Although the precise pathoetiology of Behcet's disease (BD) remains obscure, patients with BD have a high incidence of chronic infectious foci, indicating an enhanced susceptibility to chronic tonsillitis, and dental caries. Sometimes, clinical symptoms appear after treatment of these foci in BD patients. It is believed that BD might be related to an allergic reaction to a bacterial infection in view of the many clinical symptoms, especially the presence of aphthous and genital ulcerations. An attempt to obtain cutaneous responses to bacterial antigens has been carried out using various vaccines developed from bacteria isolated from the ulcerative lesions and oral cavities of BD patients. BD patients often show intense hypersensitivity to various strains of streptococci, not only by their cutaneous reactions but also by in vitro testing. In this report, we describe our previous studies on the correlation between streptococcal antigens and the pathogenesis of BD and also discuss the recent reports of other authors. The intense hypersensitivity to streptococcal antigens acquired after streptococcal infection is thought to play an important role in the appearance of symptoms in BD patients since the production of pro-inflammatory cytokines by peripheral blood mononuclear cells (PBMC) was enhanced when stimulated with streptococcal antigen in a culture system. Minocycline, an antibiotic to which certain strains of streptococci are sensitive, reduced the frequency of clinical symptoms in BD patients as well as the production of pro-inflammatory cytokines by BD-PBMC stimulated with streptococcal antigen.
Adult ; Antibiotics, Tetracycline/therapeutic use* ; Antigens, Bacterial/immunology ; Behcet's Syndrome/immunology ; Behcet's Syndrome/etiology* ; Behcet's Syndrome/drug therapy ; Cytokines/biosynthesis ; Female ; Human ; Male ; Minocycline/therapeutic use* ; Skin Tests ; Streptococcal Infections/drug therapy ; Streptococcal Infections/complications*

Behcet syndrome (BS) is a chronic systemic inflammatory disorder involving vessels of all sizes, characterized by relapsing episodes of oral and/or genital ulcers, as well as skin lesions. Ocular, vascular, gastrointestinal, neurological system involvement can cause significant morbidity and mortality. Glucocorticoids and immunosuppressants are the cornerstones for the management of BS. Biologic agents has been recommended for severe and/or refractory BS. Interferon-α (IFN-α) had multiple biological effects, such as antiviral and antiproliferative, that could regulate both innate and adaptive immunity in BS. Growing evidence showed the efficacy of IFN-α in severe and/or refractory BS. Many studies have demonstrated that IFN-α has comparable effectiveness and tolerance profiles as anti-tumor necrosis factor (TNF) agents for Behcet's uveitis with a much lower cost and steroid-and immunosuppressant-sparing effects. IFN-α has been recommended as second-line therapy for ocular involvement of BS in EULAR (The European League Against Rheumatism) 2018. IFN-α also improves mucocutaneous lesions in BS with the dosage from 3 to 9-12 million IU three times per week. A few cases indicated the therapeutic potential of IFN-α in intestinal BS. As a new trial of IFN-α in vascular BS (VBS), a recent study revealed the lower relapse rate and higher recanalization rate with IFN-α in lower extremity deep vein thrombosis (DVT). Another two case reports presented the efficacy of IFN-α in pulmonary artery involvement in BS. Also, case reports have shown successful treatment in refractory neurological involvement. There are two subtypes of IFN-α commonly used in autoimmune diseases, named IFN-α2a and IFN-α2b. IFN-α2a seemed more effective than IFN-α2b, especially in ocular and mucocutaneous involvement of BS. Side effects of IFN-α are dose-dependent and not severe. The most frequent side effects are flu-like syndrome, mild leukopenia and alopecia. Considering the potential risk of tuberculosis (TB) and hepatitis B virus (HBV) reactivation of TNF-α inhibitors, IFN-α is safe due to its anti-HBV effect and protective effect on TB. In conclusion, IFN-α is a promising choice for severe and/or refractory BS patients, especially for those who are intolerant or contraindicant to other biological agents, such as TNF inhibitors. Further prospective controlled studies are warranted to confirm the efficacy and safety of IFN-α in BS.
Behcet Syndrome/drug therapy* ; Glucocorticoids ; Humans ; Immunosuppressive Agents ; Uveitis ; Venous Thrombosis

