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Takayasu Arteritis Medication

  • Author: Jefferson R Roberts, MD; Chief Editor: Herbert S Diamond, MD  more...
 
Updated: Apr 13, 2016
 

Medication Summary

The goals of therapy in Takayasu arteritis are to reduce inflammation and suppress autoimmune disease. To treat the active disease, corticosteroids are used and gradually tapered. Cytotoxic agents such methotrexate, azathioprine, and cyclophosphamide are the main therapeutic agents when the response to steroids is unsatisfactory. As previously mentioned, anti–tumor necrosis factor (anti-TNF) agents have shown encouraging results in a small number of patients with relapsing Takayasu arteritis.[39]

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Corticosteroids

Class Summary

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli.

Prednisone

 

The immunosuppressant prednisone is a first-line therapy administered for the treatment of autoimmune disorders. It may decrease inflammation by reversing increased capillary permeability and suppressing polymorphonuclear leukocyte activity and CD4 counts.

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Antineoplastics, Other

Class Summary

Cyclophosphamide or methotrexate—either in combination with prednisone or alone—can be used to suppress active disease in patients who are not responding to prednisone.

Cyclophosphamide

 

Cyclophosphamide may be added to steroid therapy if the patient has shown minimal response to steroid treatment or has been on a steroid for a prolonged period of time. Cyclophosphamide is chemically related to nitrogen mustards. As an alkylating agent, the mechanism of action of the active metabolites may involve cross-linking of deoxyribonucleic acid (DNA), which may interfere with the growth of normal and neoplastic cells.

Methotrexate (Trexall, Rheumatrex)

 

Methotrexate may be added to treatment if steroid therapy has not been effective or if the patient has been on steroid treatment for a prolonged period of time. Methotrexate has an unknown mechanism of action in the treatment of inflammatory reactions; the drug may affect immune function. Methotrexate ameliorates symptoms of inflammation (eg, pain, swelling, stiffness). Adjust the dose gradually to attain a satisfactory response.

Effects can be observed as early as 3-6 weeks following methotrexate's administration. Methotrexate is used as second- or third-line drug to suppress active disease.

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Immunosuppressants

Class Summary

These agents inhibit key factors that mediate immune reactions.[42]

Azathioprine (Imuran, Azasan)

 

Azathioprine may be added to treatment if there has been no response to steroid therapy or if the patient has been on steroid treatment for a long period of time. Azathioprine antagonizes purine metabolism and inhibits the synthesis of DNA, ribonucleic acid (RNA), and proteins. It may decrease the proliferation of immune cells, which results in lower autoimmune activity.

Mycophenolate (CellCept, Myfortic)

 

Mycophenolate inhibits inosine monophosphate dehydrogenase (IMPDH) and suppresses de novo purine synthesis by lymphocytes, thereby inhibiting their proliferation. Mycophenolate inhibits antibody production.

Two formulations are available and are not interchangeable. The original formulation, mycophenolate mofetil (CellCept), is a prodrug that, once hydrolyzed in vivo, releases the active moiety mycophenolic acid. A newer formulation, mycophenolic acid (Myfortic), is an enteric-coated product that delivers the active moiety.

Tacrolimus (Prograf)

 

Tacrolimus suppresses humoral (T-lymphocyte) immunity.

Infliximab (Remicade)

 

Infliximab may be added to treatment if steroids and other immunosuppressant drugs are ineffective in achieving or maintaining remission. Infliximab is a chimeric IgG1k monoclonal antibody that neutralizes cytokine TNF-α and inhibits its binding to the TNF-α receptor. It reduces the infiltration of inflammatory cells and TNF-α production in inflamed areas.

Tocilizumab (Actemra)

 

Tocilizumab is an IL-6 receptor inhibitor. IL-6 inhibition results in a decreased C-reactive protein level to within the normal range, decreased values in other pharmacodynamic parameters (eg, rheumatoid factor, erythrocyte sedimentation rate, amyloid A), and an increased hemoglobin value.

Rituximab (Rituxan)

 

Rituximab is a chimeric murine/human monoclonal antibody directed against the CD20 antigen found on the surface of B-lymphocytes.

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DMARDs, TNF Inhibitors

Class Summary

These agents play an important role in modulating the inflammatory process.

Etanercept (Enbrel)

 

Etanercept may be added to treatment if steroids and other immunosuppressant drugs are ineffective in achieving or maintaining remission. Etanercept is a soluble p75 TNF receptor fusion protein (sTNFR-Ig). It inhibits TNF binding to cell surface receptors, which, in turn, decreases inflammatory and immune responses.

