Ulcerative colitis followed by the development of Behçet's disease

A 34-year-old man who had a history of ulcerative colitis (UC) was admitted to our hospital with complaints of arthralgia, erythema nodosum, recurrent oral aphthous ulcers and bloody stools. A colonoscopy revealed multiple aphthous ulcers on his cecum and colon and also revealed a transmural ulcer on his rectum consistent with a diagnosis of UC. The patient was HLA-B51 positive. Based on clinical evidence [recurrent oral ulcers, skin lesions (erythema nodosum), positivity for pathergy test] this patient was diagnosed as having Beh?et's disease with gastrointestinal involvement. We describe this rare case of Beh?et's disease with colitis and discuss the difficulties in making a differential diagnosis between Beh?et's disease and the inflammatory bowel diseases.     

Interferons and Behçet's disease

BACKGROUND: Beh?et's disease is a vasculitis of unknown origin. Various therapeutic agents have been proposed. Interferon alpha 2a or 2b appear to be the most promising. CURRENT KNOWLEDGE AND KEY POINTS: Numerous anecdotal reports and some short series have shown the efficacy of interferon in some cases previously resistant to conventional therapy, particularly to immunosuppressors. Posology, depending on the author, may vary from three million three times a week to 18 million three times a week. Interferon alpha is useful in cases of ocular, muco-cutaneous and articular involvement. Data is lacking for digestive, vascular and neurological manifestations. FUTURE PROSPECTS AND PROJECTS: Multicenter trials will nevertheless be required to determine the duration of therapy, risk of relapses, associated therapy and the optimal time to commence interferon treatment.     

A case of atrioventricular complete block due to Behçet's disease

The cardiovascular system is involved in 5% of the cases of Beh?et's disease. Thrombophlebitis. aneurysm in arteries, pericarditis, myocarditis, valvular disease, ventricular arrhythmia, and conduction system disorders may occur. A case of Beh?et's disease with complete proximal atrioventricular block is presented.     

Behçet's disease

PURPOSE OF REVIEW: To summarize recent scientific developments in the epidemiology, genetics, pathogenesis and treatment of Beh?et's disease. RECENT FINDINGS: Important genetic and immunologic studies were performed. Tumor necrosis factor-alpha-1031C allele was associated with disease susceptibility. Polymorphisms in interleukin-10, IL-8 and CD28 genes were also associated with Beh?et's disease. Association with endothelial nitric oxide synthase gene polymorphism was confirmed but was ethnic related. Significant T helper type 1 immune reaction was reconfirmed in recent studies, especially during active phases, but T helper type 2 reaction may also play a role. Interleukin-12B heterozygosity is associated with Beh?et's disease susceptibility and plays an important role in mediating T helper type 1 antistreptococcal immune response. Selenium binding protein may be a target antigen in Beh?et's uveitis. Pathergy reaction is most frequently positive in the forearm; multiple needle pricks increase positive rate. Experience with anti-tumour necrosis factor therapy for various manifestations is increasing. Cyclosporin A treatment may be associated with new onset of neuro-Beh?et. There is a high prevalence of headaches with moderate or severe disability. Cardiac manifestations include left ventricular dysfunction and coronary flow abnormalities. Anti-Saccharomyces cerevisiae antibodies may be especially common in intestinal Beh?et's disease and are also increased in healthy relatives of patients. SUMMARY: Considerable progress has been made, particularly in understanding the immunologic and genetic basis of the disease. The importance of novel serological markers and autoantigens merits further investigations.     

Beneficial effects of interferon-alpha in a case with Behçet's disease

At the age of 20 years, a Japanese man with recurrent oral aphthae, genital ulcers, folliculitis, erythema nodosum, episodic arthritis and epididymitis was diagnosed as having Behcet's disease (BD) in 1966. He has had active ocular manifestations of BD since 1990. These symptoms recurred and never abated for a long period of time. A right renal cell carcinoma developed and he underwent right nephrectomy in April 1996. Treatment with interferon-alpha was started from June 1996 as supplemental chemotherapy. No active phase developed during administration of IFN-alpha. We suggest that IFN-alpha may play a role as an immunomodulatory agent in BD.     

