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纤维肌性发育不良的临床表现和诊断

Authors
David P Slovut, MD, PhD
Jeffrey W Olin, DO
Section Editor
George L Bakris, MD
Deputy Editor
John P Forman, MD, MSc
Translators
陈峰, 主治医师

引言

纤维肌性发育不良(fibromuscular dysplasia, FMD)是一种非炎症性、非动脉粥样硬化性疾病，可导致动脉狭窄、闭塞、动脉瘤和动脉夹层形成。几乎在每一个动脉床中均有发现。最常累及的动脉是肾动脉和颈内动脉，较少见的是椎动脉、髂动脉、锁骨下动脉及内脏动脉。

根据受累的动脉节段和该病的严重程度，其表现可能有较大变化。常见的表现是高血压、短暂性脑缺血发作、脑卒中、头痛、头晕、耳鸣和搏动性耳鸣(耳内异常哗哗声响)[1]。

成人FMD的临床表现和诊断将总结在此。肾性FMD的治疗和对疑诊肾血管性高血压及脑卒中的一般评估将单独讨论。 (参见“肾动脉纤维肌性发育不良的治疗”和“诊断肾血管性高血压”和“哪些患者应评估肾血管性或其他原因的继发性高血压？”和“脑卒中亚型的临床诊断”和“脑卒中评估概述”)

流行病学

FMD患者有70%的机会累及肾动脉，大约65%的机会累及颅外脑动脉(如颈动脉)[2]。大约2/3的患者有多处动脉受累[3,4]。

成年中，FMD更常见于女性。在大多数大型病例系列研究中，85%-90%的病例是女性。在儿童中，似乎女性发病并不较多[5]。

               

