Beh?et's disease and streptococcal infection

The streptococcal antigen was determined in the plasma of 26 patients with Beh?et's disease, 30 patients with other uveitis (tuberculosis: 7, sarcoidosis: 6, Vogt-Koyanagi-Harada's disease: 6, toxoplasmosis: 6, trauma: 5), and 63 normal subjects with matched ages. The antigen titer in Beh?et's patients, either during ocular attack or remission, was significantly higher than that in the normal control subjects (P less than 0.0001) and that of other uveitis subjects (P less than 0.001). Time-course study in the Beh?et's disease cases showed that the titer was the highest during the attack and then decreased gradually as the ocular inflammation subsided. It was also found in two cases that the titer increased markedly just before the ocular attacks. There was no difference between the titer in other uveitis cases and that in the controls. By electron microscope, streptococcal L form-like structures were observed in the plasma and leukocyte layer of the blood of Beh?et's patients sampled during the ocular attacks. Streptococcal infection may play a significant role in the pathogenesis of Beh?et's disease.     

Quantitative assessment of aqueous flare and cells in uveitis

Validity of the laser flare-cell meter in uveitis was evaluated. A comparative study of slit-lamp examination and flare-cell meter measurements was carried out in the evaluation of iridocyclitis in a total of 251 measurements in patients with uveitis of various etiologies, including Beh?et's disease, Vogt-Koyanagi-Harada's disease and sarcoidosis. The data obtained by these two methods showed a significant overall correlation both in the measurement of flare intensity (Kendall tau = 0.580, P less than 0.001) and cell count (tau = 0.390, P less than 0.001). Problems inherent to the slit-lamp examination were marked in terms of wide variations in grading within groups and large overlaps in scores between groups. The flare-cell meter was highly reliable in the measurement of flare intensity; whereas it could not detect low numbers of cells in some cases. Ninety-one eyes of patients with Beh?et's disease were divided into three groups based on the interval from the last ocular attack episode. The flare-cell meter examination revealed that values for flare intensity remained abnormally high even in patients free from an attack episode for more than six months prior to the examination. It was considered that deterioration or breakdown of the blood-aqueous barrier function persists in patients with Beh?et's disease.     

Pattern of uveitis in a referral centre in Tunisia, North Africa

AIM: To analyse the pattern of uveitis in a referral centre in Tunisia, North Africa. METHODS: The study included 472 patients with uveitis examined at the Department of Ophthalmology of Monastir (Tunisia) from January 1992 to August 2003. All patients had a comprehensive ocular and systemic history, including an extensive review of medical systems. Complete ophthalmic examination was performed in all cases, including best-corrected Snellen visual acuity, slit-lamp examination, applanation tonometry, and dilated fundus examination with three-mirror lens. Standard diagnostic criteria were employed for all syndromes or entities of uveitis. RESULTS: The mean age at onset of uveitis was 34 years. The male-to-female ratio was 1:1.1. Uveitis was unilateral in 282 patients (59.7%) and bilateral in 190 patients (40.3%). Anterior uveitis was most common (166 patients; 35.2%), followed by posterior uveitis (133 patients; 28.2%), panuveitis (100 patients; 21.2%), and intermediate uveitis (73 patients; 15.5%). A specific diagnosis was found in 306 patients (64.8%). The most common cause of anterior uveitis was herpetic uveitis (56 patients; 33.7%). Toxoplasmosis was the most frequent cause of posterior uveitis (51 patients; 38.3%). Intermediate uveitis was most commonly idiopathic (63 patients; 86.3%). Beh?et's disease was the most common cause of panuveitis (36 patients; 36%), followed by Vogt-Koyanagi-Harada (VKH) disease (15 patients; 15%). A total of 16 patients (3.4%) suffered from blindness, and 59 (12.5%) from uniocular blindness. CONCLUSIONS: In a hospital population in Tunisia, the most common causes of uveitis were Beh?et's disease, herpes simplex infection, toxoplasmosis, and VKH disease.     

