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Behcet Disease Treatment & Management

  • Author: Fatima A Alnaimat, MBBS; Chief Editor: Herbert S Diamond, MD  more...
 
Updated: Apr 26, 2016
 

Approach Considerations

The treatment approach depends on the individual patient, severity of disease, and major organ involvement. The European League Against Rheumatism (EULAR) recommendations for the management of Behçet disease[37]  were developed in 2008 and aid in the management of different aspects of Behçet disease. Recommendations related to the eye, skin/mucosa, and arthritic disease are mainly evidence based, but recommendations on vascular, neurological, and gastrointestinal involvement are based largely on expert opinion and uncontrolled evidence from open trials and observational studies.[37]

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Medical Care

For oral and genital ulcerations, topical steroids or sucralfate solution are first-line therapy for mild isolated ulcerations. Colchicine has also been used to prevent mucocutaneous relapse.[38, 39] For severe mucocutaneous lesions, systemic corticosteroids, azathioprine, pentoxifylline, dapsone, interferon-alfa, colchicine, and thalidomide have demonstrated benefit.

For ocular disease, azathioprine is widely accepted as the initial agent. For severe eye disease (significant drop in visual acuity, retinal vasculitis, or macular involvement), either cyclosporine A or infliximab may be used in combination with azathioprine and corticosteroids.[40, 41, 37] Interferon-alfa, alone or in combination with corticosteroids, appears to be a second choice in eye disease.[42]

For GI lesions, based on expert opinion, 5-ASA derivatives, including sulfasalazine or mesalamine; systemic corticosteroids, azathioprine, tumor necrosis factor–α (TNF-α) antagonists, and thalidomide can be used.

Arthritis may respond to prednisone, local corticosteroid injections, and nonsteroidal anti-inflammatory drugs (NSAIDs), and colchicine. Interferon-alfa, azathioprine, and TNF-α blockers may be tried in rare cases of patients with resistant, prolonged, and disabling attacks.

Cutaneous disease with erythema nodosum is a special circumstance and may be treated with colchicine or dapsone.

CNS disease is usually treated with systemic corticosteroids, interferon-alfa, azathioprine, cyclophosphamide,[43] methotrexate, and TNF-α antagonists.[44]

Major-vessel disease with thrombotic events are treated with systemic anticoagulation in addition to corticosteroids, azathioprine, cyclophosphamide, or cyclosporine A. Pulmonary arterial aneurysms are treated with cyclophosphamide and corticosteroids.

TNF-α antagonists are increasingly used and have become standard treatment of Behçet disease that is inadequately controlled by standard immunosuppressive regimens. Infliximab has been most widely studied, but adalimumab has proved successful in cases refractory to both conventional therapy and infliximab.[45] . Etanercept is the only TNF inhibitor with data from a short term randomized controlled study with proven efficacy in suppressing most of the mucocutaneous manifestations of Behcet disease. [46]

In a multicenter study of the use of TNF-α antagonists (mainly infliximab and adalimumab) for treatment of severe and/or refractory Behçet's disease in 124 patients, clinical response rates with particular organ involvement were as follows[47] :

  • Ocular - 96.3%
  • Mucocutaneous - 88%
  • Joint - 70%
  • GI - 77.8%
  • CNS - 92.3%
  • Cardiovascular - 66.7%

Infliximab was reported to successfully treat a case of Behçet disease in pregnancy.[48]  Other therapies with data from case reports or small pilot studies include the following, among others:

Apremilast, an oral phosphodiesterase-4 inhibitor approved for the treatment of psoriasis and psoriatic arthritis, has  also been studied in Behçet syndrome. A phase 2, multicenter, placebo-controlled study of 111 patients with Behçet syndrome found apremilast to be effective in treating oral ulcers; at week 12, the mean number of oral ulcers per patient was significantly lower in the apremilast group than in the placebo group.[53] The effect of apremilast on the other manifestations of Behçet disease is not known.  

