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Clinical manifestations and diagnosis of Behçet syndrome

Clinical manifestations and diagnosis of Behçet syndrome
Authors:
Ellison L Smith, MD
Yusuf Yazici, MD
Section Editor:
Peter A Merkel, MD, MPH
Deputy Editor:
Philip Seo, MD, MHS
Literature review current through: Jan 2024.
This topic last updated: Aug 14, 2023.

INTRODUCTION — Behçet syndrome, also known as Behçet disease, is characterized by recurrent oral aphthae and any of several systemic manifestations including genital aphthae, ocular disease, skin lesions, gastrointestinal involvement, neurologic disease, vascular disease, or arthritis (picture 1A-G). Behçet syndrome may have been described by Hippocrates, but it was brought to the attention of the modern medical community by Hulusi Behçet in 1937 [1-3].

Most clinical manifestations of Behçet syndrome are believed to be due to vasculitis. Among the systemic vasculitides, Behçet syndrome is remarkable for its ability to involve blood vessels of all sizes (small, medium, and large) on both the arterial and venous sides of the circulation.

The clinical manifestations, diagnosis, and differential diagnosis of Behçet syndrome are reviewed here. The pathogenesis and treatment of this disorder are discussed separately. (See "Pathogenesis of Behçet syndrome" and "Treatment of Behçet syndrome".)

EPIDEMIOLOGY — Behçet syndrome is more common along the ancient silk road, which extends from eastern Asia to the Mediterranean [4-6]. It is most common in Turkey (80 to 370 cases per 100,000), while the prevalence ranges from 13.5 to 35 per 100,000 in Japan, Korea, China, Iran, Iraq, and Saudi Arabia [4]. Beyond the silk road, immigrant and refugee populations from areas of high Behçet syndrome prevalence demonstrate increased risk of disease development [7]. Prevalence in Paris, France in 2003 was 7.1 per 100,000, with rates of 2.4 per 100,000 in those of European ancestry, 34.6 per 100,000 in those of North African ancestry, and 17.5 per 100,000 in those of Asian ancestry [8]. Behçet syndrome is somewhat more common in males in the eastern Mediterranean area and in females in northern European countries [1]. Estimates of prevalence in the United States and Europe have ranged from 0.12 to 7.5 per 100,000. Analysis of residents of Olmsted County, Minnesota over a 45-year period identified a prevalence of 5.2 per 100,000 [9].

Behçet syndrome typically affects young adults 20 to 40 years of age but is infrequently also seen in children [10-12]. The disease appears to be more severe in young, male patients from Middle- or Far-Eastern Asia [6,13-18]. Most cases of Behçet syndrome are sporadic, although families with multiple affected members, which is known as familial clustering, have been reported, and having a first-degree relative with Behçet syndrome does increase risk for the disease [19,20]. Earlier onset of disease in successive generations, known as genetic anticipation, has been described [21].

Several studies have shown a negative impact of Behçet syndrome on quality of life and impairment in activities of daily living [22-26].

CLINICAL MANIFESTATIONS — The common clinical feature in patients with Behçet syndrome is the presence of recurrent and usually painful mucocutaneous ulcers. Other clinical manifestations of this disorder are more variable among different patients and populations.

The severity is generally greater in men. The greatest morbidity and mortality occur with ocular disease (affecting up to two-thirds of patients), vascular disease (affecting up to one-third of patients), and central nervous system disease (affecting 10 to 20 percent of patients). Cutaneous and articular manifestations are common. Renal disease and peripheral nervous system involvement are rare compared with other vasculitides [27].

A number of studies have looked for clustering of phenotypic types among patients with Behçet syndrome. These clusters include patients with predominant mucocutaneous, articular, gastrointestinal, ocular, cardiovascular, or neurologic manifestations [28-30]. Whether these clusters indicate variation in pathophysiologic mechanisms has not been determined [31].

Oral ulcerations — Most, but not all, patients initially manifest recurrent oral aphthous ulcerations (also known as canker sores), which are grossly and histologically similar to common oral ulcers and recurrent aphthous stomatitis (RAS), but which tend to be more extensive and often multiple (picture 1A-D). The ulcers are painful and, in severe cases, may limit eating. They are rounded and range in size from a few millimeters to 2 cm. They have well-defined borders, have a white-yellow necrotic base, and may have surrounding erythema. Minor ulcers are defined as those less than 1 cm in diameter, and major ulcers are defined as those at least 1 cm in diameter. Major ulcers may scar. Outer portions of the lips are not involved.

Healing of oral ulcers is typically spontaneous within one to three weeks; with recurrent lesions, however, some patients will have ulcers present continuously. Oral ulcers are typically the first to come and last to leave in the course of the disease; they may become less common after about 20 years [32]. They may be a less frequent manifestation in cigarette smokers [33]. (See "Oral lesions", section on 'Behçet syndrome'.)

Urogenital lesions — Genital ulceration, the most specific lesion for Behçet syndrome, occurs in 75 percent or more of patients with Behçet syndrome. The ulcers are similar in appearance to the oral aphthae and are usually painful [34]. Genital ulcers are most commonly found on the scrotum in men and the vulva in women (picture 1E-H). Recurrence is typically less frequent than with oral ulcerations. Scar formation is frequent for genital lesions. Scrotal scarring secondary to ulcers is rarely, if ever, seen in conditions other than Behçet syndrome.

Epididymitis, salpingitis, varicocele, and other genitourinary inflammatory conditions may also occur in patients with this disorder [35,36]. Urethritis is an unusual feature.

Cutaneous lesions — Cutaneous lesions also occur in over 75 percent of patients with Behçet syndrome. The skin manifestations vary and may include acneiform lesions, papulo-vesiculo-pustular eruptions, pseudofolliculitis, nodules, erythema nodosum (septal panniculitis), superficial thrombophlebitis, pyoderma gangrenosum-type lesions, erythema multiforme-like lesions, and palpable purpura. Biopsy of erythema nodosum lesions reveals a septal panniculitis, with medium vessel vasculitis in up to half of lesions [37].

Acneiform lesions may be more common in those with associated arthritis [38,39]. These lesions consist of papules and pustules that are indistinguishable from ordinary acne and share characteristic microbiologic flora with papulopustular lesions of acne [40]. Pustular skin lesions are often not sterile and may contain Staphylococcus aureus and Prevotella spp [40].