Efficacy of interferon alpha in the treatment of refractory and sight threatening uveitis: a retrospective monocentric study of 45 patients

AIM: Severe uveitis is potentially associated with visual impairment or blindness in young patients. Therapeutic strategies remain controversial. The efficacy of interferon alpha-2a (IFN-alpha2a) in severe uveitis, refractory to steroids and conventional immunosuppressive agents, was evaluated. PATIENTS AND METHODS: Patients were included after a major relapse of uveitis following corticosteroids and immunosuppressants. IFN-alpha2a (3 million units three times a week) was administered subcutaneously. Efficacy was assessed by improvement in visual acuity, decrease in vitreous haze, resolution of retinal vasculitis and macular oedema, assessed by fundus examination and fluorescein angiography, and decrease in oral prednisone threshold. RESULTS: 45 patients were included. Median age was 32.3 years (range 8-58) and sex ratio (F/M) was 0.66. Uveitis was associated with Beh?et's disease in 23 cases (51.1%) and with other entities in 22 cases (48.9%). Median duration of uveitis before interferon therapy was 34.9 months (range 3.4-168.7) and an average of 3.26 relapses following corticosteroids and immunosuppressants was noted. Uveitis was controlled in 82.6% of patients with Beh?et's disease and 59% of patients with other types of uveitis (p = 0.07). During a mean follow-up of 29.6 months (range 14-55), median oral prednisone threshold decreased significantly from 23.6 mg/day (range 16-45) to 10 mg/d (range 4-14) (p<0.001). Interferon was discontinued in 10 patients (22.2%) with Beh?et's disease and in four patients without Beh?et's disease. Relapses occurred in four and one cases, respectively. CONCLUSIONS: Interferon therapy appears to be an efficient strategy in severe and relapsing forms of Beh?et's disease but also in other uveitic entities. However, it seems to act more to suspend rather than cure the disease. Therefore, IFN-alpha2a may be proposed as a secondline strategy after failure of conventional immunosuppressants.     

Childhood Behçet's disease: clinical and evolutive aspects. About 13 cases

INTRODUCTION: Beh?et's disease is a systemic vasculitis which rarely occurs in childhood. The aim of this study was to evaluate clinical characteristics and outcome of Beh?et's disease in Moroccan children. MATERIAL AND METHODS: A retrospective study of 13 cases of children, 10 males and 3 females with Beh?et's disease followed up between 1990 and 1998. The diagnosis of Beh?et's disease was based on the criteria of the international study group for Beh?et's disease. All patients were studied by a complete clinical, ophthalmological and laboratory staging and treated with appropriate therapy. RESULTS: The mean age at diagnosis of Beh?et's disease was 13.9 years. Familial forms were found in 30.7% of cases. Oral aphtae were noted in all cases while genital ulcers were present in 76% of cases. Cutaneous lesions were found in only 1 case and 53.8% of children had a pathergy test. Articular involvement was found in 30.7%, neurological features in 46% and vascular manifestations in 38.4%. Only one case of intestinal involvement was noted. Ocular features (76%) were bilateral in all cases and were dominated by panuveitis complicated by macular edema ant retinal vasculitis. CONCLUSION: Beh?et's disease seems to have particular characteristics in childhood. Familial forms, articular and digestive manifestations appear to be more frequent in early stages of Beh?et's disease in children. Neurological and vascular involvement with panuveitis seems more frequent in the older children.     

Antibody titer to streptococcal and staphylococcal L-form in Beh?et's disease and other uveitis]

The antibody titer in serum to Streptococcus pyogenes L-form and Staphylococcus aureus L-form were determined by enzyme-linked immunosorbent assay in 28 patients with Beh?et's disease, 31 patients with other uveitis (sarcoidosis: 10, Harada's disease: 5, tuberculosis: 4, rheumatoid arthritis: 4, lues: 2, juvenile rheumatoid arthritis: 2, herpes simplex: 2, trauma: 2) and 16 healthy normal controls. All L-forms were induced by the penicillin disk method. The antibody titer to Streptococcus pyogenes L-form in Beh?et's disease was lower than that of other cases of uveitis and controls, and showed significant differences between controls, rheumatoid arthritis, sarcoidosis, tuberculosis and Harada's disease by the Student's t-test. The antibody titer to Staphylococcus aureus L-form in Beh?et's disease showed no difference between controls and other cases of uveitis. In each uveitis and controls, and between active and inactive stages of all uveitis, there were no differences between titers. In Beh?et's disease, antibody formation to Streptococcus pyogenes L-form may be specifically disturbed.     