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Surgical Care

GI presentations that require surgical intervention include the following:

  • Intestinal stenosis
  • Lesions unresponsive to medical therapy
  • Fistula formation
  • Perforation
  • Severe bleeding

Surgery may also be considered in the following circumstances:

  • Pulmonary aneurysms and areas that incur ischemic damage due to vasculitis or thrombosis may require resection
  • Ventricular aneurysms, coronary thrombosis, and endocardial fibrosis are occasionally amenable to surgery
  • Glaucoma, cataracts, and retinal detachment occasionally warrant surgical intervention
  • Neurosurgery may be required to correct some CNS aneurysms and clots
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Consultations

See the list below:

  • Rheumatologist
  • Urologist for genital and urologic lesions
  • Neurologist for CNS involvement
  • Ophthalmologist for ocular disease
  • Gastroenterologist for intestinal disease
  • Dermatologist for possible help with recurrent skin lesions
  • Surgeon, when indicated
  • Nephrologist for proteinuria or hematuria
  • Pulmonologist or cardiologist in rare cases of intracardiac or pulmonary thrombosis and aneurysms
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Diet

No general dietary recommendations exist for Behçet disease. Patients with severe bowel involvement are advised to follow the same GI recommendations given to patients with inflammatory bowel disease. These patients often require total parenteral nutrition.

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Activity

Activity is suggested as tolerated and may be limited owing to systemic symptoms or arthritis.

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Contributor Information and Disclosures
Author

Fatima A Alnaimat, MBBS Rheumatologist, Essentia Health

Fatima A Alnaimat, MBBS is a member of the following medical societies: American College of Physicians, American College of Rheumatology

Disclosure: Nothing to disclose.

Coauthor(s)

Jeffrey R Lisse, MD, FACP Professor, Department of Internal Medicine, Chief, Section of Rheumatology, University of Arizona School of Medicine

Jeffrey R Lisse, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, American College of Rheumatology, American Geriatrics Society, Sigma Xi

Disclosure: Received consulting fee from Genentech for consulting; Received consulting fee from Centacor for consulting; Received consulting fee from Novartis for review panel membership.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Lawrence H Brent, MD Associate Professor of Medicine, Jefferson Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, American College of Rheumatology

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Janssen<br/>Serve(d) as a speaker or a member of a speakers bureau for: Abbvie; Genentech; Pfizer; Questcor.

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

Kristine M Lohr, MD, MS Professor, Department of Internal Medicine, Interim Chief, Division of Rheumatology, Director, Rheumatology Training Program, University of Kentucky College of Medicine

Kristine M Lohr, MD, MS is a member of the following medical societies: American College of Physicians, American College of Rheumatology

Disclosure: Nothing to disclose.

Acknowledgements

Augusto C Posadas, MD Rheumatologist, Saguaro Physician Group, Tucson, AZ

Disclosure: Abbott Honoraria Speaking and teaching

References
  1. Alpsoy E. Behçet's disease: A comprehensive review with a focus on epidemiology, etiology and clinical features, and management of mucocutaneous lesions. J Dermatol. 2016 Apr 14. [Medline].

  2. Adamantiades B. A case of recurrent hypopyon iritis. Medical Society of Athens. 1930. 586-93.

  3. Behcet H. Uber rezidiverendeaphthose durch ein virus verursachte Geschwure am Mund, am Auge, und an den Genitalien. Dermatol Wochenschr. 1937. 105:1152-7.

  4. International Study Group for Behçet's Disease. Criteria for diagnosis of Behçet's disease. International Study Group for Behçet's Disease. Lancet. 1990 May 5. 335(8697):1078-80. [Medline].

  5. Emmi L, Brugnolo F, Salvati G, et al. Immunopathological aspects of Behçet's disease. Clin Exp Rheumatol. 1995 Nov-Dec. 13(6):687-91. [Medline].

  6. Kaneko S, Suzuki N, Yamashita N, Nagafuchi H, Nakajima T, Wakisaka S, et al. Characterization of T cells specific for an epitope of human 60-kD heat shock protein (hsp) in patients with Behcet's disease (BD) in Japan. Clin Exp Immunol. 1997 May. 108(2):204-12. [Medline].

  7. Hasan A, Fortune F, Wilson A, Warr K, Shinnick T, Mizushima Y, et al. Role of gamma delta T cells in pathogenesis and diagnosis of Behcet's disease. Lancet. 1996 Mar 23. 347(9004):789-94. [Medline].