Pathergy refers to an erythematous papular or pustular response to local skin injury. It is defined as a pustule-like lesion or papule that appears 48 hours after skin prick by a 20-gauge needle. Pathergy is less common in North American and North European patients with Behçet syndrome (10 to 20 percent) than in patients from more endemic areas (50 to 75 percent) [41]. Although the likelihood of a positive pathergy test has decreased over time, the specificity of the test remains relatively high [42]. Dermographism is a response to light scratching of the skin that may be present in some patients [43]. A vascular pathergy-like response may be evident after vascular procedures, resulting in phlebitis or aneurysms [44,45]. (See 'Vascular disease' below.)

Nailfold capillary abnormalities, mainly enlarged capillaries, may also be observed in patients with Behçet syndrome [46-48].

Ocular disease — Ocular disease occurs in 25 to 75 percent of patients with Behçet syndrome, depending upon the population studied, and in most cases progresses to blindness if not treated. Ocular disease typically is less severe in North American populations, resulting in a lower incidence of vision loss [49]. Male patients are more likely to get eye disease, with about 75 to 80 percent developing involvement, and also have worse visual outcomes, even with treatment.

●Uveitis is often the dominant feature of Behçet syndrome. It is typically bilateral and episodic, nongranulomatous, often involves the entire uveal tract (pan uveitis), and may not resolve completely between episodes. Isolated anterior uveitis is rare. (See "Uveitis: Etiology, clinical manifestations, and diagnosis".)

●Hypopyon is a severe anterior uveitis with purulent material in the anterior chamber that is characteristically seen in about 20 percent of patients with Behçet syndrome. Many patients with hypopyon will demonstrate retinal vasculitis [50].

●Posterior uveitis, retinal vasculitis (picture 2A-B), vascular occlusion, and optic neuritis require systemic immunosuppressive treatment and may irreversibly impair vision and progress to blindness if untreated. Intermediate and posterior uveitis cannot be assessed reliably without the use of a slit lamp. Characteristic findings include retinal infiltrates, retinal nerve fiber layer defects, occlusive retinal vasculitis, and diffuse retinal capillary leakage on fluorescein angiography, in patients without granulomatous anterior uveitis or choroiditis. Newer testing modalities including optical coherence tomography and optical coherence tomography angiography may reveal disease activity not otherwise identified and may be beneficial in identifying and monitoring early disease activity [51-59]. Parenchymal central nervous system disease is more common in patients with optic neuritis [60]. (See "Retinal vasculitis associated with systemic disorders and infections".)

Other changes that can be seen include neovascularization, secondary cataracts, glaucoma, frosted branch angiitis, macular edema and, in approximately 3 percent of patients, conjunctival ulceration [61,62]. Neovascularization most commonly occurs due to inflammation, and treatment with immunosuppression, particularly with interferon, may be beneficial [63]. Conjunctivitis, scleritis, episcleritis, and sicca syndrome are uncommon. Ocular signs may be helpful in making the diagnosis of Behçet syndrome, even in the absence of other clinical symptoms [64].

In a retrospective review of 880 Turkish patients with Behçet uveitis, 68 percent were male, and the mean age of onset was 28.5 years for men and 30 for women. Ocular disease was bilateral in 78.1 percent, and panuveitis was the most common finding. Risk of losing useful vision at 10 years was 30 percent for men and 17 percent for women, but prognosis was better in the 1990s than in the 1980s [65].

A retrospective review of childhood-onset uveitis in 36 patients with Behçet syndrome reported diagnostic and treatment findings similar to those seen in adults [11].

The Ocular Behçet Disease Research Group of Japan has proposed a scoring system called the Behçet’s disease ocular attack score (BOS24) that consists of measures of anterior chamber cells, vitreous opacities, and fundus lesions for each uveitis attack in each eye, to be used as a reproducible objective measure of disease activity [66].

Neurologic disease — Neurologic disease occurs in less than 10 percent of patients with Behçet syndrome in most series [67-70]. It is observed more frequently in males than females. In one large series, the clinical features and outcomes of 200 patients with Behçet syndrome and neurologic involvement reported that, on average, a period of approximately five to six years elapsed between the onset of the earliest non-neurologic symptoms of Behçet syndrome and the appearance of neurologic symptoms or findings [67]. Nevertheless, neurologic findings may also appear concurrently (7.5 percent) or precede non-neurologic features (3 percent). Twenty percent of those with neurologic findings were asymptomatic. (See "Treatment of Behçet syndrome", section on 'Prognosis'.)

Neurologic disease is typically classified as parenchymal or non-parenchymal:

●Parenchymal disease is subdivided into brainstem disease, multifocal (diffuse) disease (including brainstem, cerebral, or spinal cord disease), myelopathy, cerebral disease (including encephalopathy, hemiparesis, hemisensory loss, seizures, dysphagia, and mental changes such as psychosis and cognitive dysfunction), and optic neuropathy.

Parenchymal disease may also be divided into acute and chronic progressive neuro-Behçet syndrome. A meta-analysis reviewed 184 acute and 114 chronic progressive cases, and fever and higher cerebrospinal fluid cell counts were found more commonly in acute disease, and sphincter disturbances, ataxia, confusion, brainstem atrophy on magnetic resonance imaging (MRI), and cerebral changes on MRI were more common in chronic progressive disease [71,72].

Focal parenchymal lesions and complications of vascular thrombosis are the most common abnormalities. Progressive personality change, psychiatric disorders, and dementia may develop. Unlike many other systemic vasculitic disorders, peripheral neuropathy is not a common feature of Behçet syndrome, though it may develop in a subset of patients [73-75].

Parenchymal disease may be due to lesions in the corticospinal tract, brainstem, periventricular white matter, spinal cord, and basal ganglia. Brainstem disease (which may extend to the midbrain, basal ganglia, and diencephalon) including focal lesions or atrophy with signs and symptoms including ophthalmoparesis, cranial neuropathy, and cerebellar or pyramidal dysfunction are more characteristic of Behçet syndrome than multiple sclerosis. Cerebral lesions are often multiple though may be single, are often subcortical, and are not particularly peri-ventricular, as in multiple sclerosis. Spinal cord lesions (myelitis) may occur in isolation, but are more common in patients with other central nervous system lesions. The clinical presentation of parenchymal disease is often subacute and manifestations may include headache, behavior changes, and deficits reflecting areas of parenchymal involvement.