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Literature review current through: 2017-06 . | This topic last updated: 2015-06-03.
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References
Top
  1. Olin JW, Pierce M. Contemporary management of fibromuscular dysplasia. Curr Opin Cardiol 2008; 23:527.
  2. Olin JW, Froehlich J, Gu X, et al. The United States Registry for Fibromuscular Dysplasia: results in the first 447 patients. Circulation 2012; 125:3182.
  3. Lüscher TF, Keller HM, Imhof HG, et al. Fibromuscular hyperplasia: extension of the disease and therapeutic outcome. Results of the University Hospital Zurich Cooperative Study on Fibromuscular Hyperplasia. Nephron 1986; 44 Suppl 1:109.
  4. Olin JW, Sealove BA. Diagnosis, management, and future developments of fibromuscular dysplasia. J Vasc Surg 2011; 53:826.
  5. Estepa R, Gallego N, Orte L, et al. Renovascular hypertension in children. Scand J Urol Nephrol 2001; 35:388.
  6. Chiche L, Bahnini A, Koskas F, Kieffer E. Occlusive fibromuscular disease of arteries supplying the brain: results of surgical treatment. Ann Vasc Surg 1997; 11:496.
  7. Deal JE, Snell MF, Barratt TM, Dillon MJ. Renovascular disease in childhood. J Pediatr 1992; 121:378.
  8. Piercy KT, Hundley JC, Stafford JM, et al. Renovascular disease in children and adolescents. J Vasc Surg 2005; 41:973.
  9. Kløw NE, Paulsen D, Vatne K, et al. Percutaneous transluminal renal artery angioplasty using the coaxial technique. Ten years of experience from 591 procedures in 419 patients. Acta Radiol 1998; 39:594.
  10. Krijnen P, van Jaarsveld BC, Steyerberg EW, et al. A clinical prediction rule for renal artery stenosis. Ann Intern Med 1998; 129:705.
  11. Pascual A, Bush HS, Copley JB. Renal fibromuscular dysplasia in elderly persons. Am J Kidney Dis 2005; 45:e63.
  12. Plouin PF, Perdu J, La Batide-Alanore A, et al. Fibromuscular dysplasia. Orphanet J Rare Dis 2007; 2:28.
  13. Mettinger KL. Fibromuscular dysplasia and the brain. II. Current concept of the disease. Stroke 1982; 13:53.
  14. Mettinger KL, Ericson K. Fibromuscular dysplasia and the brain. I. Observations on angiographic, clinical and genetic characteristics. Stroke 1982; 13:46.
  15. Cloft HJ, Kallmes DF, Kallmes MH, et al. Prevalence of cerebral aneurysms in patients with fibromuscular dysplasia: a reassessment. J Neurosurg 1998; 88:436.
  16. Wesen CA, Elliott BM. Fibromuscular dysplasia of the carotid arteries. Am J Surg 1986; 151:448.
  17. Schievink WI, Björnsson J. Fibromuscular dysplasia of the internal carotid artery: a clinicopathological study. Clin Neuropathol 1996; 15:2.
  18. Slovut DP, Olin JW. Fibromuscular dysplasia. N Engl J Med 2004; 350:1862.
  19. Sauer L, Reilly LM, Goldstone J, et al. Clinical spectrum of symptomatic external iliac fibromuscular dysplasia. J Vasc Surg 1990; 12:488.
  20. Kolluri R, Ansel G. Fibromuscular dysplasia of bilateral brachial arteries--a case report and literature review. Angiology 2004; 55:685.
  21. Begelman SM, Olin JW. Fibromuscular dysplasia. Curr Opin Rheumatol 2000; 12:41.
  22. Stanley JC, Gewertz BL, Bove EL, et al. Arterial fibrodysplasia. Histopathologic character and current etiologic concepts. Arch Surg 1975; 110:561.
  23. Harrison EG Jr, McCormack LJ. Pathologic classification of renal arterial disease in renovascular hypertension. Mayo Clin Proc 1971; 46:161.
  24. Perdu J, Boutouyrie P, Bourgain C, et al. Inheritance of arterial lesions in renal fibromuscular dysplasia. J Hum Hypertens 2007; 21:393.
  25. Lüscher TF, Lie JT, Stanson AW, et al. Arterial fibromuscular dysplasia. Mayo Clin Proc 1987; 62:931.
  26. Dziewas R, Konrad C, Dräger B, et al. Cervical artery dissection--clinical features, risk factors, therapy and outcome in 126 patients. J Neurol 2003; 250:1179.
  27. Schievink WI, Björnsson J, Parisi JE, Prakash UB. Arterial fibromuscular dysplasia associated with severe alpha 1-antitrypsin deficiency. Mayo Clin Proc 1994; 69:1040.
  28. Siegert CE, Macfarlane JD, Hollander AM, van Kemenade F. Systemic fibromuscular dysplasia masquerading as polyarteritis nodosa. Nephrol Dial Transplant 1996; 11:1356.
  29. Kim ES, Olin JW, Froehlich JB, et al. Clinical manifestations of fibromuscular dysplasia vary by patient sex: a report of the United States registry for fibromuscular dysplasia. J Am Coll Cardiol 2013; 62:2026.
  30. So EL, Toole JF, Dalal P, Moody DM. Cephalic fibromuscular dysplasia in 32 patients: clinical findings and radiologic features. Arch Neurol 1981; 38:619.
  31. Honjo O, Yamada Y, Kuroko Y, et al. Spontaneous dissection and rupture of common iliac artery in a patient with fibromuscular dysplasia: a case report and review of the literature on iliac artery dissections secondary to fibromuscular dysplasia. J Vasc Surg 2004; 40:1032.
  32. Guill CK, Benavides DC, Rees C, et al. Fatal mesenteric fibromuscular dysplasia: a case report and review of the literature. Arch Intern Med 2004; 164:1148.
  33. Olin JW, Piedmonte MR, Young JR, et al. The utility of duplex ultrasound scanning of the renal arteries for diagnosing significant renal artery stenosis. Ann Intern Med 1995; 122:833.
  34. Vasbinder GB, Nelemans PJ, Kessels AG, et al. Accuracy of computed tomographic angiography and magnetic resonance angiography for diagnosing renal artery stenosis. Ann Intern Med 2004; 141:674.
  35. Gowda MS, Loeb AL, Crouse LJ, Kramer PH. Complementary roles of color-flow duplex imaging and intravascular ultrasound in the diagnosis of renal artery fibromuscular dysplasia: should renal arteriography serve as the "gold standard"? J Am Coll Cardiol 2003; 41:1305.
  36. Carman TL, Olin JW, Czum J. Noninvasive imaging of the renal arteries. Urol Clin North Am 2001; 28:815.
  37. Leung DA, Hoffmann U, Pfammatter T, et al. Magnetic resonance angiography versus duplex sonography for diagnosing renovascular disease. Hypertension 1999; 33:726.
  38. Malatino LS, Polizzi G, Garozzo M, et al. Diagnosis of renovascular disease by extra- and intrarenal Doppler parameters. Angiology 1998; 49:707.
  39. Sabharwal R, Vladica P, Coleman P. Multidetector spiral CT renal angiography in the diagnosis of renal artery fibromuscular dysplasia. Eur J Radiol 2007; 61:520.
  40. Funabashi N, Komiyama N, Komuro I. Fibromuscular dysplasia in renovascular hypertension demonstrated by multislice CT: comparison with conventional angiogram and intravascular ultrasound. Heart 2003; 89:639.
  41. Fink C, Hallscheidt PJ, Hosch WP, et al. Preoperative evaluation of living renal donors: value of contrast-enhanced 3D magnetic resonance angiography and comparison of three rendering algorithms. Eur Radiol 2003; 13:794.
  42. Mounier-Vehier C, Lions C, Jaboureck O, et al. Parenchymal consequences of fibromuscular dysplasia renal artery stenosis. Am J Kidney Dis 2002; 40:1138.
  43. Mounier-Véhier C, Haulon S, Devos P, et al. Renal atrophy outcome after revascularization in fibromuscular dysplasia disease. J Endovasc Ther 2002; 9:605.