Lenalidomide: An Alternative Treatment for Refractory Behçet’s Disease and Relapsing Polychondritis
  • Sofia Gomes Brazao
    Internal Medicine, Centro Hospitalar e Universitário de Coimbra - Faculty of Medicine, University of Coimbra, Coimbra, Portugal
  • Jorge Crespo
    Internal Medicine, Centro Hospitalar e Universitário de Coimbra - Faculty of Medicine, University of Coimbra, Coimbra, Portugal
  • Armando Carvalho
    Internal Medicine, Centro Hospitalar e Universitário de Coimbra - Faculty of Medicine, University of Coimbra, Coimbra, Portugal

Keywords

Behçet’s disease, MAGIC syndrome, lenalidomide, aphtosis

Abstract

Introduction:Behçet’s disease (BD) is a chronic systemic condition characterized by recurrent oral and genital ulcers, neurological and vascular manifestations, ocular and skin lesions, and a positive pathergy test. Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome is rare and consists of features of BD together with relapsing polychondritis. The treatment of this syndrome is similar that of BD and depends on disease expression and symptoms.
Case Report: We report the case of a 50-year-old man diagnosed with BD with relapsing polychondritis (MAGIC syndrome) which had evolved over approximately 35 years, with exuberant and frequent oral and genital ulcers. Various treatments were unsuccessful and had adverse effects. Lenalidomide was initiated at a dose of 5 mg per day with remission of oral and genital ulcers and no haematological or adverse effects.
Conclusion: BD is a complex disorder with a variety of presentations. Its treatment can be challenging but sometimes off-label drugs, like lenalidomide, can be effective.

VIEW THE ENTIRE ARTICLE

References

  • Criteria for diagnosis of Behçet's disease. International Study Group for Behçet's Disease. Lancet 1990;335:1078–1080.

  • International Team for the Revision of the International Criteria for Behçet's Disease (ITR-ICBD). The International Criteria for Behçet's Disease (ICBD): A collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatol Venereol 2014;28:338–347.

  • Kaneko Y, Nakai N, Kida T, Kawahito Y, Katoh N. Mouth and genital ulcers with inflamed cartilage syndrome: case report and review of the published work. Indian J Dermatol 2016;61(3):347.

  • Hatemi G, Christensen R, Bang D, et al. 2018 update of the EULAR recommendations for the management of Behçet's syndrome. Ann Rheum Dis 2018;77:808–818.

  • Ordi-Ros J, Cosiglio FJ. Indicaciones terapéuticas actuales de la talidomida y la lenalidomida. Medicina Clínica 2014;142(8):360–364.

  • Tseng S, Pak G, Washenik K, Keltz Pomeranz M, Shupack JL. Rediscovering thalidomide: a review of its mechanism of action, side effects, and potential uses. J Am Acad Dermatol 1996;35(6):969–979.

  • Tanida S, Inoue N, Kobayashi K, et al. Adalimumab for the treatment of Japanese patients with intestinal Behcet’s disease. Clin Gastroenterol Hepatol 2015;13:940–948.

  • Sim JK, Lee SY, Shim JJ, Kang KH. Pulmonary sarcoidosis induced by adalimumab: a case report and literature review. Yonsei Med J 2016;57(1):272–273.

  • Zeldis JB, Knight R, Hussein M, Chopra R, Muller G. A review of the history, properties, and use of the immunomodulatory compound lenalidomide. Ann NY Acad Sci 2011;1222(1):76–82.

  • Anderson KC. Lenalidomide and thalidomide: mechanisms of action—similarities and differences. Semin Hematol 2005;42:S3–S8.

  • Green J, Upjohn E, McCormack C, Zeldis J, Prince HM. Successful treatment of Behçet’s disease with lenalidomide. Br J Dermatol 2008;158(1):197–198.

  • Ossorio-García L, Jiménez-Gallo D, de la Varga-Martínez R, Linares-Barrios M. Lenalidomide for treatment of recurrent oral aphthae in Adamantiades-Behçet’s disease. J Deutsch Dermatol Ges 2018;16(8):1029–1031.

  • Kalampokis I, Rabinovich CE. Successful management of refractory pediatric-onset complex aphthosis with lenalidomide. J Clin Rheumatol 2014;20(4):221–223.

  • Kanavy H, Bahner J, Korman NJ. Treatment of refractory prurigo nodularis with lenalidomide. Arch Dermatol 2012;148(7):794.

  • Braunstein I, Goodman NG, Rosenbach M, Okawa J, Shah A, Krathen M, et al. Lenalidomide therapy in treatment-refractory cutaneous lupus erythematosus: histologic and circulating leukocyte profile and potential risk of a systemic lupus flare. J Am Acad Dermatol 2012;66(4):571–582.
  • Views: 1481
    HTML downloads: 122
    PDF downloads: 535


    Published: 2019-05-31
    Issue: Vol 6 No 6 (view)


    How to cite:
    1.
    Brazao SG, Crespo J, Carvalho A. Lenalidomide: An Alternative Treatment for Refractory Behçet’s Disease and Relapsing Polychondritis. EJCRIM 2019;6 doi:10.12890/2019_001117.

    Similar Articles

    You may also start an advanced similarity search for this article.

    Most read articles by the same author(s)

    1 2 > >>