These central nervous system lesions are detectable with MRI [67,76]. Acute and subacute lesions are hypointense or isointense on T1-weighted images; hyperintense on T2-weighted, FLAIR, and diffusion-weighted images; and commonly enhance with contrast. In the chronic phase, lesions may be smaller or resolve, atrophy may be present, nonspecific white matter lesions may be present, and lesions usually do not enhance. Cerebrospinal fluid (CSF) may show increased protein and increased cells, and neutrophils may predominate [77]. Pathology reveals local perivenular lymphocytic cuffing, inflammatory cell infiltration, gliosis, necrosis, and neuronal loss. Although frank vasculitis is not always observed in parenchymal lesions, it is sometimes noted in larger cerebral vessels, including arteries or veins. Arteritis may lead to ischemic strokes, dissection, aneurysmal dilatation, and subarachnoid hemorrhage.

●Non-parenchymal disease includes cerebral venous thrombosis, intracranial hypertension syndrome (pseudotumor cerebri), acute meningeal syndrome, and uncommonly stroke due to arterial thrombosis, dissection, or aneurysm [77].

Central nervous system manifestations may result from arterial or venous thrombosis, including dural sinus thrombosis [78]. Cerebral venous thrombosis may present with headache, papilledema, sixth nerve palsy, and an elevated CSF pressure [14,32,67,78-81]. An association has been observed between dural sinus thrombosis and peripheral deep venous thrombosis [67]. Thrombosis of the cerebral arteries may also be observed [67,78]. One analysis of neurologic Behçet syndrome from Turkey, involving 26 children and 702 adults, found that dural venous sinus thrombosis was much more common in children than parenchymal neurologic involvement, although parenchymal disease was more frequent in adults [82].

Vascular disease — Most clinical manifestations of Behçet syndrome are believed to be due to vasculitis, and Behçet is remarkable for its ability to involve blood vessels of all sizes (small, medium, and large) on both the arterial and venous sides of the circulation (table 1).

Vascular involvement is one of the major causes of morbidity and mortality in Behçet syndrome. In particular, pulmonary artery aneurysm carries a high mortality of approximately 25 percent, and early recognition is important [83].

Prevalence — Vascular disease in Behçet syndrome is more common in men. A study of 2319 Turkish patients with Behçet found a prevalence of vascular disease of 14.3 percent and was most common in males. In this study, 53.3 percent suffered superficial venous thrombosis, 29.8 percent deep venous thrombosis, and 3.6 percent arterial disease [84].

Another study of 796 Chinese patients with Behçet syndrome found vascular involvement in 12.8 percent of patients. The male-to-female ratio was approximately 4:1 and the mean age of onset was 29.5 years. Vascular disease was the presenting manifestation of Behçet syndrome in 27.5 percent of cases; 54.9 percent had arterial disease, 70.6 percent had venous disease, and 25.5 percent had both. Women were more likely to have arterial disease. Patients with vascular manifestations of Behçet syndrome were more likely to have cardiac involvement [85].

A comparative study of two large cohorts of patients with Behçet syndrome and vascular involvement included 141 patients followed in France and 160 patients followed in Turkey [86]. After a median of 77 months, 562 vascular events occurred, including 440 venous events, 115 arterial events, and 7 cardiac thrombi. Vascular disease was the presenting manifestation in 28 percent. After a median follow-up of approximately 2 years, the first relapse occurred in 44.7 percent of patients. Immunosuppression after the initial vascular event appeared to reduce the likelihood of a relapse.

Arterial disease — Arterial disease is most commonly a small vessel vasculitis, but medium and large vessel disease may also develop.

Large vessel vascular involvement occurs in approximately one-third of patients with arterial disease [87]. In these patients, perivascular and endovascular inflammation may lead to hemorrhage, stenosis, aneurysm formation, thrombus formation in both arteries and veins. Progression and recurrence are more likely in these patients, and immunosuppressive treatment of this inflammation has been found to be beneficial, though patients may also require vascular surgery intervention [88]. (See "Treatment of Behçet syndrome", section on 'Large artery disease'.)

Carotid, pulmonary, aortic, iliac, femoral, and popliteal arteries are most commonly involved; cerebral and renal arteries are uncommonly involved [50]. Acute myocardial infarction can occur due to coronary artery vasculitis but is uncommon. Atherosclerosis does not appear to occur at an accelerated rate in Behçet syndrome, as has been observed in autoimmune diseases such as systemic lupus erythematosus [15].

Pulmonary artery aneurysms involving the large proximal branches of the pulmonary arteries are the most common pulmonary vascular lesion in Behçet syndrome and are uncommonly seen in diseases other than Behçet syndrome [83]. Hemoptysis is the most common presenting symptom; cough, dyspnea, fever, and pleuritic pain are other presenting symptoms [89,90]. A misdiagnosis of pulmonary embolism and subsequent anticoagulation can lead to a poor outcome if the underlying inflammatory large vessel vasculitis is not appreciated [89]. Although pulmonary artery aneurysms may be suspected on chest radiograph and echocardiography, a computed tomography (CT) angiogram, MR angiogram, or plain angiogram are the usual imaging modalities in which pulmonary artery aneurysms are identified. Pulmonary infarction does not commonly occur. Hemoptysis may be the result of pulmonary artery-bronchus fistulae and frequently coexists with venous obstruction elsewhere [91].

Pulmonary artery thrombosis and aneurysms in association with peripheral thrombophlebitis are known as Hughes-Stovin syndrome, and this syndrome most commonly occurs in Behçet syndrome and may represent a part of the spectrum of manifestations seen in Behçet syndrome [92-94]. Pulmonary artery thrombosis may occur with pulmonary artery aneurysms or may occur independently; a study of 47 patients with pulmonary artery disease and Behçet syndrome identified 26 patients with pulmonary artery aneurysms, 13 with pulmonary artery thrombosis, and 8 with both. Presenting manifestations were similar. Most patients had active Behçet syndrome at presentation, 85 percent had pulmonary nodules, 13 percent had cavitation, and 77 percent had peripheral venous thrombosis [90].

Venous disease — Venous disease resulting in venous thrombosis is more common than arterial involvement, and is often an early feature of Behçet syndrome. Superior and inferior vena cava occlusion, Budd-Chiari syndrome, dural sinus thrombosis, and other venous obstructive lesions can occur in addition to the more common superficial and deep vein thrombosis [95]. Thrombosis of the lower extremities may lead to a post-thrombophlebitic syndrome, and the risk may be reduced by immunosuppressive treatment [50,96-98].

