Takayasu arteritis
Revisión del 07:24 20 feb 2017 de Devin Smith (discusión | contribs.) (Created page with "==Background== *A vasculitis that affects large vessels leading to fibrosis and narrowing of the vessels <ref>Hellmann, DB et al. Chapter 31. Takayasu Arteritis. In: Imboden J...")
Background
- A vasculitis that affects large vessels leading to fibrosis and narrowing of the vessels [1]
- Most often affects women of Asian descent during reproductive age
- Diagnosis is often delayed due to the rarity of the disease and varied symptoms
- The disease process chronically improves and relapses[2]
Clinical Features
- Hypertension
- Congestive Heart Failure
- CVA
- Dizziness
- Pulselessness
- Claudication
- Bruits
- Visual Disturbance
- Arthralgias/Myalgias
- Aortic Regurgitation
- Weight Loss
- Valvular Regurgitation
- Arterial Aneurysm
Differential Diagnosis
- Giant cell arteritis
- Thromboangiitis obliterans (Buerger's Disease)
- Systemic lupus erythematosus
- Syphilis
- Rheumatoid Arthritis
- Behcet's Disease
- Sarcoidosis
Evaluation
- Work up should be tailored to the patient's symptoms as this disease process affects multiple organ systems due to vascular damage
Laboratory
- ESR, CRP, WBC may or may not be elevated
- Anemia may be present
Imaging
- Angiography revealing narrowing/occlusion of large vessels (e.g. Aorta) is the gold standard [3]
- MRA or CTA can be used as well to appreciate vascular stenoses
Management
- Oral Prednisone 1 mg/kg for the first month followed by a taper
- Taper to approximately 10 mg/day
- The addition of Methotrexate or Mycophenolate mofetil to Prednisone therapy may be more effective
- Additional processes (e.g. Hypertension or Congestive Heart Failure) will require disease specific treatments
- In advanced disease processes, Cardiac or Vascular Surgery or Interventional Radiology may be required for angioplasty, stenting, or valvular repair
Disposition
- This will require clinical judgement on a case by case basis
- Patients should have a solid follow up plan with their primary care doctor or rheumatologist prior to discharge
- Patients with severe aortic regurgitation, large aortic aneurysm, or another concerning end product of this disease process will likely require inpatient admission with specialty service care
References
- ↑ Hellmann, DB et al. Chapter 31. Takayasu Arteritis. In: Imboden JB, Hellmann DB, Stone JH. eds. CURRENT Diagnosis & Treatment: Rheumatology, 3e New York, NY: McGraw-Hill; 2013.
- ↑ Hellmann, DB et al. Rheumatologic, Immunologic, & Allergic Disorders. In: Papadakis MA, McPhee SJ, Rabow MW. eds. Current Medical Diagnosis & Treatment 2017 New York, NY: McGraw-Hill
- ↑ Lin, PH et al. Arterial Disease. In: Brunicardi F, Andersen DK, Billiar TR, Dunn DL, Hunter JG, Matthews JB, Pollock RE. eds. Schwartz's Principles of Surgery, 10e New York, NY: McGraw-Hill; 2015.