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Calcium Channel Blockers

Class Summary

These drugs can be used to treat hypertension associated with arteritis. On occasion, combinations are required. Therapy can be individualized.

Nifedipine (Procardia)

 

Nifedipine is one of the more common channel blockers used for hypertension associated with arteritis.

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Antiplatelet Agents, Hematologic

Class Summary

These drugs help to prevent cerebrovascular accidents and improve renal and systemic function.

Ticlopidine hydrochloride

 

Ticlopidine hydrochloride interferes with platelet membrane function by inhibiting adenosine diphosphate (ADP) ̶ induced platelet-fibrinogen binding and subsequent platelet-platelet interaction. It is used as a second-line antiplatelet therapy for patients who are intolerant to aspirin therapy or in whom such therapy fails.

Clopidogrel (Plavix)

 

Clopidogrel is a thienopyridine derivative chemically related to ticlopidine that inhibits platelet aggregation; it selectively inhibits ADP binding to its platelet receptor and subsequent ADP-mediated activation of the glycoprotein GPIIb/IIIa complex, thereby inhibiting platelet aggregation.

Aspirin and extended-release dipyridamole (Aggrenox)

 

This drug combination has antithrombotic action. Aspirin inhibits prostaglandin synthesis, preventing the formation of platelet-aggregating thromboxane A2. It may be used in low dose to inhibit platelet aggregation and improve complications of venous stases and thrombosis.

Dipyridamole is a platelet adhesion inhibitor that possibly inhibits red blood cell uptake of adenosine, itself an inhibitor of platelet reactivity. In addition, dipyridamole may inhibit phosphodiesterase activity leading to increased cyclic-3', 5'-adenosine monophosphate within platelets and formation of the potent platelet activator thromboxane A2.

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Anticoagulants, Hematologic

Class Summary

In patients with renal failure due to crescentic glomerulonephritis and nephrotic syndrome, low-dose heparin followed by oral anticoagulation leads to improved renal and systemic function. It probably reduces the destructive effects of fibrin thrombi in the small vessels of the kidney.

Warfarin (Coumadin, Jantoven)

 

Warfarin interferes with hepatic synthesis of vitamin K ̶ dependent coagulation factors. It is used for the prophylaxis and treatment of venous thrombosis, pulmonary embolism, and thromboembolic disorders.

Tailor the dose to maintain an international normalized ratio (INR) in the range of 2-3.

Heparin

 

Heparin augments the activity of antithrombin III. It does not actively lyse, but it is able to block further thrombogenesis. Heparin prevents reaccumulation of a clot after a spontaneous fibrinolysis.

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Contributor Information and Disclosures
Author

Jefferson R Roberts, MD Chief of Rheumatology Service, Tripler Army Medical Center; Assistant Clinical Professor of Medicine, Uniformed Services University of the Health Sciences

Jefferson R Roberts, MD is a member of the following medical societies: American College of Physicians, American College of Rheumatology, Society for Simulation in Healthcare

Disclosure: Nothing to disclose.

Coauthor(s)

Rodger Stitt, MD Department of Internal Medicine, Tripler Army Medical Center, Honolulu

Rodger Stitt, MD is a member of the following medical societies: American College of Physicians

Disclosure: Nothing to disclose.

 

Disclosure: Nothing to disclose.

Phalgoon A Shah, MD Resident Physician, Department of Medicine, Tripler Army Medical Center

Phalgoon A Shah, MD is a member of the following medical societies: American College of Physicians

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, Phi Beta Kappa

Disclosure: Nothing to disclose.

Additional Contributors

Gabriel Bucurescu, MD, MS Staff Neurologist, Neurology Service, Philadelphia Veterans Affairs Medical Center

Gabriel Bucurescu, MD, MS is a member of the following medical societies: American Academy of Neurology, American Clinical Neurophysiology Society, American Epilepsy Society

Disclosure: Nothing to disclose.

Robert E Wolf, MD, PhD Professor Emeritus, Department of Medicine, Louisiana State University School of Medicine in Shreveport; Chief, Rheumatology Section, Medical Service, Overton Brooks Veterans Affairs Medical Center

Robert E Wolf, MD, PhD is a member of the following medical societies: American College of Rheumatology, Arthritis Foundation, Society for Leukocyte Biology

Disclosure: Nothing to disclose.