In one study of 493 cases of Behçet syndrome, 53 (10 percent) were found to have one or more large vessel thrombosis [95]. Fourteen of these 53 patients (26 percent) had hepatic vein thrombosis, 8 also had inferior vena cava thrombosis, and 4 had both inferior vena cava and portal vein thrombosis. A case-control study of 73 patients with Behçet syndrome found a 14-fold increased risk of venous thrombosis compared with controls [99]. Venous thrombosis, thrombophlebitis, folliculitis, and retinal vasculitis were more common in men than in women. Venous disease may also be more common in patients with a positive pathergy test or ocular involvement.

Patients with Behçet syndrome who develop Budd-Chiari syndrome appear to have a more complicated clinical course [95,100]. In a retrospective study comparing 61 patients with Budd-Chiari syndrome with 169 Behçet patients without, patients with Budd-Chiari syndrome were more likely to have lower extremity deep vein and inferior vena cava thrombosis, had earlier disease onset, and had higher mortality rates, particularly in those with inferior vena cava thrombosis [100]. However, patients with chronic and silent Budd-Chiari syndrome had a relatively better prognosis.

Post-thrombotic syndrome is common in patients with Behçet syndrome after deep venous thrombosis. In a study of 205 patients with Behçet syndrome with deep venous thrombosis, 62 percent had post-thrombotic syndrome, and 18 percent severe post-thrombotic syndrome [97]. Immunosuppressive therapy and/or anticoagulation did not reduce the risk of post-thrombotic syndrome, but immunosuppression reduced the risk of severe post-thrombotic syndrome, and anticoagulation improved collateral flow scores [97].

Pulmonary disease — In addition to pulmonary vascular lesions, already discussed (see 'Vascular disease' above), radiographic abnormalities including loss of lung volume, well-defined opacities, and indistinct nodular or reticular shadows have been noted, but only rarely has histopathologic correlation been available. Among the various pathologic findings are pulmonary infarction, hemorrhage, and both organizing and eosinophilic pneumonias [101]. Miscellaneous other pulmonary findings found in patients with Behçet syndrome include pleural effusion, pulmonary arteritis or venulitis, bronchial stenosis, abscess, obstructive airway disease, chronic bronchitis, pulmonary hypertension and fibrosis [89,102].

Arthritis — A nonerosive, asymmetric, usually nondeforming arthritis occurs in about one-half of patients with Behçet syndrome, particularly during exacerbations. The arthritis most commonly affects the medium and large joints, including the knee, ankle, and wrist. Inflammation is evident on synovial fluid and biopsy specimens [103]. In many patients, the arthritis is intermittent, lasting one to three weeks, though manifestations may be persistent. As an example, in a study of Greek patients, the arthritis was oligoarticular in about two-thirds, monoarticular in about one-third, and polyarticular in less than 5 percent [27]. Despite its nonerosive and nondeforming character, one study using the multidimensional Health Assessment Questionnaire showed that arthritis was associated with overall functional impairment and pain similar to that seen in patients with rheumatoid arthritis [104].

Renal disease — Renal involvement in Behçet syndrome is less frequent and often less severe than in other types of vasculitis. Patients with renal disease may have proteinuria, hematuria, or mild renal insufficiency but can progress to renal failure.

Although urinary abnormalities (proteinuria and/or hematuria) occur in approximately 10 percent of patients, serious renal lesions are rare [105]. In a review of 4212 patients, glomerulonephritis was diagnosed by renal biopsy in only seven cases. This would represent a minimum incidence of disease.

The spectrum of renal diseases associated was illustrated in a review of 159 patients with Behçet syndrome: AA (secondary) amyloidosis was present in 69, glomerulonephritis in 51, vascular disease (mostly renal arterial aneurysms) in 35, and interstitial nephritis in 4 [106]. Among the patients with glomerulonephritis, there was a spectrum of lesions ranging from immunoglobulin A (IgA) nephropathy to crescentic glomerulonephritis.

Patients with AA amyloidosis typically present with nephrotic syndrome or at least significant proteinuria. In a series of 14 cases, the mean time from Behçet syndrome onset to amyloid nephropathy was eight years (range 3 to 15 years) [107].

Cardiac disease — Symptomatic cardiac disease is uncommon in Behçet syndrome. Abnormalities that can occur include pericarditis, myocarditis, coronary arteritis with or without myocardial infarction, coronary artery aneurysms, atrial septal aneurysm, conduction system disturbances, ventricular arrhythmias, endocarditis, endomyocardial fibrosis, mitral valve prolapse, intracardiac thrombosis, and valvular insufficiency including aortic regurgitation [108-115]. A review of 6636 patients with Behçet syndrome in the Korean National Health Insurance Service database demonstrated an increased incidence of atrial fibrillation of 2.3 per 1000 person-years compared with 1.1 in the control group [116].

Atherosclerosis does not appear to occur at an accelerated rate in most studies in Behçet syndrome as it does in other autoimmune diseases like systemic lupus erythematosus [15,117-120].

Gastrointestinal involvement — Symptoms of intestinal Behçet syndrome include abdominal pain, diarrhea, weight loss, and bleeding. Gastrointestinal involvement can be difficult to differentiate from inflammatory bowel disease, as other clinical signs and symptoms may also overlap, leading to difficult diagnostic issues. Gastrointestinal ulcerations occur in some patients with Behçet syndrome, and intestinal perforation can occur. Discrete ulcerations can be found throughout the gastrointestinal tract but are most often seen in the terminal ileum, cecum, and ascending colon [121,122]. Volcano-shaped ulcers in the ileocecal region have been described, and these findings should prompt consideration for Behçet syndrome. Pathology of deep ulcers shows chronically active nonspecific inflammation [123]. An association between Behçet syndrome, myelodysplasia, chromosomal trisomy 8 allele, and gastrointestinal involvement has been described [124-126].

Small intestinal bacterial overgrowth has been described in patients with treated Behçet intestinal disease in endoscopic and radiologic remission, and treatment of bacterial overgrowth may be beneficial for these patients [127].

Oral ulcers that frequently occur in patients with inflammatory bowel disease are indistinguishable from the oral aphthae of Behçet syndrome; thus, inflammatory bowel disease must be considered before making the diagnosis of Behçet syndrome. (See "Overview of gastrointestinal manifestations of vasculitis" and "Clinical manifestations, diagnosis, and prognosis of ulcerative colitis in adults" and "Clinical manifestations, diagnosis, and prognosis of Crohn disease in adults".)