Phacoemulsification cataract extraction and intraocular lens implantation in patients with uveitis

PURPOSE: To analyze the outcomes of phacoemulsification cataract extraction and intraocular lens (IOL) implantation in patients with uveitis. SETTING: Miyata Eye Hospital, Miyakonojo, Miyazaki, Japan. METHODS: The records of 95 patients (131 eyes) with uveitis who had phacoemulsification cataract extraction and IOL implantation between 1990 and 2001 were retrospectively examined. The postoperative visual outcomes and complications were analyzed. RESULTS: The mean age of the 36 men and 59 women was 61.7 years (range 30 to 87 years) At the final follow-up examination, 111 eyes (84.7%) had improved visual acuity and 97 eyes (74.0%) had a final visual acuity of 0.5 or better. Patients with Beh?et's disease had significantly worse visual outcomes than patients with other clinical etiologies of uveitis such as human T-lymphotropic virus type 1 uveitis and Vogt-Koyanagi-Harada disease. In 17 eyes (13.0%), relapse of intraocular inflammation occurred within 6 months after surgery; the rate of relapse was highest in patients with Beh?et's disease (35.2%). Posterior synechias occurred in 8 eyes (6.1%), pupillary capture in 1 eye (0.8%), intraocular pressure elevation in 11 eyes (8.4%), and cystoid macula edema in 8 eyes (6.1%). In 31 eyes (23.7%), posterior capsule opacification required neodymium:YAG capsulotomy. CONCLUSIONS: The outcomes of phacoemulsification cataract extraction and IOL implantation in patients with uveitis were satisfactory. Patients with Beh?et's disease related to intraocular inflammation, however, appeared to have a higher risk for complications and therefore worse outcomes than patients with other clinical etiologies of uveitis.     

Conjunctival ulcers in Behçet's disease

PURPOSE: To describe the occurrence of conjunctival ulcers as a manifestation of Beh?et's disease. DESIGN: Retrospective, noncomparative, interventional case series with histopathologic correlation. METHODS: Six patients who fulfilled the diagnostic criteria for Beh?et's disease and presented with painful conjunctival ulcers were included in the study. Three of these ulcers were biopsied and studied histologically and immunohistochemically. The lesions were treated with topical or subconjunctival injection of corticosteroids and, in one case, with oral indomethacin. RESULTS: Although all six patients fulfilled the diagnostic criteria for Beh?et's disease, two developed uveitis and other signs of Beh?et's disease only months to years after the appearance of the conjunctival ulcers. The 3- to 5-mm, round to oval ulcers were located in the limbal and/or bulbar conjunctiva. Histopathology revealed disrupted epithelium, infiltration of both acute and chronic inflammatory cells, and high endothelial venules. Immunohistochemical analysis of the infiltrating lymphocytes revealed primarily T-cell populations admixed with several B cells and CD68-positive histiocytes. After treatment, the conjunctival lesions invariably healed without scarring. CONCLUSIONS: In addition to the oral and genital ulceration, ulcers can also be found in the conjunctiva of patients with Beh?et's disease. Although this is a rare clinical sign, when accompanied by uveitis or orogenital ulcers, it may suggest a diagnosis of Beh?et's disease.     

Ocular involvement in Beh?et's disease

Beh?et's disease is a chronic systemic disorder characterized by recurrent uveitis, aphthous lesions of the mouth, and ulcerations of the genitalia. Vision loss from ocular involvement is frequently the presenting symptom and optometrists should be aware of the various ocular signs of Beh?et's disease. Management of the Beh?et's patient involves systemic therapy and therefore requires comanagement with an internist and/or ophthalmologist. A case report of this infrequent type of uveitis is described along with a discussion of the care and visual prognosis of the Beh?et's patient.     

