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Lenalidomide: An Alternative Treatment for Refractory Behçet’s Disease and Relapsing Polychondritis

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 consi...

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Autores principales: Brazão, Sofia Gomes, Crespo, Jorge, Carvalho, Armando
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SMC Media Srl 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6601695/
https://www.ncbi.nlm.nih.gov/pubmed/31293992
http://dx.doi.org/10.12890/2019_001117
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author Brazão, Sofia Gomes
Crespo, Jorge
Carvalho, Armando
author_facet Brazão, Sofia Gomes
Crespo, Jorge
Carvalho, Armando
author_sort Brazão, Sofia Gomes
collection PubMed
description 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. LEARNING POINTS: Behçet’s disease (BD) is a chronic systemic autoinflammatory disease whose treatment can be challenging and is dependent on disease expression and symptoms. Thalidomide can be effective for treating recurrent oral aphthae, but its use is limited by adverse effects, mostly sensory polyneuropathy. Lenalidomide is a more powerful thalidomide analogue with fewer adverse effects and may be effective for the treatment of BD.
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spelling pubmed-66016952019-07-10 Lenalidomide: An Alternative Treatment for Refractory Behçet’s Disease and Relapsing Polychondritis Brazão, Sofia Gomes Crespo, Jorge Carvalho, Armando Eur J Case Rep Intern Med Articles 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. LEARNING POINTS: Behçet’s disease (BD) is a chronic systemic autoinflammatory disease whose treatment can be challenging and is dependent on disease expression and symptoms. Thalidomide can be effective for treating recurrent oral aphthae, but its use is limited by adverse effects, mostly sensory polyneuropathy. Lenalidomide is a more powerful thalidomide analogue with fewer adverse effects and may be effective for the treatment of BD. SMC Media Srl 2019-05-31 /pmc/articles/PMC6601695/ /pubmed/31293992 http://dx.doi.org/10.12890/2019_001117 Text en © EFIM 2019 This article is licensed under a Commons Attribution Non-Commercial 4.0 License (https://creativecommons.org/licenses/by-nc/4.0/)
spellingShingle Articles
Brazão, Sofia Gomes
Crespo, Jorge
Carvalho, Armando
Lenalidomide: An Alternative Treatment for Refractory Behçet’s Disease and Relapsing Polychondritis
title Lenalidomide: An Alternative Treatment for Refractory Behçet’s Disease and Relapsing Polychondritis
title_full Lenalidomide: An Alternative Treatment for Refractory Behçet’s Disease and Relapsing Polychondritis
title_fullStr Lenalidomide: An Alternative Treatment for Refractory Behçet’s Disease and Relapsing Polychondritis
title_full_unstemmed Lenalidomide: An Alternative Treatment for Refractory Behçet’s Disease and Relapsing Polychondritis
title_short Lenalidomide: An Alternative Treatment for Refractory Behçet’s Disease and Relapsing Polychondritis
title_sort lenalidomide: an alternative treatment for refractory behçet’s disease and relapsing polychondritis
topic Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6601695/
https://www.ncbi.nlm.nih.gov/pubmed/31293992
http://dx.doi.org/10.12890/2019_001117
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