The clinical course of intestinal Behçet syndrome is variable. In a study of 130 patients with intestinal Behçet syndrome, 75 percent had mild clinical activity or remission at five years, while 16 percent had multiple relapses or chronic symptoms. The group with multiple relapses or chronic symptoms was younger and had a higher erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and disease activity index, and a lower albumin at presentation [128].

Other

●Constitutional symptoms – Patients with Behçet syndrome may suffer from constitutional symptoms including fever and malaise [129]. A number of studies have reported lower sleep quality in patients with Behçet syndrome [130].

●Mood disorders – Depression and anxiety are more common in patients with Behçet syndrome [131-133].

●Inner ear – Inner ear involvement may cause tinnitus, deafness or hearing loss, or dizziness [134-136].

●Genitourinary symptoms – Lower urinary tract symptoms including urinary frequency, nocturia, urgency, and urinary incontinence are not uncommon [137]. Problems with urinary and erectile function may be due to neural or vascular disease [132,138].

●Amyloidosis – Amyloidosis is an uncommon complication of Behçet syndrome but is often fatal [107]. A cohort study of 3820 patients with Behçet syndrome estimated the prevalence of AA amyloidosis at 0.05 percent [139]. In this cohort, one-third of patients died within six months after the diagnosis of amyloidosis.

●Fibromyalgia – Fibromyalgia co-occurs in many patients with Behçet syndrome. The prevalence of fibromyalgia among patients with Behçet syndrome appears to be in the range of approximately 17 to 37 percent, based on a few small studies [140-142]. These studies have also found that fibromyalgia does not appear to be associated with disease activity. As an example, a study of 70 patients with Behçet syndrome found that 37.1 percent of patients met the American College of Rheumatology (ACR) criteria for fibromyalgia, and fibromyalgia was associated with anxiety and depression but not with Behçet syndrome activity [140]. In another study of 104 patients with Behçet syndrome, 18 of whom had fibromyalgia, fibromyalgia was not associated with disease activity but was associated with increased fatigue, headache, pain, arthralgia, and the patient's impression of disease activity [141].

●Vitamin deficiencies – Increased rates of iron, vitamin B12, and folate deficiencies have been observed in patients with Behçet syndrome [143]. This may be associated with the presence of serum gastric parietal cell antibodies in patients with Behçet syndrome [144].

●Possible increased malignancy risk – Several studies have suggested an increased risk of malignancy in patients with Behçet syndrome [145,146]. A meta-analysis of five studies observed a positive association between Behçet syndrome and cancer, with a pooled relative risk of 1.19 (95% CI, 1.09-1.30) [147]. In a cohort study including 451 cases of Behçet syndrome with a median age of 43, 11 malignancies were identified at a median of 124 months follow-up, which represented a standardized incidence ratio of 2.84 compared with expected counts [146]. Myelodysplasia has been described in Behçet patients, commonly presents with gastrointestinal manifestations, and is associated with chromosomal trisomy 8 allele [124-126,148-150].

●COVID-19-related issues – Most studies suggest that patients with Behçet syndrome have some increased risk of severe acute respiratory syndrome coronavirus disease 2 (SARS-CoV-2) infection, but do not have an increased risk of coronavirus disease 2019 (COVID-19)-associated mortality, though it is not uncommon for patients to suffer an exacerbation of their Behçet syndrome, often in association with not taking immunosuppressive treatment during their COVID-19 infection [151-157].

Exacerbations of Behçet syndrome after COVID-19 vaccination have been reported, most often resulting in mucocutaneous or articular symptoms [158]. More severe organ involvement such as uveitis, thrombosis, and stroke have rarely been described after vaccination [159]. Behçet patients on immunosuppressive therapy may have a reduced immune response to vaccination [160]. There are rare case reports of new-onset Behçet-like illnesses after COVID-19 vaccination [161,162].

LABORATORY TESTING — There are no pathognomonic laboratory tests in Behçet syndrome. Laboratory markers of inflammation such as erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) may be elevated in association with increased disease activity [163]. Other laboratory findings indicating possible disease activity may include increases in ferritin, white blood cells, neutrophils, platelets, plateletcrit, red blood cell distribution width (RDW), neutrophil-lymphocyte ratio, monocyte-lymphocyte ratio, and platelet-lymphocyte ratio [164-166]. The systemic immune-inflammation index (SII), calculated by multiplying neutrophils times platelets divided by lymphocytes, may be elevated in association with increased disease activity [167].

SPECIAL POPULATIONS

Children — Although there are limited data on children with Behçet syndrome, clinical manifestations appear to generally be similar to those in adults [10-12,168-173]. Among some populations, there may be differences in the frequencies or types of certain manifestations, including neurologic disease [12,82,174]. In a comparison of a pediatric clinical database and an adult clinical database from Florence, Italy, juvenile-onset disease was associated with more familiar predisposition, articular manifestations were more common in juvenile-onset disease, and venous vascular events were more common in adult-onset disease, while human leukocyte antigen (HLA) B51 positivity and other clinical manifestations occurred at similar rates [175].

Pregnant women — Studies suggest that in many patients with Behçet syndrome, disease activity is somewhat reduced during pregnancy; however, the risk of complications may be higher in patients with Behçet syndrome than in matched controls without Behçet syndrome [176-180]. In a retrospective analysis of 76 pregnancies in 46 patients with Behçet syndrome, 36 percent of pregnancies were associated with flares, and the annual incidence of flares per patient was approximately threefold lower during pregnancy than before or after pregnancy [178]. The overall rate of pregnancy complications was 16 percent. Complications included miscarriages, caesarean section, medical termination of pregnancy, HELLP syndrome (hemolysis, elevated liver enzymes, low platelet count), and immune thrombocytopenia. Complications were more common in patients with history of deep venous thrombosis, and flares were less common in patients on colchicine. A case control study of 31 patients during 135 pregnancies demonstrated higher rates of miscarriage, pregnancy complications, and cesarean sections in patients with Behçet syndrome compared with controls [176]. A retrospective analysis of 66 pregnancies in 26 women with Behçet syndrome reported miscarriages in 24.2 percent, intrauterine deaths in 3 percent, and preterm labor in 24 percent [180]. They observed an increased risk of preterm labor and low birthweight in patients with active Behçet syndrome and on colchicine treatment.

