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Renal artery stenosis

MedGen UID:
19727
Concept ID:
C0035067
Disease or Syndrome
Synonyms: Renal Artery Stenoses; Renal Artery Stenosis; Stenoses, Renal Artery; Stenosis, Renal Artery
SNOMED CT: Renal artery stenosis of unknown cause (282664001); RAS - Renal artery stenosis (302233006); Renal artery stenosis (302233006)
 
HPO: HP:0001920

Definition

The presence of stenosis of the renal artery. [from HPO]

Term Hierarchy

Conditions with this feature

Neurofibromatosis, type 1
MedGen UID:
18013
Concept ID:
C0027831
Neoplastic Process
Neurofibromatosis 1 (NF1) is a multisystem disorder characterized by multiple café au lait macules, intertriginous freckling, multiple cutaneous neurofibromas, and learning disability or behavior problems. About half of people with NF1 have plexiform neurofibromas, but most are internal and not suspected clinically. Plexiform neurofibromas can cause pain, neurologic deficits, and abnormalities of involved or adjacent structures. Less common but potentially more serious manifestations include optic nerve and other central nervous system gliomas, malignant peripheral nerve sheath tumors, scoliosis, tibial dysplasia, vasculopathy, and gastrointestinal, endocrine, or pulmonary disease.
Pheochromocytoma
MedGen UID:
18419
Concept ID:
C0031511
Neoplastic Process
Hereditary paraganglioma-pheochromocytoma (PGL/PCC) syndromes are characterized by paragangliomas (tumors that arise from neuroendocrine tissues distributed along the paravertebral axis from the base of the skull to the pelvis) and pheochromocytomas (paragangliomas that are confined to the adrenal medulla). Sympathetic paragangliomas cause catecholamine excess; parasympathetic paragangliomas are most often nonsecretory. Extra-adrenal parasympathetic paragangliomas are located predominantly in the skull base and neck (referred to as head and neck PGL [HNPGL]) and sometimes in the upper mediastinum; approximately 95% of such tumors are nonsecretory. In contrast, sympathetic extra-adrenal paragangliomas are generally confined to the lower mediastinum, abdomen, and pelvis, and are typically secretory. Pheochromocytomas, which arise from the adrenal medulla, typically lead to catecholamine excess. Symptoms of PGL/PCC result from either mass effects or catecholamine hypersecretion (e.g., sustained or paroxysmal elevations in blood pressure, headache, episodic profuse sweating, forceful palpitations, pallor, and apprehension or anxiety). The risk for developing metastatic disease is greater for extra-adrenal sympathetic paragangliomas than for pheochromocytomas.
Williams syndrome
MedGen UID:
59799
Concept ID:
C0175702
Disease or Syndrome
Williams syndrome (WS) is characterized by cardiovascular disease (elastin arteriopathy, peripheral pulmonary stenosis, supravalvar aortic stenosis, hypertension), distinctive facies, connective tissue abnormalities, intellectual disability (usually mild), a specific cognitive profile, unique personality characteristics, growth abnormalities, and endocrine abnormalities (hypercalcemia, hypercalciuria, hypothyroidism, and early puberty). Feeding difficulties often lead to poor weight gain in infancy. Hypotonia and hyperextensible joints can result in delayed attainment of motor milestones.
Atherosclerosis-deafness-diabetes-epilepsy-nephropathy syndrome
MedGen UID:
349198
Concept ID:
C1859596
Disease or Syndrome
A rare, severe, circulatory system disease characterized by premature, diffuse, severe atherosclerosis (including the aorta and renal, coronary, and cerebral arteries), sensorineural deafness, diabetes mellitus, progressive neurological deterioration with cerebellar symptoms and photomyoclonic seizures, and progressive nephropathy. Partial deficiency of mitochondrial complexes III and IV in the kidney and fibroblasts (but not in muscle) may be associated. There have been no further descriptions in the literature since 1994.
Grange syndrome
MedGen UID:
355427
Concept ID:
C1865267
Disease or Syndrome
Grange syndrome (GRNG) is a rare early-onset disease characterized by hypertension and multifocal stenoocclusive lesions of renal, cerebral, and abdominal arteries. Bone fragility, syndactyly, brachydactyly, congenital heart defects, and learning disabilities have been reported with variable expressivity and incomplete penetrance (summary by Rath et al., 2019).
Alagille syndrome due to a JAG1 point mutation
MedGen UID:
365434
Concept ID:
C1956125
Disease or Syndrome
Alagille syndrome (ALGS) is a multisystem disorder with a wide spectrum of clinical variability; this variability is seen even among individuals from the same family. The major clinical manifestations of ALGS are bile duct paucity on liver biopsy, cholestasis, congenital cardiac defects (primarily involving the pulmonary arteries), butterfly vertebrae, ophthalmologic abnormalities (most commonly posterior embryotoxon), and characteristic facial features. Renal abnormalities, growth failure, developmental delays, splenomegaly, and vascular abnormalities may also occur.
Arterial calcification, generalized, of infancy, 1
MedGen UID:
1631685
Concept ID:
C4551985
Disease or Syndrome
Generalized arterial calcification of infancy (GACI) is characterized by infantile onset of widespread arterial calcification and/or narrowing of large and medium-sized vessels resulting in cardiovascular findings (which can include heart failure, respiratory distress, edema, cyanosis, hypertension, and/or cardiomegaly). Additional findings can include typical skin and retinal manifestations of pseudoxanthoma elasticum (PXE), periarticular calcifications, development of rickets after infancy, cervical spine fusion, and hearing loss. While mortality in infancy is high, survival into the third and fourth decades has occurred.
Neurodevelopmental disorder with microcephaly, cataracts, and renal abnormalities
MedGen UID:
1634867
Concept ID:
C4693567
Disease or Syndrome