  8. Suzuki Y, Hoshi K, Matsuda T, et al. Increased peripheral blood gamma delta+ T cells and natural killer cells in Behçet's disease. J Rheumatol. 1992 Apr. 19(4):588-92. [Medline].

  9. Direskeneli H, Eksioglu-Demiralp E, Kibaroglu A, Yavuz S, Ergun T, Akoglu T. Oligoclonal T cell expansions in patients with Behçet's disease. Clin Exp Immunol. 1999 Jul. 117(1):166-70. [Medline]. [Full Text].

  10. Direskeneli H, Eksioglu-Demiralp E, Yavuz S, Ergun T, Shinnick T, Lehner T, et al. T cell responses to 60/65 kDa heat shock protein derived peptides in Turkish patients with Behçet's disease. J Rheumatol. 2000 Mar. 27(3):708-13. [Medline].

  11. Frassanito MA, Dammacco R, Cafforio P, Dammacco F. Th1 polarization of the immune response in Behçet's disease: a putative pathogenetic role of interleukin-12. Arthritis Rheum. 1999 Sep. 42(9):1967-74. [Medline].

  12. Sugi-Ikai N, Nakazawa M, Nakamura S, Ohno S, Minami M. Increased frequencies of interleukin-2- and interferon-gamma-producing T cells in patients with active Behçet's disease. Invest Ophthalmol Vis Sci. 1998 May. 39(6):996-1004. [Medline].

  13. Hamzaoui K, Berraies A, Kaabachi W, Ammar J, Hamzaoui A. Pulmonary manifestations in Behcet disease: impaired natural killer cells activity. Multidiscip Respir Med. 2013 Apr 4. 8(1):29. [Medline].

  14. Hallett MB, Lloyds D. Neutrophil priming: the cellular signals that say 'amber' but not 'green'. Immunol Today. 1995 Jun. 16(6):264-8. [Medline].

  15. Sakane T, Takeno M, Suzuki N, Inaba G. Behçet's disease. N Engl J Med. 1999 Oct 21. 341(17):1284-91. [Medline].

  16. Sakane T, Suzuki N, Takeno M. Innate and acquired immunity in Behçet’s disease. 8th International Congress on Behçet’s Disease. Reggio Emilia, Italy, 7-9 October 1998. Program and Abstracts: 56.

  17. Takeno M, Shimayano Y, Suzuki N, Sakane T. Prolonged survival of autoprimed neutrophils from patients with Behçet ’s disease.: 8th International Congress on Behçet’s Disease. Reggio Emilia, Italy, 7-9 October 1998. Program and Abstracts: 57.

  18. Koné-Paut I, Geisler I, Wechsler B, et al. Familial aggregation in Behçet's disease: high frequency in siblings and parents of pediatric probands. J Pediatr. 1999 Jul. 135(1):89-93. [Medline].

  19. de Menthon M, Lavalley MP, Maldini C, Guillevin L, Mahr A. HLA-B51/B5 and the risk of Behçet's disease: a systematic review and meta-analysis of case-control genetic association studies. Arthritis Rheum. 2009 Oct 15. 61(10):1287-96. [Medline].

  20. Kiraz S, Ertenli I, Oztürk MA, et al. Pathological haemostasis and "prothrombotic state" in Behçet's disease. Thromb Res. 2002 Jan 15. 105(2):125-33. [Medline].

  21. Krause I, Yankevich A, Fraser A, Rosner I, Mader R, Zisman D, et al. Prevalence and clinical aspects of Behcet's disease in the north of Israel. Clin Rheumatol. 2007 Apr. 26(4):555-60. [Medline].

  22. Calamia KT, Wilson FC, Icen M, Crowson CS, Gabriel SE, Kremers HM. Epidemiology and clinical characteristics of Behçet's disease in the US: a population-based study. Arthritis Rheum. 2009 May 15. 61(5):600-4. [Medline]. [Full Text].

  23. Saadoun D, Wechsler B, Desseaux K, et al. Mortality in Behçet's disease. Arthritis Rheum. 2010 Sep. 62(9):2806-12. [Medline].