Adult ; Behcet Syndrome ; complications ; drug therapy ; Drugs, Chinese Herbal ; therapeutic use ; Humans ; Male ; Uveitis ; complications ; drug therapy

Behcet's disease is particularly prevalent in "Silk Route" populations, but it has a global distribution. The diagnosis of the disease is based on clinical criteria as there is as yet no pathognomonic test, and mucocutaneous lesions, which figure prominently in the presentation and diagnosis, may be considered the diagnostic hallmarks. Among the internationally accepted criteria, painful oral and genital ulcers, cutaneous vasculitic lesions and reactivity of the skin to needle prick or injection (the pathergy reaction) are considered hallmarks of Behcet's disease, and often precede other manifestations. Their recognition may permit earlier diagnosis and treatment, with salutary results. This paper describes the various lesions that constitute the syndrome and focuses on those that may be considered characteristic.
Behcet Syndrome/drug therapy/*pathology ; Female ; Humans ; Male ; Oral Ulcer/drug therapy/pathology ; Skin Ulcer/drug therapy/pathology ; Thrombophlebitis/drug therapy/pathology

A patient with Behcet's syndrome in whom multiple pulmonary artery aneurysms were completely resolved after a combined treatment with corticosteroid and chlorambucil is reported.
Aneurysm/*drug therapy ; Behcet Syndrome/*drug therapy ; Case Report ; Chlorambucil/*therapeutic use ; Drug Therapy, Combination ; Human ; Male ; Prednisone/*therapeutic use ; *Pulmonary Artery

Approximately 50 % of patients with scleritis are known to be associated with systemic connective tissue diseases or vasculitic diseases such as rheumatoid arthritis (RA), Wegener's granulomatosis, relapsing polychondritis, and systemic lupus erythematosus. The patients with scleritis in association with Behcet's disease (BD) have been rarely described in the literature. We report a 46-year-old female patient with BD who developed the nodular scleritis.
Anti-Inflammatory Agents, Non-Steroidal/therapeutic use ; Behcet's Syndrome/drug therapy ; Behcet's Syndrome/diagnosis* ; Behcet's Syndrome/complications* ; Case Report ; Cyclophosphamide/therapeutic use ; Drug Therapy, Combination ; Female ; Follow-Up Studies ; Human ; Middle Age ; Prednisolone/therapeutic use ; Scleritis/drug therapy ; Scleritis/diagnosis* ; Scleritis/complications*

Behcet's disease (BD) is a chronic inflammatory condition involving several organs, such as skin, mucous membrane, eye, joint, intestine, lung and central nervous system. Ankylosing spondylitis (AS) is a prototype of seronegative spondyloarthropathy, and a chronic systemic inflammatory disorder of the axial skeleton, mainly affecting the sacroiliac joint and spine. In the latter, systemic complications may develop in addition to joint involvement. The coexistence of BD and AS has been rarely reported in the literature. The inclusion of BD among seronegative spondyloarthritides and whether sacroiliitis (SI) develops in BD are still being debated. We describe a 28-year-old man who has fulfilled the diagnostic criteria for BD and AS as well.
Adult ; Angiography ; Anti-Inflammatory Agents/administration +ACY- dosage ; Behcet's Syndrome/drug therapy ; Behcet's Syndrome/diagnosis+ACo- ; Behcet's Syndrome/complications+ACo- ; Case Report ; Drug Therapy, Combination ; Human ; Magnetic Resonance Imaging ; Male ; Spondylitis, Ankylosing/drug therapy ; Spondylitis, Ankylosing/diagnosis+ACo- ; Spondylitis, Ankylosing/complications+ACo- ; Treatment Outcome

Behcet's disease is a chronic multisystemic disorder involving many organs and characterized by recurrent oral and genital ulcers and relapsing iritis. A case of BD with large vein thrombosis involving superior and inferior vena cava is presented. Large vein thrombosis in BD is not commonly developed and most commonly observed in the inferior or superior vena cava. A review of the literature emphasizes the rarity of the combined superior and inferior vena caval occlusion. Existence of extensive large vein occlusion in BD is associated with limited therapy and poor prognosis.
Adult ; Behcet's Syndrome/diagnosis ; Behcet's Syndrome/complications* ; Disease Progression ; Fatal Outcome ; Female ; Fibrinolytic Agents/therapeutic use ; Human ; Phlebography ; Thrombosis/etiology* ; Thrombosis/drug therapy ; Thrombosis/diagnosis ; Vena Cava, Inferior/radiography* ; Vena Cava, Superior/radiography* ; Substances: Fibrinolytic Agents