Professional guidelines

PubMed

Gangat N, Szuber N, Tefferi A
Am J Hematol 2023 Jun;98(6):965-981. Epub 2023 Apr 3 doi: 10.1002/ajh.26920. PMID: 36966432
Arab SF, Alhumaid AA, Abu Alnasr MT, Altuwaijri TA, Al-Ghofili H, Al-Salman MM, Altoijry A
Saudi J Kidney Dis Transpl 2022 Jan-Feb;33(1):147-159. doi: 10.4103/1319-2442.367807. PMID: 36647988
Sarathy H, Salman LA, Lee C, Cohen JB
Med Clin North Am 2022 Mar;106(2):269-283. Epub 2022 Feb 2 doi: 10.1016/j.mcna.2021.11.004. PMID: 35227430Free PMC Article

Recent clinical studies

Etiology

Arab SF, Alhumaid AA, Abu Alnasr MT, Altuwaijri TA, Al-Ghofili H, Al-Salman MM, Altoijry A
Saudi J Kidney Dis Transpl 2022 Jan-Feb;33(1):147-159. doi: 10.4103/1319-2442.367807. PMID: 36647988
Safian RD
Prog Cardiovasc Dis 2021 Mar-Apr;65:60-70. Epub 2021 Mar 18 doi: 10.1016/j.pcad.2021.03.003. PMID: 33745915
Prince M, Gupta A, Bob-Manuel T, Tafur J
Prog Cardiovasc Dis 2020 Jan-Feb;63(1):58-63. Epub 2019 Dec 7 doi: 10.1016/j.pcad.2019.12.001. PMID: 31821813
Prince M, Tafur JD, White CJ
JACC Cardiovasc Interv 2019 Mar 25;12(6):505-517. doi: 10.1016/j.jcin.2018.10.023. PMID: 30898248
Bavishi C, de Leeuw PW, Messerli FH
Am J Med 2016 Jun;129(6):635.e5-635.e14. Epub 2015 Oct 29 doi: 10.1016/j.amjmed.2015.10.010. PMID: 26522797

Diagnosis

Arab SF, Alhumaid AA, Abu Alnasr MT, Altuwaijri TA, Al-Ghofili H, Al-Salman MM, Altoijry A
Saudi J Kidney Dis Transpl 2022 Jan-Feb;33(1):147-159. doi: 10.4103/1319-2442.367807. PMID: 36647988
Aly A, Burt R, Violari E, Peña C, Bryce Y
Tech Vasc Interv Radiol 2022 Dec;25(4):100863. Epub 2022 Sep 6 doi: 10.1016/j.tvir.2022.100863. PMID: 36404064
Safian RD
Prog Cardiovasc Dis 2021 Mar-Apr;65:60-70. Epub 2021 Mar 18 doi: 10.1016/j.pcad.2021.03.003. PMID: 33745915
Bob-Manuel T, White CJ
Interv Cardiol Clin 2020 Apr;9(2):169-185. Epub 2020 Jan 31 doi: 10.1016/j.iccl.2019.11.002. PMID: 32147118
Stevens S
Nurs Clin North Am 2018 Dec;53(4):569-578. Epub 2018 Oct 12 doi: 10.1016/j.cnur.2018.07.007. PMID: 30388982

