Rheumatology Book

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Takayasu Arteritis

Aka: Takayasu Arteritis, Young Female Arteritis, Occlusive Thromboarteriopathy, Martorell Syndrome, Raeder-Harbitz Syndrome, Reverse Coarctation, Middle Aortic Syndrome, Idiopathic Medial Aortopathy and Arteriopathy
  1. Epidemiology
    1. Age: 20 to 40 years old
    2. Gender: Women account for up to 90% of cases
    3. Race: More common in asian descent
  2. Pathophysiology
    1. Rare, large vessel Vasculitis
    2. Granulomatous inflammation of the aorta and its main branches
  3. Symptoms
    1. Phase 1: Inflammatory Response (Early in course)
      1. Fatigue
      2. Weight loss
      3. Low-grade fever
      4. Night Sweats
      5. Arthralgias
      6. Myalgias
    2. Phase 2: Vascular Insufficiency (Later in course)
      1. Finger coldness and numbness
      2. Skin lesions similar to Erythema Nodosum or Pyoderma Gangrenosum
      3. Focal symptoms are related to specific arterial involvement
        1. Carotid Artery involvement with CNS symptoms
        2. Abdominal aorta with lower extremity Claudication
  4. Signs
    1. Hypertension
    2. Diminished Arterial Pulses (may start in upper extremities)
    3. Visual disturbances
    4. Neurologic deficit
    5. Blood Pressure discrepancy (e.g. decreased in lower extremities)
    6. Vascular Bruits
  5. Diagnosis: ACR Criteria (three of six present for diagnosis)
    1. Age at disease onset <40 years old
    2. Extremity Claudication
    3. Decreased brachial artery pulse
    4. Blood Pressure difference between arms >10 mmHg
    5. Subclavian Artery or Aortic Bruit
    6. Abnormal arteriogram
  6. Labs
    1. Complete Blood Count (CBC)
      1. Normochromic Normocytic Anemia
    2. Increased acute phase reactants
      1. Increased C-Reactive Protein (C-RP)
      2. Increased Erythrocyte Sedimentation Rate (ESR)
      3. Pentraxin-3
    3. Biopsy of vascular tissue
      1. Cytotoxic Lymphocyte infiltration (esp. gamma delta T Lymphocytes)
  7. Imaging: Angiography
    1. CT angiogram or the chest AbdomenPelvis (aortic survey)
      1. Alternatively, MRA Angiogram or Standard Angiography of arterial tree may be performed instead
      2. Findings
        1. Smoothly tapering vessel lumens or Occlusion
        2. Vessel wall thickening
    2. Doppler Ultrasound
      1. May identify vessel wall inflammation
  8. Differential Diagnosis
    1. See Vasculitis
    2. Fibromuscular dysplasia
    3. Ergotamine toxicity
    4. Ehlers-Danlos Syndrome
    5. Temporal Arteritis
  9. Management
    1. Monitoring for response with acute phase reactants and imaging
    2. High dose Corticosteroids (e.g. Prednisone 40-60 mg orally daily)
      1. Response to Corticosteroids in only 50% of patients
    3. Immunosuppressants (e.g. cyclophosphamide, Azathioprine, Methotrexate)
      1. Typically administered in combination with Corticosteroids
    4. Hypertension Management
    5. Surgery Indications (for disease refractory to Corticosteroids and immunosuppressants)
      1. Hypertension with critical Renal Artery Stenosis
      2. Extremity Claudication limiting Activities of Daily Living
      3. Cerebrovascular ischemia or critical stenosis of three of more Cerebral Vessels
      4. Moderate Aortic Regurgitation
      5. Cardiac ischemia with confirmed Coronary Artery involvement
  10. Complications