DIAGNOSIS

When to consider the diagnosis — Because oral aphthous ulcers are so common in the general population and Behçet syndrome is so rare, Behçet syndrome is best diagnosed in the context of recurrent aphthous ulcerations along with characteristic systemic manifestations. Systemic manifestations which should raise suspicion for Behçet syndrome include ocular disease, especially hypopyon, panuveitis, or retinal vasculitis; neurologic disease including characteristic central nervous system parenchymal findings; vascular disease, particularly pulmonary artery aneurysms, Budd-Chiari syndrome, and cerebral venous thrombosis; and patients with pathergy manifestations. Oral ulcerations also tend to be more frequent and severe in patients with Behçet syndrome. Genital ulcerations are more specific and less sensitive for the detection of Behçet syndrome. Behçet syndrome should also be considered more strongly in patients with the aforementioned symptoms who live along the Silk Road from eastern Asia to the Mediterranean Sea [181,182].

Our diagnostic criteria — There are no pathognomonic laboratory tests in Behçet syndrome; as a result, the diagnosis is made on the basis of the clinical findings. In the absence of other systemic diseases, we diagnose Behçet syndrome in patients with recurrent oral aphthae (at least three times in one year) plus two of the following clinical features:

●Recurrent genital aphthae (aphthous ulceration or scarring).

●Eye lesions (including anterior or posterior uveitis, cells in vitreous on slit lamp examination, or retinal vasculitis observed by an ophthalmologist).

●Skin lesions (including erythema nodosum, pseudofolliculitis, papulopustular lesions, or acneiform nodules consistent with Behçet syndrome).

●A positive pathergy test. Pathergy is defined by a papule 2 mm or more in size developing 24 to 48 hours after oblique insertion of a 20-gauge needle 5 mm into the skin, generally performed on the forearm.

This approach is consistent with the International Study Group (ISG) diagnostic criteria published in 1990 (table 2) [34]. These remain the most widely used and well-accepted criteria among experts in Behçet syndrome. (See 'Classification criteria' below.)

Pathergy is less common in Northern European and North American patients. Thus, it has been suggested that other features might be substituted for pathergy in these populations, including aseptic meningoencephalitis, cerebral vasculitis, recurrent phlebitis, arteritis, synovitis, epididymitis, or focal bowel ulceration [183].

There are patients who do not meet these criteria in whom the diagnosis of Behçet syndrome is still made, and establishing the diagnosis in such patients is much more difficult. Thus, it is often appropriate to refer a patient in whom the diagnosis of Behçet syndrome is suspected to a rheumatologist with experience in this disease.

Classification criteria — Several diagnostic and classification criteria have been developed for Behçet syndrome. Like other sets of criteria, these were developed to categorize patients for study purposes and were not developed to diagnose disease in individuals [34,184,185]. It has been suggested that their accuracy is better in populations with low prevalence than in those with high [185].

As mentioned above, we prefer the International Study Group (ISG) diagnostic criteria published in 1990 (table 2) [34]. These criteria appear to be relatively sensitive and specific [183,186]. As an example, in one report of 32 clinically diagnosed patients with Behçet syndrome and 56 controls with other rheumatic diseases, the sensitivity and specificity were 95 and 100 percent, respectively [183]. Crohn disease, ulcerative colitis, and familial Mediterranean fever share some clinical manifestations with Behçet syndrome. Including patients with these disorders among controls did not lead to substantially different sensitivity of specificity of the criteria [187].

The International Criteria for Behçet's disease (ICBD) were developed in 2006 in an effort to improve sensitivity compared with the ISG criteria, but they are not widely accepted [188,189]. Each of several findings is assigned a point value; the criteria require a total of at least four points for diagnosis of Behçet syndrome:

●Genital aphthosis – Two points

●Ocular lesions (anterior uveitis, posterior uveitis, or retinal vasculitis) – Two points

●Oral aphthosis – Two points

●Skin lesions (pseudofolliculitis or erythema nodosum) – One point

●Vascular lesions (superficial phlebitis, deep vein thrombosis, large vein thrombosis, arterial thrombosis, or aneurysm) – One point

●Neurologic manifestations – One point

●Pathergy – One point

Validation studies have estimated a sensitivity of 87 to 96.5 percent, a specificity of 73.7 to 95.6 percent, and an accuracy of 88.9 to 97.3 percent for these criteria [190].

Case-cohort analysis of two datasets, including possible Behçet's patients and mimickers from a uveitis registry, reported sensitivity of the ISG and ICBD criteria of 90.0 and 97.6 percent, respectively, and specificity of 98.8 and 90.8 percent with ICBD scores of 4 or greater, as well as sensitivity of the ISG and ICBD criteria of 92.3 and 97.5 percent, respectively, and specificity of 98.8 and 99.6 percent with ICBD scores of 5 or greater. Nested case-control analysis showed similar results [191].

In another study looking at Behçet syndrome in the United Kingdom, where the pretest probability of having Behçet syndrome is low, found that among 281 patients, the sensitivity of the ISG and ICBD criteria was 77.9 and 97.9 percent, respectively, with a specificity of 69.1 and 19.1 percent, highlighting the issues of using ICBD criteria in geographies where the pretest odds are low, as it has worse specificity, which would classify many more patients as having Behçet syndrome when they do not compared with the ISG criteria [192].

The Standardization of Uveitis Nomenclature (SUN) Working Group used a machine-learning method to develop a classification criterion for Behçet uveitis consisting of a diagnosis of Behçet syndrome via the ISG criteria in combination with characteristic uveitis including anterior uveitis, anterior and intermediate uveitis, or posterior or panuveitis with retinal vasculitis and/or focal retinal infiltrates, and they reported 94 to 96.3 percent overall accuracy [193].

A consensus classification criteria has been developed for pediatric Behçet syndrome that may be more sensitive than prior criteria in the pediatric population (table 3) [194,195].

Measures of disease damage — Instruments have been constructed that allow measurement of disease damage in Behçet syndrome patients. Both the Behçet’s Syndrome Overall Damage Index (BODI) and Behçet’s Disease Damage Index (BDI) have shown good convergent and discriminant validity with the vasculitis damage index (VDI) [196,197].

DIFFERENTIAL DIAGNOSIS — The differential diagnosis varies with each patient's clinical features [198,199]. While it is beyond the scope of this review to provide a comprehensive list of all the possible alternative diagnoses for Behçet syndrome, we present several here.