Therapy

Arab SF, Alhumaid AA, Abu Alnasr MT, Altuwaijri TA, Al-Ghofili H, Al-Salman MM, Altoijry A
Saudi J Kidney Dis Transpl 2022 Jan-Feb;33(1):147-159. doi: 10.4103/1319-2442.367807. PMID: 36647988
Safian RD
Prog Cardiovasc Dis 2021 Mar-Apr;65:60-70. Epub 2021 Mar 18 doi: 10.1016/j.pcad.2021.03.003. PMID: 33745915
Prince M, Tafur JD, White CJ
JACC Cardiovasc Interv 2019 Mar 25;12(6):505-517. doi: 10.1016/j.jcin.2018.10.023. PMID: 30898248
Rimoldi SF, Scherrer U, Messerli FH
Eur Heart J 2014 May 14;35(19):1245-54. Epub 2013 Dec 23 doi: 10.1093/eurheartj/eht534. PMID: 24366917
Cooper CJ, Murphy TP, Cutlip DE, Jamerson K, Henrich W, Reid DM, Cohen DJ, Matsumoto AH, Steffes M, Jaff MR, Prince MR, Lewis EF, Tuttle KR, Shapiro JI, Rundback JH, Massaro JM, D'Agostino RB Sr, Dworkin LD; CORAL Investigators
N Engl J Med 2014 Jan 2;370(1):13-22. Epub 2013 Nov 18 doi: 10.1056/NEJMoa1310753. PMID: 24245566Free PMC Article

Prognosis

Rumman RK, Matsuda-Abedini M, Langlois V, Radhakrishnan S, Lorenzo AJ, Amaral J, Mertens L, Parekh RS
Am J Hypertens 2018 May 7;31(6):687-695. doi: 10.1093/ajh/hpy014. PMID: 29373648
Coleman DM, Stanley JC
J Vasc Surg 2015 Sep;62(3):779-85. Epub 2015 Jul 26 doi: 10.1016/j.jvs.2015.05.034. PMID: 26213273
Cooper CJ, Murphy TP, Cutlip DE, Jamerson K, Henrich W, Reid DM, Cohen DJ, Matsumoto AH, Steffes M, Jaff MR, Prince MR, Lewis EF, Tuttle KR, Shapiro JI, Rundback JH, Massaro JM, D'Agostino RB Sr, Dworkin LD; CORAL Investigators
N Engl J Med 2014 Jan 2;370(1):13-22. Epub 2013 Nov 18 doi: 10.1056/NEJMoa1310753. PMID: 24245566Free PMC Article
Plouin PF, Bax L
Nat Rev Nephrol 2010 Mar;6(3):151-9. Epub 2010 Jan 26 doi: 10.1038/nrneph.2009.230. PMID: 20101256
Kendrick J, Chonchol M
Adv Chronic Kidney Dis 2008 Oct;15(4):355-62. doi: 10.1053/j.ackd.2008.07.004. PMID: 18805381

Clinical prediction guides

Gupta R, Assiri S, Cooper CJ
Curr Cardiol Rep 2017 Sep;19(9):75. doi: 10.1007/s11886-017-0894-2. PMID: 28752274
Schoepe R, McQuillan S, Valsan D, Teehan G
Adv Exp Med Biol 2017;956:209-213. doi: 10.1007/5584_2016_89. PMID: 27873231
Smith A, Gaba RC, Bui JT, Minocha J
Tech Vasc Interv Radiol 2016 Sep;19(3):211-7. Epub 2016 Jun 3 doi: 10.1053/j.tvir.2016.06.006. PMID: 27641455
Coleman DM, Stanley JC
J Vasc Surg 2015 Sep;62(3):779-85. Epub 2015 Jul 26 doi: 10.1016/j.jvs.2015.05.034. PMID: 26213273
Zeller T, Macharzina R, Rastan A, Beschorner U, Noory E
Vasa 2014 Jan;43(1):27-38. doi: 10.1024/0301-1526/a000325. PMID: 24429328

Recent systematic reviews

Hinojosa-Gonzalez DE, Salgado-Garza G, Torres-Martinez M, Villegas-De Leon SU, Bueno-Gutierrez LC, Herrera-Carrillo FE, Gonzalez-Urquijo M, Segura Ibarra V, Fabiani MA, Flores-Villalba E
J Endovasc Ther 2022 Apr;29(2):294-306. Epub 2021 Aug 16 doi: 10.1177/15266028211038593. PMID: 34399594
van Brussel PM, van de Hoef TP, de Winter RJ, Vogt L, van den Born BJ
JACC Cardiovasc Interv 2017 May 22;10(10):973-985. doi: 10.1016/j.jcin.2017.02.046. PMID: 28521931
Raman G, Adam GP, Halladay CW, Langberg VN, Azodo IA, Balk EM
Ann Intern Med 2016 Nov 1;165(9):635-649. Epub 2016 Aug 16 doi: 10.7326/M16-1053. PMID: 27536808
de Mast Q, Beutler JJ
J Hypertens 2009 Jul;27(7):1333-40. doi: 10.1097/HJH.0b013e328329bbf4. PMID: 19365285
Balk E, Raman G, Chung M, Ip S, Tatsioni A, Alonso A, Chew P, Gilbert SJ, Lau J
Ann Intern Med 2006 Dec 19;145(12):901-12. Epub 2006 Oct 24 doi: 10.7326/0003-4819-145-12-200612190-00143. PMID: 17062633

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