflect abnormal deposition of collagen and smooth muscle hyperplasia in contrast with cellular infiltration, fibrinoid necrosis, and healing elastic lamina in PAN.…”

Section: Discussionmentioning

confidence: 99%

A rare case of shock

Felton

Drewe²,

Jivan³

et al. 2003

Annals of the Rheumatic Diseases

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A 48 year old non-smoking women presented with a six hour history of colicky right upper quadrant pain radiating to the back preceded by an episode of syncope. Past history included hyperthyroidism treated by partial thyroidectomy.Initial examination showed mild right upper quadrant tenderness. Investigations demonstrated a normocytic anaemia (haemoglobin 92 g/l), white cell count 15.3×l0 9 /l, with normal liver function tests and amylase. Twelve hours later she developed shock associated with a distending abdomen.Emergency laparotomy showed a haemoperitoneum and huge retroperitoneal haematoma with catastrophic bleeding from the duodenojejunal region requiring 13 units of blood and 8 litres of colloid and crystalloid. Haemostasis was achieved with aortic cross suturing around a branch of the superior mesenteric artery (SMA) and packing the peritoneum. Packs were removed 24 hours later without complication.A postoperative mesenteric angiogram delineated multiple small aneurysms in the gastroduodenal artery and its pancreatic branches, hepatic artery, and ileal branches of the SMA with microaneurysms of the main renal, lobar, and segmental arteries (fig 1). Differential diagnosis included polyarteritis nodosa (PAN) and fibromuscular dysplasia (FMD).Further investigations, including erythrocyte sedimentation rate, C reactive protein, autoantibody screen, antineutrophil cytoplasmic antibodies, rheumatoid factor, and cardiolipin antibodies, were unremarkable. Hepatitis B and C and syphilis serology were negative. α 1 Antitrypsin levels, fundoscopy, urine microscopy, echocardiography, and muscle biopsies were also normal.Subsequent magnetic resonance angiography (MRA) with gadolinium enhancement was performed to determine the extent of disease and identify vessels amenable for biopsy. Intracranial and superficial femoral vessels appeared unaffected. Mesenteric images demonstrated mild irregularities of the SMA and the suspicion of a splenic artery aneurysm only. Biopsy was not performed.Further evaluation of her family history showed that her father and paternal uncle had both died in the fifth decade after a second myocardial infarction and a cerebrovascular accident, respectively.Fibromuscular dysplasia was considered to be the diagnosis. The patient has remained well during three years' follow up without immunosuppression. Tachycardia and hypertension (recorded during waking hours on 24 hour monitoring) was controlled with propanolol.

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“…It is a well-recognized cause of hypertension in young Caucasian women when the renal arteries are involved [8]. However, other vascular beds may also be involved in 28% of patients with FMD [9]. Dysplastic lesions of the cerebrovascular, visceral, iliac, and brachial arteries have been described.…”

Section: Discussionmentioning

confidence: 99%

“…strated that FMD is a progressive disease [12]. Treatment of fibromuscular dysplastic lesions is only recommended in symptomatic cases and is either surgical or by percutaneous transluminal angioplasty [9,13].…”

Section: Discussionmentioning

confidence: 99%