OBJECTIVE: To explore the effectiveness and safety of golimumab in the treatment of severe/refractory cardiovascular Behcet syndrome (BS). METHODS: We retrospectively analyzed the clinical data of nine patients diagnosed with severe/refractory cardiovascular BS and treated with golimumab from February 2018 to July 2020 in Peking Union Medical College Hospital. We analyzed levels of erythrocyte sedimentation rate (ESR) and high-sensitivity C-reactive protein (hsCRP), imaging findings, and the doses of glucocorticoids and immunosuppressive agents during the period of combined treatment with golimumab. RESULTS: Nine patients were enrolled, including 8 males and 1 female, with a mean age and median course of (37.0±8.6) years and 120 (60, 132) months, respectively. Seven patients presented with severe aortic regurgitation combined with other cardiovascular involvement secondary to BS. Two patients presented with large vessel involvement, including multiple aneurysms and vein thrombosis. Prior to golimumab treatment, seven patients were treated with glucocorticoids and multiple immunosuppres-sants [with a median number of 3 (1, 3) types] while still experienced disease progression or elevated inflammation biomarkers during postoperative period. Eight patients with disease progression, uncontrolled inflammation and history of severe postoperative complications required effective and fast control of inflammation during perioperative period. One patient had adverse reaction with tocilizumab and switched to golimumab during perioperative period. The patients were treated with golimumab 50 mg every 4 weeks, along with concomitant treatment of glucocorticoid and immunosuppressants. After a median follow-up of (16.3±5.6) months, all the patients achieved clinical improvement. Vascular lesions were radiologically stable and no vascular progressive or newly-onset of vascular lesions was observed. The eight patients who experienced cardiac or vascular operations showed no evidence of postoperative complications. The ESR and hsCRP levels decreased significantly [16.5 (6.8, 52.5) mm/h vs. 4 (2, 7) mm/h, and 21.24 (0.93, 32.51) mg/L vs. 0.58 (0.37, 1.79) mg/L (P < 0.05), respectively]. The dose of prednisone was tapered from 35 (15, 60) mg/d to 10.0 (10.0, 12.5) mg/d. No prominent adverse reactions were observed. CONCLUSION Our study suggests that golimumab is effective in the treatment of severe/refractory cardiovascular BS. Combination immunosuppression therapy with golimumab contributes to control of inflammation, reduction of postoperative complications and tapering the dose of glucocorticoids or immunosuppressants.
Adult ; Antibodies, Monoclonal/therapeutic use* ; Behcet Syndrome/drug therapy* ; Drug Therapy, Combination ; Female ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Treatment Outcome

PURPOSE: Behcet's disease (BD) is rarely reported to be associated with malignancies in the literature. However, the frequency of cancer in BD patients remains unknown. This study evaluated cancer morbidity in BD patients compared with that in the general population of Korea. MATERIALS AND METHODS: A retrospective chart review was performed on 506 patients visiting our hospital from 1994 to 2011 for BD. We analyzed the standardized morbidity rate (SMR), which is the ratio of observed to expected malignancies. Furthermore, we reviewed cases of solid cancer in BD patients in the literature. RESULTS: Of the 506 patients with BD, 11 (2.17%) developed cancer. We found a variety of solid cancers without predominance and no hematologic malignancies. The total number of cancers observed was less than expected, which was determined from the statistical data of the National Cancer Information Center of Korea, with an SMR of 0.023 (95% confidence interval, 0.012-0.039). CONCLUSION: BD may be associated with a lower cancer-related morbidity compared with the general population of Korea.
Adult ; Behcet Syndrome/*complications/drug therapy/epidemiology ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Neoplasms/*epidemiology/etiology/surgery ; Republic of Korea/epidemiology ; Retrospective Studies