Behcets Disease

Behcet s disease (BD) is a multisystem disease characterized by mucocutaneous, ocular, articular, vascular, intestinal, urogenital, and neurologic involvement. BD was first described as a triad of recurrent aphthous stomatitis, genital aphthae, and relapsing uveitis in 1937 by Hulusi Behget, a Turkish dermatologist (1), but descriptions of the disease features date back to Hippocrates, from his third book of epidemiology written in the fifth century BC. 

BD has a worldwide distribution, although most cases are reported from J an, the Middle East, and the Mediterranean. This pattern of origin, corresponding to the 

The underlying pathogenesis of most of the pulmonary lesions is believed to be an immune complex vascuhtis affecting all types of blood vessels (12). 

Minor aphthous, major aphthous, or herpetifom ulceration which are recurrent at least three times in one 12-month period Plus two of the following  

Cells in vitreous on slit lamp examination Or retinal vasculitis observed by qualified physician Skin lesions Erythema nodosum like Pseudo foUicuhtis Papulopustular lesions Or acneiform nodules Positive pathergy test 

This is a chronic inflammatory disease, which can affect the gut as well as other organs. There is relatively little information on the role of free radicals in this condition. Neutrophil chemiluminescence was increased in patients with intestinal Behcet s compared to normal controls (Suematsu et al., 1987a) and there is some evidence for endothelial injury by neutrophil-derived oxidants (Niwa et al., 1982). Preliminary studies with lip)osomal-encapsulated SOD demonstrated marked improvement in 12 out of 16 pjatients wdth active Behcet s disease (Niwa etal., 1985). 

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Primary angiitis of the CNS causes inflammation particularly in small leptomeningeal vessels, whereas larger vessels can also be affected. Systemic variants of vasculitis and secondary arteritis of the CNS affect mostly small or medium-sized arteries to different degrees, and occasionally the venous system is also afflicted as in Behcet disease. Segmental stenoses are frequently found, often not including bifurcations and alternating with arterial dilatations. This pattern is not definitively specific and can also be seen in atherosclerosis. Even with an optimal MRA technique, DSA still remains necessary for the depiction of tiny vessel lesions (Fig. 5.26). 

Risk of stroke in a cohort of 815 patients with calcification of the aortic valve with or without stenosis. Stroke 27 847-851 Borhani Haghighi A, Pourmand R, Nikseresht AR (2005). Neuro-Behcet disease. A review. Neurologist 11 80-89... 

Behcet disease Complete absence of the TAP1C allele.3 Linkage disequilibrium between HLA-DQB1 0501 and TAP2B (Gonzalez-Escribano et al., 1995). 

Ertenli I, Kiraz S, Calguneri M, et al. Synovial fluid cytokine levels in Behcet s disease. Clin Exp Rheumatol 2001 19(5 Suppl 24) S37 11. 

The autoimmune rheumatic diseases consists of Rheumatoid Arthritis (RA), Spondylarthritis (SpA), Systemic Lupus Erythematosus (SLE), Polymyositis, Dermatomyositis, Polymyalgia Rheumatica, Acute Temporal Arteritis, Giant Cell Arteritis, Behcet s Disease, Sjorgren s Syndrome, Felty s Syndrome and Mixed Connective Tissue Disease (MCTD). Spondylarthritis (SpA) can be subdivided in Reactive Arthritis (ReA), Ankylosing Spondylitis (AS), Psoriatic Arthritis (PsA), Arthritis associated with the inflammatory bowel diseases are Crohn s disease and Ulcerative Colitis (IBD), Undifferentiated SpA (UspA) and Sacro-ilitis, Juvenile SpA and Acute Anterior Uveitis (AAU). 

Dermatological system (erythroderma, icthyosis, Stevens-Johnson syndrome, Behcet s disease, acute blistering diseases)... 

Siikakis PP. 2002. Behcet s disease A new target for anti-tumour necrosis factor treatment. Ann Rheum Dis. 61 ii51-u56. 

Primary systematic vasculitis Rheumatoid disease Sjogren s syndrome Behcet s disease... 