Mohammed Mubashir Ahmed, MD Associate Professor, Department of Medicine, Division of Rheumatology, University of Toledo College of Medicine

Mohammed Mubashir Ahmed, MD is a member of the following medical societies: American College of Physicians, American College of Rheumatology, American Federation for Medical Research

Disclosure: Nothing to disclose.

Acknowledgements

Elliot Goldberg, MD Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine, Temple University School of Medicine

Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, and American College of Rheumatology

Disclosure: Nothing to disclose.

B Mark Keegan, MD, FRCPC Assistant Professor of Neurology, College of Medicine, Mayo Clinic; Master's Faculty, Mayo Graduate School; Consultant, Department of Neurology, Mayo Clinic, Rochester

B Mark Keegan, MD, FRCPC is a member of the following medical societies: American Academy of Neurology, American Medical Association, and Minnesota Medical Association

Disclosure: Novartis Consulting fee Consulting

Sydney Louis, MBBCh, MD Emeritus Professor, Department of Neurology, The Warren Alpert Medical School of Brown University

Sydney Louis, MBBCh, MD is a member of the following medical societies: American Academy of Neurology

Disclosure: Nothing to disclose.

Michael G Rossman, MD, LTC, MC, FS Fellow, Department of Rheumatology, Walter Reed Army Medical Center

Michael G Rossman, MD, LTC, MC, FS is a member of the following medical societies: American College of Physicians, American College of Rheumatology, American Medical Association, and Society of US Army Flight Surgeons

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Florian P Thomas, MD, MA, PhD, Drmed Director, Spinal Cord Injury Unit, St Louis Veterans Affairs Medical Center; Director, National MS Society Multiple Sclerosis Center; Director, Neuropathy Association Center of Excellence, Professor, Department of Neurology and Psychiatry, Associate Professor, Institute for Molecular Virology, and Department of Molecular Microbiology and Immunology, St Louis University School of Medicine

Florian P Thomas, MD, MA, PhD, Drmed is a member of the following medical societies: American Academy of Neurology, American Neurological Association, American Paraplegia Society, Consortium of Multiple Sclerosis Centers, and National Multiple Sclerosis Society

Disclosure: Nothing to disclose.

References
  1. Jennette JC, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994 Feb. 37(2):187-92. [Medline].

  2. Hata A, Noda M, Moriwaki R, Numano F. Angiographic findings of Takayasu arteritis: new classification. Int J Cardiol. 1996 Aug. 54 Suppl:S155-63. [Medline].

  3. Arnaud L, Haroche J, Mathian A, Gorochov G, Amoura Z. Pathogenesis of Takayasu's arteritis: a 2011 update. Autoimmun Rev. 2011 Nov. 11(1):61-7. [Medline].

  4. Aggarwal A, Chag M, Sinha N, Naik S. Takayasu's arteritis: role of Mycobacterium tuberculosis and its 65 kDa heat shock protein. Int J Cardiol. 1996 Jul 5. 55(1):49-55. [Medline].

  5. Kumar Chauhan S, Kumar Tripathy N, Sinha N, Singh M, Nityanand S. Cellular and humoral immune responses to mycobacterial heat shock protein-65 and its human homologue in Takayasu's arteritis. Clin Exp Immunol. 2004 Dec. 138(3):547-53. [Medline]. [Full Text].

  6. Soto ME, Vargas-Alarcón G, Cicero-Sabido R, Ramírez E, Alvarez-León E, Reyes PA. Comparison distribution of HLA-B alleles in mexican patients with takayasu arteritis and tuberculosis. Hum Immunol. 2007 May. 68(5):449-53. [Medline].

  7. Yagi K, Kobayashi J, Yasue S, Yamaguchi M, Shiobara S, Mabuchi H. Four unrelated cases with Takayasu arteritis and CD36 deficiency: possible link between these disorders. J Intern Med. 2004 Jun. 255(6):688-9. [Medline].

  8. Hall S, Barr W, Lie JT, Stanson AW, Kazmier FJ, Hunder GG. Takayasu arteritis. A study of 32 North American patients. Medicine (Baltimore). 1985 Mar. 64(2):89-99. [Medline].

  9. Numano F, Kobayashi Y. Takayasu arteritis--beyond pulselessness. Intern Med. 1999 Mar. 38(3):226-32. [Medline].

  10. Jain S, Kumari S, Ganguly NK, Sharma BK. Current status of Takayasu arteritis in India. Int J Cardiol. 1996 Aug. 54 Suppl:S111-6. [Medline].

  11. Phillip R, Luqmani R. Mortality in systemic vasculitis: a systematic review. Clin Exp Rheumatol. 2008 Sep-Oct. 26(5 Suppl 51):S94-104. [Medline].