Incidence of endogenous uveitis at Kyushu University Hospital

PURPOSE: We performed a clinical statistical study on recent patients with endogenous uveitis at the clinic of the Department of Ophthalmology, Kyushu University Hospital. SUBJECTS: We studied 616 patients with endogenous uveitis who first visited the clinic of the Department of Ophthalmology, Kyushu University Hospital, between January 1996 and December 2001. RESULTS AND CONCLUSION: The most frequent clinical entity was sarcoidosis (8.6%), followed by Beh?et's disease (8.4%), Vogt-Koyanagi-Harada syndrome (6.5%), human T-lymphotropic virus type I (HTLV-1) uveitis (3.9%), and toxoplasmosis (2.6%). Unclassified uveitis comprised 58.1% in our study. Next we classified the subjects into four age groups; adolescent (0-19 years old), young (20-39 years old), middle-aged (40-59 years old), and elderly (60-years old). We also classified the disease into four groups: uveitis pan, anterior, intermediate, and posterior uveitis, according to the site of inflammation. The most frequent clinical entity was Beh?et's disease in the young group, and sarcoidosis in the elderly group. The frequency of unclassified uveitis was high in the adolescent and the elderly groups. As to the anatomic diagnosis of uveitis, panuveitis was most frequent, followed by anterior, posterior, and intermediate uveitis. Finally we investigated the frequency of secondary glaucoma. The frequency in all 616 patients was 19.7%. Secondary glaucoma was more frequent in male patients and more frequent in the middle-aged and the elderly groups.     

Uveitis associated with human T-cell lymphotropic virus type I.

Seroepidemiologic, clinical, and virologic studies were performed to determine whether human T-cell lymphotropic virus type I was closely associated with uveitis in two hospitals. One hospital was in an endemic area of the virus (Miyakonojo, Miyazaki) and the other hospital was in a less endemic area (Kurume). In the endemic area, the seroprevalence of the virus in patients with uveitis without defined causes (35.4%, 62 of 175 patients) was significantly higher than that in patients with nonuveitic ocular diseases (16.1%, 42 of 261 patients), or in patients with uveitis with defined causes (10.3%, eight of 78 patients). The seroprevalence in younger patients (20 to 49 years of age) with uveitis without defined causes in the area was 44.8% (30 of 67 patients), whereas it was only 9.3% (ten of 107 patients) in the other two groups. A similar observation was recorded even in the less endemic area (Kurume). Because the seroprevalence of the virus in the general population is known to be low in younger patients and to increase with age, these findings were interpreted to indicate that the association of human T-cell lymphotropic virus type I with uveitis was significant. Most patients, particularly those aged 20 through 49 years, had an intermediate uveitis characterized by a moderate inflammation in the vitreous body accompanied by an iritis and retinal vasculitis. The ocular symptoms in the patients differed from those of other types of uveitis common in Japan (Beh?et's disease, Vogt-Koyanagi-Harada's disease, and toxoplasmosis, for example).(ABSTRACT TRUNCATED AT 250 WORDS)     

Opacification of a hydrophilic acrylic intraocular lens with exacerbation of Behçet's uveitis

Beh?et's disease is 1 of the most common causes of uveitis in the Eastern world. Its common ocular complications are uveitis, cataract, and obliteration of retinal vessels. Phacoemulsification with intraocular lens (IOL) implantation in patients with Beh?et's disease is known to be a safe procedure. We managed a patient with Beh?et's disease who had aggravated uveitis and opacification of a hydrophilic acrylic IOL (ACRL-C160, Ophthalmed) 4 months after cataract surgery. Recalcitrant uveitis despite maximum tolerable medication and IOL opacification with vitreous opacity necessitated an IOL exchange and trans pars plana vitrectomy. After the procedure, the eye became quiescent. However, the visual acuity was 20/200 because of the obliteration of retinal vessels.     

Beh?et disease in children. 3 Tunisian cases and review of the literature]

OBJECTIVE: To describe epidemiological, clinical and evolutive features of ophthalmological involvment of Beh?et disease in children in tunisian patients. PATIENTS AND METHODS: In a retrospective study from 1990 to 1996, we collected three observations in children in 130 recorded cases of Beh?et disease. Patients have been explored by a complete ophthalmologic staging and are treated and followed in our institution for theirs oculars problems. RESULTS: The three children, all males, aged from 12 to 13 years, represented 2.3% of all the Beh?et disease diagnosed during the same period. Oral and/or genital aphtosis are present in all 3 cases and cutaneous or articular involvement is seen in 2 patients. Ocular Beh?et disease involvement, bilateral in all the cases, is dominated by uveitis (posterior in 3 patients or anterior in 2) complicated by macular edema in 3 cases and cataract or pseudo-trou in 1 case. CONCLUSION: Beh?et disease in children is characterized by the frequency of familial forms and articular involvement. The most characteristic ocular feature is represented by the posterior uveitis with a visual prognosis poor in short term. Specific criteria of Beh?et disease is necessary for children for a early and best identification.     

