Interferon therapy for Behçet's disease

doi:10.1016/S0190-9622(94)70226-8

Journal of the American Academy of Dermatology

Volume 31, Issue 4, October 1994, Pages 617-619

https://doi.org/10.1016/S0190-9622(94)70226-8 Get rights and content

Background: Behçet's disease was first described in 1937 as a trisymptom complex. No standard therapy has been established. Its cause is presumed to be viral or immunologic.

Objective: We sought to determine whether patients with Behçet's disease benefit from interferon, which has antiviral, immunomodulatory, antiproliferative, and antitumoral properties.

Methods: Fourteen patients were initially given 3 MU interferon alfa-2a subcutaneously three times per week. The doses were gradually increased to 12 MU. Each patient received a total dose of 216 MU during the 2-month study. Therapy with 9 MU interferon alfa-2a once a month was continued in half the patients. All patients were observed before and after treatment for 6-month periods, and all symptoms were recorded.

Results: All patients were symptom free by the end of the 2-month treatment period. The frequencies of oral and genital ulcers as well as pustular vasculitis decreased significantly in the posttreatment period as compared with the pretreatment period.

Conclusion: The data suggest that interferon treatment appears to be effective for patients with Behçet's disease.

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Interferon-alpha was beneficial for the treatment of arthritis in 4 observational studies. Three of these reported complete response in all of 11, 24 and 8 patients, respectively and one showed a significant decrease in the mean duration and the frequency of arthritis in 9 patients as explained in more detail in the Online Supplement [38–41]. Four studies reported favourable results with infliximab for joint involvement.
The aim of this systematic review was to inform the update of European League Against Rheumatism (EULAR) Recommendations for the management of Behçet’s syndrome (BS), on the evidence for the treatment of skin, mucosa and joint involvement of BS.
A systematic literature search, data extraction, statistical analyses and assessment of the quality of evidence were performed according to a pre-specified protocol using the PRISMA guidelines. Studies that assessed the efficacy of an intervention in comparison to an active comparator or placebo for oral ulcers, genital ulcers, papulopustular lesions, nodular lesions or arthritis were included. Where possible, risk ratios were calculated for binary outcomes and mean difference for continuous outcomes.
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Behçet's syndrome (BS) is a multisystem vasculitis with an unknown etiology and an unique geographic distribution. The pathogenesis of BS is complex and currently not fully understood. Since the 1970's there have been different attempts to classify BS within the specific group of diseases such as spondyloarthritis, autoimmune and more recently autoinflammatory disease. However current evidence suggests that BS does not easily fit into any one of these diseases and we propose the possibility that the construct we take as BS might represent more than one disease.
The management of BS usually depends on clinical presentation and organ involvement. Traditional immunosuppressives and concomitant use of corticosteroids have been the main therapeutic choice for many years. There is growing experience with biologic therapeutics especially with tumor necrosis factor-alpha (TNF-alpha) antagonists in the management of refractory eye, gastrointestinal and neurologic involvement in BS.
In this review we have proposed a different perspective about the disease mechanisms of BS and have summarized the current management strategies in BS in the light of available evidence.
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This disease can also involve the gastrointestinal, pulmonary, cardiovascular, renal, urologic, musculoskeletal, and central nervous systems. Interferon alfa has been shown to effectively treat Behçet's disease in one randomized, placebo-controlled, double-blind study,165 several prospective, open-label studies,166-174 and one retrospective study175 (Table XII). Topical interferon alfa does not appear to be effective in the treatment of oral disease.176
The introduction of a number of biologic therapies into the market has revolutionized the practice of dermatology. These therapies include interferons, intravenous immunoglobulin, infliximab, adalimumab, etanercept, efalizumab, alefacept, and rituximab. Most dermatologists are familiar with the Food and Drug Administration–approved indications of these medications. However, numerous off-label uses have evolved. As part 1 of a 2-part series, this article will review the literature regarding the off-label uses of the interferons and intravenous immunoglobulin in dermatology.
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¹: From the Department of Dermatology, Akdeniz University Medical Faculty.

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Interferon therapy for Behçet's disease

Lancet

J Am Acad Dermatol

Über rezidivierende aphthöse, durch ein Virus Verursachte Geschwüre am Mund, am auge, und an den Genitalien

Dermatol Wochenschr

The oral cavity

Behçet's disease

Behçet's syndrome

N Engl J Med

Blood vessel-based inflammatory disorder