●Oral aphthae – Oral aphthae are present in almost all patients with Behçet syndrome, but can also be observed in a variety of other conditions (table 4). The differential diagnosis of recurrent oral ulcers include rheumatologic diseases such as systemic lupus erythematosus (SLE) and reactive arthritis; gastrointestinal diseases such as inflammatory bowel diseases and celiac disease; autoinflammatory conditions such as periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome, hyperimmunoglobulin D syndrome, and A20 haploinsufficiency (HA20) [200] (see "The autoinflammatory diseases: An overview"); infections such as herpes simplex virus (HSV) and human immunodeficiency virus (HIV); dermatologic diseases such as Stevens-Johnson syndrome, pemphigoid, pemphigus, lichen planus, and linear immunoglobulin A (IgA) bullous dermatosis; hematologic conditions such as cyclic neutropenia; as well as a variety of other conditions such as recurrent aphthous stomatitis (RAS) and nutritional deficiencies. Medications such as methotrexate and other chemotherapeutic agents can cause oral ulcers. Dental prosthetics and oral hygiene products can cause oral irritation and ulceration. (See "Oral lesions".)

●Genital ulcerations – Other causes of genital ulceration and an approach to the differential diagnosis and evaluation of genital ulcers are discussed in detail elsewhere. (See "Approach to the patient with genital ulcers".)

●Gastrointestinal symptoms – Many patients with Behçet syndrome experience abdominal pain and symptoms that may be similar to inflammatory bowel disease. In addition, gastrointestinal involvement from inflammatory bowel disease can be difficult to differentiate from Behçet syndrome. As an example, patients with Behçet syndrome may have gastrointestinal ulcerations that are indistinguishable from Crohn disease unless granulomas are identified in biopsy samples. (See "Clinical manifestations, diagnosis, and prognosis of Crohn disease in adults" and "Clinical manifestations, diagnosis, and prognosis of ulcerative colitis in adults".)

●Ocular inflammation – Uveitis, a feature of Behçet syndrome, can also occur with Crohn disease, ulcerative colitis, spondyloarthritis, and other conditions. However, conjunctivitis, scleritis, episcleritis, and sicca syndrome are uncommon and should increase the suspicion for an alternative diagnosis. (See "Uveitis: Etiology, clinical manifestations, and diagnosis".)

●Arthritis – The asymmetric, non-erosive arthritis that can be observed in patients with Behçet syndrome can also be seen with other systemic rheumatic diseases including systemic lupus erythematosus (SLE), reactive arthritis, ankylosing spondylitis, juvenile idiopathic arthritis, psoriatic arthritis, and some vasculitides. An asymmetric, non-erosive arthritis can also be a manifestation of sarcoidosis and inflammatory bowel disease. (See "Evaluation of the adult with polyarticular pain".)

●Drug-related – There have been case reports of Behçet-type disease manifestations in patients treated with interleukin (IL) 17 inhibitors including secukinumab [201-204].

●Other – A factitious "pseudo-Behçet" syndrome has also been described, with predominant mucocutaneous involvement.

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Behçet syndrome" and "Society guideline links: Vasculitis".)

INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)

●Basics topic (see "Patient education: Behçet syndrome (The Basics)")

●Beyond the Basics topic (see "Patient education: Vasculitis (Beyond the Basics)")

Patient information can also be obtained from The American Behçet's Disease Association:

The American Behçet's Disease Association

PO Box 80576

Rochester, MI 48308

Telephone: 1-800-723-4238 or 1-800-7-BEHCET

Email: [email protected]

www.behcets.com

SUMMARY AND RECOMMENDATIONS

●Definition – Behçet syndrome is a rare disease characterized by recurrent oral aphthae and any of several systemic manifestations including genital aphthae, ocular disease, skin lesions, gastrointestinal disease, neurologic disease, vascular disease, and arthritis. Most clinical manifestations of Behçet syndrome are believed to be due to vasculitis. (See 'Introduction' above.)

●Epidemiology – Behçet syndrome is more common (and often more severe) along the ancient silk road, which extends from eastern Asia to the Mediterranean; it is most common in Turkey, while the prevalence is much lower in North America and northern Europe. It typically affects young adults 20 to 40 years of age. (See 'Epidemiology' above.)

●Clinical manifestations

•Oral ulcerations and urogenital lesions – The common clinical feature in patients with Behçet syndrome is the presence of recurrent and usually painful mucocutaneous ulcers (picture 1A-G). Other clinical manifestations of this disorder are more variable among different patients and populations. (See 'Clinical manifestations' above and 'Oral ulcerations' above and 'Urogenital lesions' above.)

•Ocular, neurologic, and vascular involvement – The greatest morbidity and mortality occur with ocular disease (affecting up to two-thirds of patients), including uveitis and other changes; with vascular disease, including pulmonary artery aneurysms; and with central nervous system disease, including focal parenchymal lesions, complications of vascular thrombosis, and other abnormalities. Vasculitis in patients with Behçet syndrome is remarkable for its ability to involve blood vessels of all sizes and to involve both arteries and veins (table 1). (See 'Vascular disease' above and 'Ocular disease' above and 'Neurologic disease' above.)

•Other manifestations – Cutaneous manifestations are common, which include acne, folliculitis, and erythema nodosum lesions. Pathergy may be seen as an erythematous papular or pustular response to local skin injury. Arthritis may be present, which is characteristically intermittent, inflammatory but nonerosive, asymmetric, and, usually, nondeforming; it often occurs during disease exacerbations and most commonly affects the medium and large joints. A variety of gastrointestinal symptoms may also occur. (See 'Cutaneous lesions' above and 'Arthritis' above and 'Gastrointestinal involvement' above.)

•Less common manifestations – Less common manifestations include renal disease and peripheral nervous system involvement. Cardiac and pulmonary disease may also be present. (See 'Renal disease' above and 'Neurologic disease' above and 'Cardiac disease' above and 'Pulmonary disease' above.)

●Diagnosis – There are no pathognomonic laboratory tests in Behçet syndrome; as a result, the diagnosis is made on the basis of the clinical findings. In the absence of other systemic diseases, we diagnose Behçet syndrome in patients with recurrent oral aphthae (at least three times in one year) plus two of the following clinical features:

•Recurrent genital aphthae

•Eye lesions (including anterior or posterior uveitis, cells in vitreous on slit lamp examination, or retinal vasculitis observed by an ophthalmologist)

•Skin lesions (including erythema nodosum, pseudo-vasculitis, papulopustular lesions, or acneiform nodules consistent with Behçet syndrome)

•A positive pathergy test.