Serum immunosuppressive acidic protein (IAP) in inflammatory ocular disease

The titer of serum immunosuppressive acidic protein (IAP) was determined by the method of single radial immunodiffusion (Matsuda et al) in Beh?et's disease, sarcoidosis, tuberculosis, toxoplasmosis, etiology-unknown anterior uveitis (granulomatous, non-granulomatous), allergic conjunctivitis and scleritis. The titer of IAP in these diseases except allergic conjunctivitis was significantly higher than in normal controls. In all the diseases studied, the titer during the active stage was significantly higher than that during the inactive stage. The titer in Beh?et's disease was significantly higher than that in sarcoidosis, particularly during the active stage. In three cases of Behcet's disease, the titer was increased markedly a few days before the ocular attacks. There was no difference in the titer between etiology-unknown granulomatous and non-granulomatous anterior uveitis. IAP may be useful as a clinical index in inflammatory ocular diseases.     

Human recombinant interferon alfa-2a for the treatment of Behçet's disease with sight threatening posterior or panuveitis

BACKGROUND: Beh?et's disease is a multisystem vasculitis of unknown origin. Standard treatment mainly comprises systemic immunosuppressive agents. Ocular involvement, mostly posterior uveitis with retinal vasculitis, leads to blindness in 20-50% of the involved eyes within 5 years. The efficacy of interferon alfa-2a was studied in patients with sight threatening posterior uveitis or retinal vasculitis. METHODS: 50 patients were included in this open, non-randomised, uncontrolled prospective study. Recombinant human interferon alfa-2a (rhIFNalpha-2a) was applied at a dose of 6 million units subcutaneously daily. Dose reduction was performed according to a decision tree until discontinuation. Disease activity was evaluated every 2 weeks by the Beh?et's disease activity scoring system and the uveitis scoring system. RESULTS: Response rate of the ocular manifestations was 92% (three non-responder, one incomplete response). Mean visual acuity rose significantly from 0.56 to 0.84 at week 24 (p<0.0001). Posterior uveitis score of the affected eyes fell by 46% every week (p<0.001). Remission of retinal inflammation was achieved by week 24. Mean Beh?et's disease activity score fell from 5.8 to 3.3 at week 24 and further to 2.8 at week 52. After a mean observation period of 36.4 months (range 12-72), 20 patients (40%) are off treatment and disease free for 7-58 months (mean 29.5). In the other patients maintenance IFN dosage is three million units three times weekly. CONCLUSIONS: rhIFNalpha-2a is effective in ocular Beh?et's disease, leading to significant improvement of vision and complete remission of ocular vasculitis in the majority of the patients.     

Beh?et's disease in Taiwan--a review of 53 cases

Clinical manifestations and immunogenetic aspects of Beh?et's disease were investigated in the Veterans General Hospital of the National Yang-Ming Medical College, Taiwan. Diagnosis of Beh?et's disease was made according to the diagnostic criteria proposed by the Japanese Research Committee of Beh?et's disease. Fifty-three patients including 33 men and 20 women have been diagnosed as having Beh?et's disease in the past 10 years; 20 cases with the complete type and 33 cases with the incomplete type. About half of these cases were diagnosed in the past 2 years, predominantly by ophthalmologists. Of the 53 cases, ocular lesions were found in 35 cases, 25 men and 10 women. The ages of the onset of the ocular lesions ranged from 10 to 50 years, with the peak distribution between 20-30 years. Bilateral involvement was seen in 24 men and 8 women, and a total of 67 eyes were affected; 37 eyes (55.2%) had the final visual acuity of 0.1 or lower. Immunogenetic study revealed that the incidence of HLA-B51 was significantly higher in the patients than in the normal population.     