    1. Cardiovascular and systemic Hypertension complications
      1. Arterial aneurysm or dissection
      2. Dilated Cardiomyopathy
      3. Aortic Valve Insufficiency
      4. Congestive Heart Failure
    2. Neurologic
      1. Cerebrovascular Accident
      2. Seizure Disorder
      3. Intracranial Aneurysm
      4. Encephalitis
    3. Gastrointestinal
      1. Mesenteric Iscehmia
    4. Ocular
      1. Retinal ischemia
      2. Retinal Detachment
  11. References
    1. Hunder (2011) in UpToDate, Clinical features and diagnosis of Takayasu Arteritis, Accessed 11/30/11
    2. Mezan (2022) Crit Dec Emerg Med 36(8): 10-2
    3. Natraj Setty (2017) J Rare Dis Res Treat 2(2): 63-8
      1. https://www.rarediseasesjournal.com/articles/takayasus-arteritis--a-comprehensive-review.pdf
    4. Russo (2018) Front Pediatr 6:265 +PMID: 30338248 [PubMed]
    5. Serra (2016) Ann Vasc Surg 35:210-25 +PMID:27238990 [PubMed]

Takayasu Arteritis (C0039263)

Definition (MSHCZE) Vaskulitida postihující aortu a hlavní tepny z ní odstupující, zejm. tepny z oblouku aorty, které zásobují krví hlavu a horní končetiny. Postihuje obv. mladší ženy, etiologie je neznámá, vyskytuje se spíše vzácně. Onemocnění začíná celkovými příznaky (systémová fáze) s horečkou, únavou, artralgiemi, myalgiemi, nočním pocením, hubnutím apod. V okluzní fázi jsou příznaky související s poruchou prokrvení v daných oblastech. Bývají klaudikace horních končetin, bolesti hlavy, závratě, parestezie, poruchy zraku. Hypertenze může vést k srdečnímu selhání. Při fyzikálním vyšetření jsou šelesty nad velkými tepnami, na tepnách horní končetiny někdy není hmatný puls (tzv. "bezpulsová nemoc"). Pro diagnózu je zásadní arteriografie. Laboratorní nález je nespecifický s obrazem chronického zánětu (anemie, zrychlená sedimentace). Změny lze pozorovat i na očním pozadí. Neléčené onemocnění mělo špatnou prognózu. Léčebně se podávají kortikoidy, ev. s imunosupresivy, k odstranění stenóz se používá perkutánní angioplastika či bypass. (cit. Velký lékařský slovník online, 2012 http://lekarske.slovniky.cz/ )
Definition (NCI) A large vessel vasculitis affecting the aorta and its branches. It usually affects young females. It causes vascular obstruction, resulting in asymmetric pulses.
Definition (MSH) A chronic inflammatory process that affects the AORTA and its primary branches, such as the brachiocephalic artery (BRACHIOCEPHALIC TRUNK) and CAROTID ARTERIES. It results in progressive arterial stenosis, occlusion, and aneurysm formation. The pulse in the arm is hard to detect. Patients with aortitis syndrome often exhibit retinopathy.
Concepts Disease or Syndrome (T047)
MSH D013625
ICD9 446.7
ICD10 M31.4
SnomedCT 359789008, 195361009, 42153001, 155445002, 266323005
English Arteritis, Takayasu, Pulseless Disease, Arteritis, Takayasu's, Arteritis, Takayasus, Takayasus Arteritis, Aortic arch syndrome [Takayasu], TAKAYASUS ARTERITIS, TAKAYASU ARTERITIS, YOUNG FEMALE ARTERITIS, PULSELESS DISEASE, PULSELESS DIS, Takayasu Syndrome, Takayasu's Arteritis, Takayasu's disease (diagnosis), Takayasu Arteritis, Disease, Takayasu, Takayasu Disease, Takayasu disease, Takayasu Arteritis [Disease/Finding], Female Arteritides, Young, Young Female Arteritis, Arteritides, Young Female, Arteritis, Young Female, Young Female Arteritides, Female Arteritis, Young, takayasu's disease, aortic arch syndrome, arteritis