  12. Kerr GS, Hallahan CW, Giordano J, Leavitt RY, Fauci AS, Rottem M, et al. Takayasu arteritis. Ann Intern Med. 1994 Jun 1. 120(11):919-29. [Medline].

  13. Maksimowicz-McKinnon K, Clark TM, Hoffman GS. Limitations of therapy and a guarded prognosis in an American cohort of Takayasu arteritis patients. Arthritis Rheum. 2007 Mar. 56(3):1000-9. [Medline].

  14. Park MC, Lee SW, Park YB, Chung NS, Lee SK. Clinical characteristics and outcomes of Takayasu's arteritis: analysis of 108 patients using standardized criteria for diagnosis, activity assessment, and angiographic classification. Scand J Rheumatol. 2005 Jul-Aug. 34(4):284-92. [Medline].

  15. Abularrage CJ, Slidell MB, Sidawy AN, Kreishman P, Amdur RL, Arora S. Quality of life of patients with Takayasu's arteritis. J Vasc Surg. 2008 Jan. 47(1):131-6; discussion 136-7. [Medline].

  16. Akar S, Can G, Binicier O, Aksu K, Akinci B, Solmaz D, et al. Quality of life in patients with Takayasu's arteritis is impaired and comparable with rheumatoid arthritis and ankylosing spondylitis patients. Clin Rheumatol. 2008 Jul. 27(7):859-65. [Medline].

  17. Maksimowicz-McKinnon K, Clark TM, Hoffman GS. Limitations of therapy and a guarded prognosis in an American cohort of Takayasu arteritis patients. Arthritis Rheum. 2007 Mar. 56(3):1000-9. [Medline].

  18. Soto ME, Espinola N, Flores-Suarez LF, Reyes PA. Takayasu arteritis: clinical features in 110 Mexican Mestizo patients and cardiovascular impact on survival and prognosis. Clin Exp Rheumatol. 2008 May-Jun. 26(3 Suppl 49):S9-15. [Medline].

  19. Francès C, Boisnic S, Blétry O, Dallot A, Thomas D, Kieffer E. Cutaneous manifestations of Takayasu arteritis. A retrospective study of 80 cases. Dermatologica. 1990. 181(4):266-72. [Medline].

  20. Arend WP, Michel BA, Bloch DA, Hunder GG, Calabrese LH, Edworthy SM, et al. The American College of Rheumatology 1990 criteria for the classification of Takayasu arteritis. Arthritis Rheum. 1990 Aug. 33(8):1129-34. [Medline].

  21. Siglock TJ, Brookler KH. Sensorineural hearing loss associated with Takayasu's disease. Laryngoscope. 1987 Jul. 97(7 Pt 1):797-800. [Medline].

  22. Raza K, Karokis D, Kitas GD. Cogan's syndrome with Takayasu's arteritis. Br J Rheumatol. 1998 Apr. 37(4):369-72. [Medline].

  23. Miller DV, Maleszewski JJ. The pathology of large-vessel vasculitides. Clin Exp Rheumatol. 2011 Jan-Feb. 29(1 Suppl 64):S92-8. [Medline].

  24. Gilden D, White TM, Nagae L, Gurdin WH, Boyer PJ, Nagel MA. Successful Antiviral Treatment of Giant Cell Arteritis and Takayasu Arteritis. JAMA Neurol. 2015 Aug. 72 (8):943-6. [Medline].

  25. Katz-Agranov N, Tanay A, Bachar DJ, Zandman-Goddard G. What to do when the Diagnosis of Giant Cell Arteritis and Takayasu's Arteritis Overlap. Isr Med Assoc J. 2015 Feb. 17 (2):123-5. [Medline]. [Full Text].

  26. Tripathy NK, Chandran V, Garg NK, Sinha N, Nityanand S. Soluble endothelial cell adhesion molecules and their relationship to disease activity in Takayasu's arteritis. J Rheumatol. 2008 Sep. 35 (9):1842-5. [Medline].

  27. Schmidt WA, Blockmans D. Use of ultrasonography and positron emission tomography in the diagnosis and assessment of large-vessel vasculitis. Curr Opin Rheumatol. 2005 Jan. 17(1):9-15. [Medline].

  28. Andrews J, Mason JC. Takayasu's arteritis--recent advances in imaging offer promise. Rheumatology (Oxford). 2007 Jan. 46(1):6-15. [Medline].