Differential diagnosis in infectious posterior uveitis

Infectious aetiologies represent the largest entity in patients affected by posterior uveitis. As a single diagnosis, ocular toxoplasmosis ist the most frequently diagnosed infectious entity, wheres Beh?et's uveitis represents the most frequently encountered immunologic aetiology. In acute posterior uveitis, an exact clinical diagnosis is sometimes prohibited by a dense vitreal infiltration. In this situation, the clinical course and progression of the disease may help in the differential diagnosis. Rapidly progressive instances such as viral retinal necrosis may be differentiated from cases of ocular toxoplasmosis and Beh?et's disease, which tend to present in the office within one to two weeks of symptoms, and from other more slowly progressive diseases such as mycotic or nematode infections. The clinical picture at presentation is influenced by the duration of disease, underlying systemic conditions, and by the natural course of disease including the healing process and scare formation. Generally, visual function has to be assumed at risk, if a lesion is located close to the macula, if frequent recurrences increase the likelihood of macular involvement and, furthermore, if secondary complications affect the macula, e. g., macular oedema. In paediatric cases, the risk of amblyopia is frequently under-estimated, namely in the context of persistent dense vitreal infiltration. Therefore, a rapid interdisciplinary clinically tailored diagnostic access has to be reinforced in order to establish an early, targeted and etiologically based therapeutic strategy including steroid-sparing immunomodulating agents and possibly surgical alternatives.     

Fluorescein iris angiography and anterior segment fluorophotometry in patients with Beh?et's disease

Fluorescein iris angiography and anterior segment fluorophotometry were performed in 63 eyes of 34 patients with Beh?et's disease during its remission stage. Thirty normal eyes were studied as the control. A variety of leakage patterns comprising posterior chamber leakage and paravascular leakage were detected in 32 eyes with Beh?et's disease by means of fluorescein iris angiography, while no leakage was detected in the control eyes. These leakage patterns were frequently seen in the posterior type uveitis group of Beh?et's patients, but were scarce in the anterior type uveitis group. By anterior segment fluorophotometry, the levels of fluorescein concentration in the anterior chamber were significantly higher in the posterior type uveitis eyes of Beh?et's patients than those in either the anterior type uveitis eyes or the controls (P less than 0.05-P less than 0.01). The results indicated that the damage to the vascular system still existed in the remission stage of the disease, and that this abnormality occurred more markedly in the posterior type group than in the anterior type group of Beh?et's disease eyes.     

Clinical and immunological studies of Beh?et's disease

8 patients with clinically diagnosed Beh?et disease were immunologically investigated. Uveitis was present in 7 of these patients. Although uveitis is a rare presentation of Beh?et's disease, it tends to be bilateral when it does occur and to involve both the anterior and posterior segments repeatedly. Nowadays it is rare to observe a hypopyon, thanks to early initiation of therapy. In the late stages, recurrent vitreous haemorrhage complicates the course of the disease and can lead to blindness. HLA-B5 can frequently be demonstrated in patients with Beh?et's disease. Detailed serological investigations, as measles hemagglutination inhibition test+complement fixation test (CFT), rubella hemagglutination inhibition test, hepatitis HB2 antigen, adenovirus antigen, varicella zoster CFT, mumps CFT, herpes simplex CFT, anticytomegalovirus IgM and IgG, toxoplasmosis immunofluorescence, tuberculosis Middlebrock-Dubois, were unable to uncover a connection with past or present infections. Determinations of haptoglobulin, lactate dehydrogenase, albumin and ceruloplasmin showed nothing worth remarking. Electrophoresis can indicate the duration of the illness. In 1 patient the lymphocyte adherence inhibition test was abnormal.     

Beh?et's syndrome: ocular features in an Australian population

Inflammatory eye disease (IED) is often the most severe manifestation of Beh?et's syndrome (BS). This disease is a common cause of uveitis in Mediterranean countries, the Middle East and Japan. In order to ascertain the prevalence of this disease in Australia, we reviewed the aetiology of patients attending our uveitis clinic over a five-year period. Twelve of 426 patients with inflammatory eye disease had definite Beh?et's syndrome. Four patients had anterior uveitis, four had posterior uveitis, four had retinal vasculitis and one also had optic neuritis. Although inflammatory eye disease was the initial clinical feature of Beh?et's syndrome in only three of our patients, it was the feature that led to a definite diagnosis in all but one patient. The inflammatory eye disease of Beh?et's syndrome was characteristically recurrent and severe with significantly decreased vision in 10 eyes, cataracts in six eyes, macula oedema in four eyes and glaucoma in two eyes. We conclude that Beh?et's syndrome is a rare cause of inflammatory eye disease in Australia and is unlikely to be recognised as a distinct clinical entity prior to the onset of ocular involvement. The visual prognosis of ocular inflammation in Beh?et's syndrome remains guarded despite the use of a variety of immunosuppressive agents.