takayasu, takayasu disease, takayasu syndrome, arteritis takayasu's, arteritis takayasus's, takayasu's arteritis, aortic arch arteritis, middle aortic syndrome, takayasu arteritis, takayasus arteritis, (Aortic arch arteritis) or (Takayasu's disease[& pulseless]), (Aortic arch arteritis) or (Takayasu's disease[& pulseless]) (disorder), Takayasu's disease, Pulseless disease, Takayasu's arteritis, Acquired aortoarteritis, Aortic arch arteritis, Atypical coarctation, Martorell syndrome, Nonspecific aortoarteritis, Nonspecific arteritis, Occlusive thromboarteriopathy, Primary arteritis, Raeder-Harbitz syndrome, Reverse coarctation, Takayasu's arteriopathy, Young female arteritis, Idiopathic medial aortopathy AND arteriopathy, Sclerosing aortitis AND arteritis, Takayasu's disease (disorder), pulseless disease, disease; pulseless, Takayasu, pulseless; disease, aortic arch; arteritis, arteritis; aortic arch, Idiopathic medial aortopathy and arteriopathy, Middle aortic syndrome, Sclerosing aortitis and arteritis, Takayasu's disease [Ambiguous]
Dutch polsloze ziekte, ziekte van Takayasu, aortaboog; arteriitis, arteriitis; aortaboog, disease; pulseless, pulseless; disease, Aortaboogsyndroom [Takayasu], Aortaboogsyndroom, Arteritis, Takayasu-, Polsloze ziekte, Takayasu-arteritis, Takayasu-syndroom
German Pulslos-Krankheit, Takayasu-Krankheit, Aortenbogen-Syndrom [Takayasu-Syndrom], Takayasu Krankheit, Arteritis, Takayasu-, Pulseless Disease, Takayasu-Arteritis, Takayasu-Syndrom
Portuguese Doença de Takayasu, Doença sem pulso, Síndrome Aortítica, Arterite de Takayasu, Doença sem Pulso
Spanish Enfermedad de Takayasu, Enfermedad sin pulsos, arteritis y aortitis esclerosante, arteritis del arco aórtico, arteritis inespecífica, síndrome aórtico medio, arteriopatía de Takayasu, síndrome de Raeder - Harbitz, arteriopatía y aortopatía media idiopática, enfermedad de Takayasu (concepto no activo), aortoarteritis adquirida, aortoarteritis inespecífica, arteriopatía medial idiopática y arteriopatía, arteritis Y aortitis esclerosante, arteritis de Takayasu (trastorno), arteritis de Takayasu, arteritis primaria, coartación atípica, coartación reversible, enfermedad de Takayasu, enfermedad sin pulso, síndrome de Martorell, síndrome de Raeder-Harbitz, tromboarteriopatía oclusiva, Arteritis de Takayasu, Enfermedad sin Pulso
Swedish Takayasus arterit
Japanese タカヤスビョウ, ミャクナシビョウ, タカヤスドウミャクエン, Takayasu病, 高安動脈炎, 脈なし病, 高安病, 高安症候群, 動脈炎-高安
Czech Takayasův syndrom, bezpulsová nemoc, Bezpulzová nemoc, bezpulzová nemoc, Takayasova arteritida, beztepová nemoc, Takayasuova arteriitida, Takayasuova nemoc, arteriitida mladých žen
Finnish Takayasun tauti
Italian Sindrome di Takayasu, Malattia senza polso, Malattia di Takayasu, AT, Arterite di Takayasu
Russian TAKAIASU ARTERIIT, ARTERIIT TAKAIASU, PUL'SA OTSUTSTVIIA BOLEZN', TAKAIASU SINDROM, АРТЕРИИТ ТАКАЯСУ, ПУЛЬСА ОТСУТСТВИЯ БОЛЕЗНЬ, ТАКАЯСУ АРТЕРИИТ, ТАКАЯСУ СИНДРОМ
Korean 대동맥활 증후군[다까야수]
Polish Zespół Takayasu, Zespół zapalenia aorty, Choroba Takayasu, Zapalenie tętnic Takayasu
Hungarian Takayasu-betegség, Takayasu-arteritis, Pulzus nélküli betegség
Norwegian Takayasus arteritt
French Maladie de Takayasu, Artérite de Takayasu, Artérite oblitérante de Takayashu, Maladie de Takayashu, Maladie des femmes sans pouls
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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