  29. Andrews J, Al-Nahhas A, Pennell DJ, Hossain MS, Davies KA, Haskard DO, et al. Non-invasive imaging in the diagnosis and management of Takayasu's arteritis. Ann Rheum Dis. 2004 Aug. 63(8):995-1000. [Medline]. [Full Text].

  30. Dagna L, Salvo F, Tiraboschi M, et al. Pentraxin-3 as a marker of disease activity in takayasu arteritis. Ann Intern Med. 2011 Oct 4. 155(7):425-33. [Medline].

  31. Direskeneli H, Aydin SZ, Merkel PA. Assessment of disease activity and progression in Takayasu's arteritis. Clin Exp Rheumatol. 2011 Jan-Feb. 29(1 Suppl 64):S86-91. [Medline].

  32. Magnani L, Versari A, Salvo D, et al. [Disease activity assessment in large vessel vasculitis]. Reumatismo. 2011. 63(2):86-90. [Medline].

  33. Blockmans D. PET in vasculitis. Ann N Y Acad Sci. 2011 Jun. 1228:64-70. [Medline].

  34. Fujita T, Ohtsuka M, Uchida E, Yamaguchi H, Nakajima T, Akazawa H, et al. Takayasu arteritis evaluated by multi-slice computed tomography in an old man. Int J Cardiol. 2008 Apr 10. 125(2):286-7. [Medline].

  35. Ragab Y, Emad Y, El-Marakbi A, Gheita T. Clinical utility of magnetic resonance angiography (MRA) in the diagnosis and treatment of Takayasu's arteritis. Clin Rheumatol. 2007 Aug. 26(8):1393-5. [Medline].

  36. Salvarani C, Magnani L, Catanoso M, et al. Tocilizumab: a novel therapy for patients with large-vessel vasculitis. Rheumatology (Oxford). 2012 Jan. 51(1):151-6. [Medline].

  37. Unizony S, Stone JH, Stone JR. New treatment strategies in large-vessel vasculitis. Curr Opin Rheumatol. 2013 Jan. 25(1):3-9. [Medline].

  38. Yokoe I, Haraoka H, Harashima H. A patient with Takayasu's arteritis and rheumatoid arthritis who responded to tacrolimus hydrate. Intern Med. 2007. 46(22):1873-7. [Medline].

  39. Maksimowicz-McKinnon K, Hoffman GS. Takayasu arteritis: what is the long-term prognosis?. Rheum Dis Clin North Am. 2007 Nov. 33(4):777-86, vi. [Medline].

  40. Hoffman GS, Merkel PA, Brasington RD, et al. Anti-tumor necrosis factor therapy in patients with difficult to treat Takayasu arteritis. Arthritis Rheum. 2004 Jul. 50(7):2296-304. [Medline].

  41. Youngstein T, Peters JE, Hamdulay SS, Mewar D, Price-Forbes A, Lloyd M, et al. Serial analysis of clinical and imaging indices reveals prolonged efficacy of TNF-a and IL-6 receptor targeted therapies in refractory Takayasu arteritis. Clin Exp Rheumatol. 2014 May-Jun. 32(3 Suppl 82):S11-8. [Medline].

  42. Tanaka F, Kawakami A, Iwanaga N, Tamai M, Izumi Y, Aratake K, et al. Infliximab is effective for Takayasu arteritis refractory to glucocorticoid and methotrexate. Intern Med. 2006. 45(5):313-6. [Medline].

 
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Complete occlusion of the left common carotid artery in a 48-year-old woman with Takayasu disease. Also note narrowing of the origin of the right subclavian artery and a narrowed small vessel with subsequent aneurysmal dilatation on the right side. Image courtesy of Robert Cirillo, MD.
Characteristic long, tapered narrowing of the distal aorta and iliac vessels. Image courtesy of Robert Cirillo, MD.
Image obtained in the same patient as in Image 2 reveals narrowing of the proximal descending aorta and right brachiocephalic artery. Image courtesy of Robert Cirillo, MD.
Aortogram of a 15-year-old girl with Takayasu arteritis. Note large aneurysms of descending aorta and dilatation of innominate artery. Image courtesy of Christine Hom, MD.
MRI of thorax of 15-year-old girl with Takayasu arteritis. Note aneurysms of descending aorta. Image courtesy of Christine Hom, MD.
Coronal MRI of abdomen of 15-year-old girl with Takayasu arteritis. Note thickening and tortuosity of abdominal aorta proximal to kidneys. Image courtesy of Christine Hom, MD.
 
 
 
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