  24. Akman-Demir G, Serdaroglu P, Tasçi B. Clinical patterns of neurological involvement in Behçet's disease: evaluation of 200 patients. The Neuro-Behçet Study Group. Brain. 1999 Nov. 122 ( Pt 11):2171-82. [Medline].

  25. Gungor AN, Kalkan G, Oguz S, Sen B, Ozoguz P, Takci Z, et al. Behcet disease and pregnancy. Clin Exp Obstet Gynecol. 2014. 41 (6):617-9. [Medline].

  26. Alpsoy E, Zouboulis CC, Ehrlich GE. Mucocutaneous lesions of Behcet's disease. Yonsei Med J. 2007 Aug 31. 48(4):573-85. [Medline].

  27. Calamia KT, Schirmer M, Melikoglu M. Major vessel involvement in Behçet disease. Curr Opin Rheumatol. 2005 Jan. 17(1):1-8. [Medline].

  28. Kobayashi K, Ueno F, Bito S, et al. Development of consensus statements for the diagnosis and management of intestinal Behçet's disease using a modified Delphi approach. J Gastroenterol. 2007 Sep. 42(9):737-45. [Medline].

  29. Hemmen T, Perez-Canto A, Distler A, et al. IgA nephropathy in a patient with Behçet's syndrome--case report and review of literature. Br J Rheumatol. 1997 Jun. 36(6):696-9. [Medline].

  30. Hashimoto T, Toya Y, Kihara M, Yabana M, Inayama Y, Tanaka K, et al. Behçet's disease complicated by IgA nephropathy with nephrotic syndrome. Clin Exp Nephrol. 2008 Jun. 12(3):224-7. [Medline].

  31. Akpolat T, Akkoyunlu M, Akpolat I, et al. Renal Behçet's disease: a cumulative analysis. Semin Arthritis Rheum. 2002 Apr. 31(5):317-37. [Medline].

  32. Kokturk A. Clinical and Pathological Manifestations with Differential Diagnosis in Behçet's Disease. Patholog Res Int. 2012. 2012:690390. [Medline]. [Full Text].

  33. Arevalo JF, Lasave AF, Al Jindan MY, Al Sabaani NA, Al-Mahmood AM, Al-Zahrani YA, et al. Uveitis in Behçet Disease in a Tertiary Center Over 25 Years: The KKESH Uveitis Survey Study Group. Am J Ophthalmol. 2015 Jan. 159(1):177-184.e2. [Medline].

  34. Saadoun D, Wechsler B, Resche-Rigon M, Trad S, Le Thi Huong D, Sbai A, et al. Cerebral venous thrombosis in Behçet's disease. Arthritis Rheum. 2009 Apr 15. 61(4):518-26. [Medline].

  35. Seyahi E, Cakmak OS, Tutar B, Arslan C, Dikici AS, Sut N, et al. Clinical and Ultrasonographic Evaluation of Lower-extremity Vein Thrombosis in Behcet Syndrome: An Observational Study. Medicine (Baltimore). 2015 Nov. 94 (44):e1899. [Medline].

  36. Oktayoglu P, Mete N, Caglayan M, Bozkurt M, Bozan T, Em S, et al. Elevated serum levels of calprotectin (MRP8/MRP14) in patients with Behçet's disease and its association with disease activity and quality of life. Scand J Clin Lab Invest. 2014 Dec 4. 1-7. [Medline].

  37. Hatemi G, Silman A, Bang D, et al. EULAR recommendations for the management of Behçet disease. Ann Rheum Dis. 2008 Dec. 67(12):1656-62. [Medline].

  38. Aktulga E, Altac M, Muftüoglu A, et al. A double blind study of colchicine in Behçet's disease. Haematologica. 1980 Jun. 65(3):399-402. [Medline].

  39. Calis M, Ates F, Yazici C, Kose K, Kirnap M, Demir M, et al. Adenosine deaminase enzyme levels, their relation with disease activity, and the effect of colchicine on adenosine deaminase levels in patients with Behçet's disease. Rheumatol Int. 2005 Aug. 25(6):452-6. [Medline].