Neurological involvement in Behcet s disease may be subclassified into two major forms a vascular-inflammatory process with focal or multifocal parenchymal involvement and a cerebral venous sinus thrombosis with intracranial hypertension. The vasculitis and meningitis may affect cerebral arteries, particularly in the posterior circulation, to cause ischemic stroke and possibly intracranial hemorrhage (Farah et al. 1998 Krespi et al 2001 Siva et al. 2004 Borhani Haghighi et al. 2005). 

Subarachnoid hemorrhage due to septic embolic infarction in infective endocarditis. Cerebrovascular Diseases 9 182-184 Krespi Y, Akman-Demir G, Poyraz M et al (2001). Cerebral vasculitis and ischemic stroke in Behcet s disease report of one case and review of the literature. European Journal of Neurology 8 719-722... 

Behcet s disease (+) HLA-B5 or (-) BW51 — Internist, rheumatologist Behget s skin puncture test... 

Niino M, Ogata A, Kikuchi S, Tashiro K, Nisliihira J (2000) Macrophage migi adon inliibitory factor in die cerebrospinal fluid of padents widi convendonal and opdc-spinal forms of muldple sclerosis and neuro-Behcet s disease. J Neurol Sci 179 127—131. 

Diseases in which immunosuppression may be useful include tissue transplantation, inflammatory bowel disease, rheumatoid arthritis, chronic active hepatitis, systemic lupus erythematosus, glomerulonephritis, nephrotic syndrome, some haemolytic anaemias and thrombocytopenias, uveitis, myasthenia gravis, polyarteritis, polymyositis, systemic sclerosis, Behcet s syndrome. 

Bayraktar, Y., Balkanci, F., Bayraktar, M., Calguneri, M. Budd-Chiari syndrome a common comphcation of Behcet s disease. Amer. J. Gastroenterol. 1997 92 858 -862... 

Yamamoto JH, Minami M, Inaba G, Masuda K, Mochizuki M (1993) Cellular autoimmunity to retinal specific antigens in patients with Behcet s disease. Br J Ophthalmol 77 584—589. 

Gonzalez-Escribano NF, Morales J, Garcia-Lozano JR, Castillo MJ, Sanchez-Roman J, Nunez-Roldan A, Sanchez B (1995) TAP polymorphism in patients with Behcet s disease. Ann Rheum Dis, 54 386-388. 

Azathioprine is indicated in renal homotransplantation (five-year patient survival rate of 35%) in rheumatoid arthritis (for patients with severe, active, and erosive disease not responding to conventional therapies) and in chronic ulcerative colitis, myasthenia gravis, and Behcet s syndrome (adverse effects may offset its limited value). As with other cytotoxic drugs, azathioprine can affect rapidly growing cells, resulting in leukopenia, thrombocytopenia, and gastrointestinal toxicity. In addition, hepatotoxicity (cholestasis) has been reported. Many of the general problems of immunosuppression, such as increased risk of infections, can also occur. 

Chlorambucil (0.1 to 0.2 mg/kg/d for 3 to 6 weeks) will provide palhation in chronic lymphocytic leukemia, mahg-nant lymphomas including lymphosarcoma, giant follicular lymphoma, and Hodgkin s disease. In addition, it has been used in the treatment of uveitis and meningoencephalitis associated with Behcet s disease. Chlorambucil is absorbed orally, metabolized extensively, and the metabolite is... 

Nervous system Cerebral tumor-like lesions, uncommon in Behcet s disease, have been reported in a man taking ciclosporin who developed a lymphocytic meningitis and relapsed after rechallenge [8 ]. 

Table 1 Frequency of Clinical Manifestations in Behcet s Disease...

International Study Group for Behcet s Disease. Criteria for diagnosis of Behfet s disease. Lancet 1990 335 1078-1080. 

Ning-Sheng L, Chun-Liang L, Ray-Sheng L. Bronchiohtis obhterans organizing pneumonia in a patient with Behcet s disease. Scand J Rheumatol 2004 33(6) 437-440. 

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