Behçet's disease associated with Crohn's disease

INTRODUCTION: The association of Beh?et's disease and of Crohn's disease is exceptional, and raises a nosological problem. CASE REPORT: A 24 year-old female developed since 1996 chronic diarrhea, abdominal pain, recurrent oral and genital ulcer and polyarthralgia. Endoscopic colonoscopy examination showed a diffuse colitis. Histological examination revealed epitheloid granuloma without vasculitis compatible with a Crohn's disease. The patient was treated with oral prednisone (1mg/kg/day). In November 1998, she was admitted for diarrhea. Many pseudofolliculitis lesions, uveitis and positive pathergy test were noted. The HLA was B 51. The diagnosis of Crohn's disease associated with Beh?et's disease was made. She was treated with high doses of prednisone (1mg/kg/day and 6 monthly intravenous pulses of cyclophosphamide). Skin lesions and diarrhea improved within few days, and cleared completely within five months. Presently, the patient remains clinically free of disease. CONCLUSION: While being of different pathogenic origin, Beh?et's disease and Crohn's disease may coexist within one and the same patient and cause diagnostic and therapeutic problems.     

Immunopathogenesis of Behçet's disease

A working hypothesis is postulated for the immunopathogenesis of Beh?et's disease (BD). Microbial infection induced stress upregulates HSP65 and MICA gene product which stimulate gamma delta and alpha beta T cell receptor positive cells, to generate effector and suppressor T cells (fig. 1). These activated T cells, antibodies and neutrophils induce cytokines to modulate the immune response. The end result of these complex cellular and cytokine immune interactions in HLA-B51 or genetically related subjects is to induce pathological changes consistent with BD.     

Behçet's disease

Beh?et's disease (BD) is a chronic, relapsing multisystem vasculitis with predominant involvement of the oral and genital mucosa. It has a worldwide distribution, but the prevalence is highest in Central Asia and the Far East (along the ancient 'Silk Route'). Genetic, environmental, immunological and haemostatic factors play a role in the aetiopathogenesis. The International Study Group for BD proposed criteria for the diagnosis of this condition, the essential feature being recurrent oral ulceration. Genital ulcers and skin manifestations are common, while ocular changes are the most important cause of morbidity. Almost any organ in the body can be involved, and systemic involvement may portend a poorer prognosis. There is no pathognomonic test for BD and the diagnosis is made on clinical findings. Treatment of BD would require multidisciplinary cooperation, and early referral to an ophthalmologist is advisable to prevent ocular morbidity. Topical and systemic agents (colchicine, dapsone and thalidomide) are useful in controlling exacerbation of the oral and genital ulcers. Severe disease may require immunosuppressive agents and, more recently, biological agents have been used successfully. It tends to follow an unpredictable course, and the eventual prognosis depends on the systemic involvement.     

Behçet's disease

Beh?et's disease (BD) is a systemic disorder characterized by recurrent attacks of acute inflammation. Major symptoms are oral aphthous ulcers, uveitis, skin lesions, and genital ulcerations. Involvement of vessels, gastrointestinal (GI) tract, and central nervous system (CNS) is less frequent but is associated with a poor prognosis. Pulmonary complications of BD include aneurysms of the aorta, great vessels, or pulmonary arteries; arterial or venous thrombosis; pulmonary parenchymal changes; pleurisy, and intracardiac thrombosis. Hemoptysis caused by pulmonary artery aneurysms may lead to lethal hemorrhage. Recent advances in therapeutic strategies have improved the prognosis. In this review, the salient clinical and histopathological features of BD and treatment strategies are discussed.     

Behçet's disease

Beh?et's disease is a systemic vasculitis characterized by recurrent oral and genital ulcers, and ocular inflammation, and which may involve the joints, skin, central nervous system and gastrointestinal tract. It is most common in those of Mediterranean and Eastern origin, although it also affects Caucasians. The aetiology of the disease remains unknown, but the most widely held hypothesis of disease pathogenesis is that of a profound inflammatory response triggered by an infectious agent in a genetically susceptible host. Supporting this is the consistent association of disease susceptibility with polymorphisms in the human leukocyte antigen complex, particularly HLA-B*51. The diagnosis is a clinical one, and although there is no single laboratory test specific for the diagnosis of Beh?et's disease, the 1990 classification criteria perform well in a clinical context. Whereas many favoured treatments for single or multisystem disease still lack a sound evidential base, cyclosporin and azathioprine perform well in clinical trials, and evidence is accumulating for the efficacy of anti-tumour necrosis factor therapy in particular clinical situations. This review will focus on recent developments in the understanding of disease pathogenesis and clinical diagnosis, and review the evidence base for both established and new agents in the therapeutic strategy.