  40. Markomichelakis N, Delicha E, Masselos S, Sfikakis PP. Intravitreal infliximab for sight-threatening relapsing uveitis in Behçet disease: a pilot study in 15 patients. Am J Ophthalmol. 2012 Sep. 154(3):534-541.e1. [Medline].

  41. Keino H, Okada AA, Watanabe T, Taki W. Decreased ocular inflammatory attacks and background retinal and disc vascular leakage in patients with Behcet's disease on infliximab therapy. Br J Ophthalmol. 2011 Sep. 95(9):1245-50. [Medline].

  42. Deuter CM, Zierhut M, Mohle A, et al. Long-term remission after cessation of interferon-a treatment in patients with severe uveitis due to Behçet's disease. Arthritis Rheum. 2010 Sep. 62(9):2796-805. [Medline].

  43. Nuñez-Sotelo CM, Gutiérrez-Gonzalez LA, Rodriguez MA. Rapid, complete and sustained response to corticosteroids and pulse cyclophosphamide therapy in a patient with behçet and central nervous disease. Maedica (Buchar). 2012 Dec. 7(4):348-51. [Medline]. [Full Text].

  44. Borhani Haghighi A, Pourmand R, Nikseresht AR. Neuro-Behçet disease. A review. Neurologist. 2005 Mar. 11(2):80-9. [Medline].

  45. Ueda A, Takeno M, Ishigatsubo Y. Adalimumab in the management of Behçet's disease. Ther Clin Risk Manag. 2015. 11:611-9. [Medline].

  46. Melikoglu M, Fresko I, Mat C, Ozyazgan Y, Gogus F, Yurdakul S, et al. Short-term trial of etanercept in Behçet's disease: a double blind, placebo controlled study. J Rheumatol. 2005 Jan. 32 (1):98-105. [Medline].

  47. Vallet H, et al; French Behçet Network. Efficacy of anti-TNF alpha in severe and/or refractory Behçet's disease: Multicenter study of 124 patients. J Autoimmun. 2015 Aug. 62:67-74. [Medline].

  48. Takayama K, Ishikawa S, Enoki T, Kojima T, Takeuchi M. Successful Treatment with Infliximab for Behçet Disease during Pregnancy. Ocul Immunol Inflamm. 2013 Apr 25. [Medline].

  49. Davatchi F, Shams H, Rezaipoor M, Sadeghi-Abdollahi B, Shahram F, Nadji A, et al. Rituximab in intractable ocular lesions of Behcet's disease; randomized single-blind control study (pilot study). Int J Rheum Dis. 2010 Aug. 13 (3):246-52. [Medline].

  50. Sadreddini S, Noshad H, Molaeefard M, Noshad R. Treatment of retinal vasculitis in Behçet's disease with rituximab. Mod Rheumatol. 2008. 18 (3):306-8. [Medline].

  51. Perez-Pampin E, Campos-Franco J, Blanco J, Mera A. Remission induction in a case of refractory Behçet disease with alemtuzumab. J Clin Rheumatol. 2013 Mar. 19(2):101-3. [Medline].

  52. Mesquida M, Victoria Hernández M, Llorenç V, et al. Behçet disease-associated uveitis successfully treated with golimumab. Ocul Immunol Inflamm. 2013 Apr. 21(2):160-2. [Medline].

  53. Hatemi G, Melikoglu M, Tunc R, Korkmaz C, Turgut Ozturk B, Mat C, et al. Apremilast for Behçet's syndrome--a phase 2, placebo-controlled study. N Engl J Med. 2015 Apr 16. 372 (16):1510-8. [Medline].

  54. Estrach C, Mpofu S, Moots RJ. Behçet's syndrome: response to infliximab after failure of etanercept. Rheumatology (Oxford). 2002 Oct. 41(10):1213-4. [Medline].

  55. Sfikakis PP, Markomichelakis N, Alpsoy E, et al. Anti-TNF therapy in the management of Behcet's disease--review and basis for recommendations. Rheumatology (Oxford). 2007 May. 46(5):736-41. [Medline].

 
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Oral aphthous ulcers secondary to Behçet disease.
 
 
 
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