Improvement of neurological and ocular symptoms of Behçet’s disease after the introduction of infliximab
- 248 Downloads
Behçet’s disease is a chronic inflammatory condition of unknown origin characterized by multiple organ involvement. The most common symptoms of Behçet’s disease are recurrent oral and/or genital ulcerations in combination with symptoms affecting eyes, skin, central and peripheral nervous system, blood vessels and gastrointestinal tract. We present a 43-year-old female patient with the history of recurrent episodes of genital and oral ulcerations, elevated acute phase reactants and skin lesions. The diagnosis of Behçet’s disease has been delayed (for more than 10 years) and reached only after she developed neurological and ocular symptoms. Treatment with glucocorticoids and azathioprine was partially successful. High doses of glucocorticoids were needed to control the disease and cyclosporine A was nephrotoxic. Remission was reached after the introduction of infliximab (plus methotrexate) and glucocorticoids were stopped. In the recent years, infliximab has been accepted as a standard therapy for refractory cases of Behçet’s disease (neurological, ocular or gastrointestinal). Our patient presented with refractory ocular and neurological symptoms and infliximab was effective for both manifestations. Long-term side-effects of glucocorticoids and other immunosuppressants can be avoided with TNF-α blockade. We emphasize the importance of a timely and accurate diagnosis and significance of excluding more common diseases in a work-up algorithm.
KeywordsBehçet’s disease Neurologic and ocular involvement Glucocorticoids Infliximab
Concept: MR, MB; design: MB; supervision: BA; materials: MH, NV; data collection and/or processing: MH, NV; analysis and/or interpretation: BA; literature search: MR; writing manuscript: MR, MB; critical review: BA.
This research received no specific Grant from any funding agency in the public, commercial, or not-for-profit sectors.
Compliance with ethical standards
Conflict of interest
The authors have no conflicts of interest to declare.
All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
Informed consent was obtained from all individual participants included in the study.
- 6.Seaman G, Pearce RA (1997) Behcet’s disease manifestation in a population drawn from the UK Behcet’s syndrome society. In: Hamza M (ed). Behcet’s Disease, Pub Adhoua, Tunisia. pp 196–199Google Scholar
- 8.International Study Group for Behçet’s Disease (1990) Criteria for diagnosis of Behçet’s disease. Lancet Lond Engl 335(8697):1078–1080Google Scholar
- 10.Mege JL, Dilsen N, Sanguedolce V, Gul A, Bongrand P, Roux H et al (1993) Overproduction of monocyte derived tumor necrosis factor alpha, interleukin (IL) 6, IL-8 and increased neutrophil superoxide generation in Behçet’s disease. A comparative study with familial Mediterranean fever and healthy subjects. J Rheumatol 20(9):1544–1549PubMedGoogle Scholar
- 13.Mochizuki M, Akduman L, Nussenblatt R. Behc¸et’s disease. In: Pepose JS, Holland GN, Wilhelmus KR (eds). Ocular infection and immunity. St Louis: Mosby. 1996;663–675Google Scholar
- 34.Hibi T, Hirohata S, Kikuchi H, Tateishi U, Sato N, Ozaki K et al (2016) Infliximab therapy for intestinal, neurological, and vascular involvement in Behcet disease: Efficacy, safety, and pharmacokinetics in a multicenter, prospective, open-label, single-arm phase 3 study. Medicine (Baltimore) 95(24):e3863CrossRefGoogle Scholar