This approach is consistent with the International Study Group (ISG) criteria published in 1990 (table 2). (See 'Diagnosis' above.)

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Topic 8223 Version 40.0

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110 : Cardiovascular involvement in Behçet's disease.

111 : Spectrum of cardiac lesions in Behçet disease: a series of 52 patients and review of the literature.

112 : A rare but serious manifestation of Behçet's disease: intracardiac thrombus in 22 patients.

113 : Intracardiac thrombus in patients with Behcet's disease: clinical correlates, imaging features, and outcome: a retrospective, single-center experience.

114 : Characteristic Echocardiographic Manifestations of Behçet's Disease.

115 : Coronary Involvement in Behçet's Disease: what are its Risks and Prognosis? (Rare Cases and Literature Review).

116 : Increased risk of atrial fibrillation in patients with Behçet's disease: A nationwide population-based study.

117 : Is Behçet's syndrome associated with an increased risk of ischemic heart disease? A real-world evidence in Taiwan.

118 : Ischaemic heart disease in Behçet's syndrome: a systematic review and meta-analysis.

119 : Investigating the link between ischemic heart disease and Behcet's disease: A cross-sectional analysis.

120 : Risk of ischaemic heart diseases and stroke in behçet disease: A systematic review and meta-analysis.

121 : An update on the diagnosis, treatment, and prognosis of intestinal Behçet's disease.

122 : Intestinal Behçet and Crohn's disease: two sides of the same coin.

123 : Evidence-based diagnosis and clinical practice guidelines for intestinal Behçet's disease 2020 edited by Intractable Diseases, the Health and Labour Sciences Research Grants.

124 : Gastrointestinal Behcet's-like disease with myelodysplastic neoplasms with trisomy 8: a French case series and literature review.

125 : Cancer Risk in Patients with Intestinal Behçet's Disease: A Nationwide Population-Based Study.

126 : How to treat myelodysplastic syndrome with clinical features resembling Behçet syndrome: a case-based systematic review.

127 : Rediscover the clinical value of small intestinal bacterial overgrowth in patients with intestinal Behçet's disease.

128 : Clinical course of intestinal Behcet's disease during the first five years.

129 : Fever in Behçet's syndrome.

130 : Sleep quality in Behçet's disease: a systematic literature review.

131 : The psychological impact of Behçet's disease.

132 : Sexual dysfunction and depression in Behçet's disease in comparison to healthy controls.

133 : Association Between Behçet's Disease and Depression.

134 : Audio-vestibular disturbance in patients with Behçet's disease.

135 : Behçet disease as a cause of hearing loss: A prospective, placebo-controlled study of 29 patients.

136 : Auditory involvement in Behcet's disease: relationship with demographic, clinical, and therapeutic characteristics.

137 : Is There a Correlation Between Behçet Disease and Lower Urinary Tract Symptoms?

138 : Evaluation and therapeutic approaches of voiding and erectile dysfunction in neurological Behçet's syndrome.

139 : Frequency of AA amyloidosis has decreased in Behçet's syndrome: a retrospective study with long-term follow-up and a systematic review.

140 : Fibromyalgia in Behçet's disease is associated with anxiety and depression, and not with disease activity.

141 : The prevalence of fibromyalgia in patients with Behçet's disease and its relation with disease activity.

142 : The Frequency of Fibromyalgia and its Relationship With Disease Activity in Female Patients With Behçet's Disease: A Cross Sectional Study.

143 : Significantly higher frequencies of hemoglobin, iron, vitamin B12, and folic acid deficiencies and of hyperhomocysteinemia in patients with Behcet's disease.

144 : Hematinic deficiencies and hyperhomocysteinemia in gastric parietal cell antibody-positive or gastric and thyroid autoantibodies-negative Behcet's disease patients.

145 : Cancer risk in patients with Behçet disease: A nationwide population-based dynamic cohort study from Korea.

146 : Cancer incidence in Behçet's disease.

147 : Risk of malignancy in Behcet disease: A meta-analysis with systematic review.

148 : The association of Behçet's disease with myelodysplastic syndrome in Japan: a review of the literature.

149 : High incidence of morphological myelodysplasia and apoptotic bone marrow cells in Behçet's disease.

150 : Behcet's disease associated with bone marrow failure in Korean patients: clinical characteristics and the association of intestinal ulceration and trisomy 8.

151 : Management of patients with Behçet's disease during the COVID-19 pandemic.

152 : Prevalence and clinical course of SARS-CoV-2 infection in patients with Behçet's syndrome.

153 : Coronavirus disease 2019 in patients with Behcet's disease: a report of 59 cases in Iran.

154 : Clinical course of COVID-19 infections in patients with Behçet's disease in The Netherlands.

155 : Clinical course of Covid-19 in a cohort of patients with Behçet disease.

156 : COVID-19 in patients with Behçet's disease: Outcomes and rate of Behçet's exacerbations in a retrospective cohort.

157 : The incidence, clinical characteristics, and outcome of COVID-19 in a prospectively followed cohort of patients with Behçet's syndrome.

158 : Effects of anti-SARS-CoV-2 vaccination on safety and disease exacerbation in patients with Behçet syndrome in a monocentric cohort.

159 : Safety of SARS-CoV-2 vaccination in patients with Behcet's syndrome and familial Mediterranean fever: a cross-sectional comparative study on the effects of M-RNA based and inactivated vaccine.

160 : Behçet's patients' response to COVID-19 vaccination.

161 : Aseptic Meningitis, Mucocutaneous Lesions and Arthritis after COVID-19 Vaccination in a 15-Year-Old Boy.

162 : Emergence of Behçet's disease post-SARS-CoV2-vaccination: two clinical cases in Japan.

163 : Predictive value of erythrocyte sedimentation rate and C-reactive protein in Behcet's disease activity and manifestations: a cross-sectional study.

164 : Effect of combined colchicine-corticosteroid treatment on neutrophil/lymphocyte ratio: a predictive marker in Behçet disease activity.

165 : Correlation of clinical signs and symptoms of Behçet's disease with platelet-to-lymphocyte ratio (PLR) and neutrophil-to-lymphocyte ratio (NLR).

166 : Evaluation of diagnostic performance of haematological parameters in Behçet's disease.

167 : A cutoff value for the Systemic Immune-Inflammation Index in determining activity of Behçet disease.

168 : Paediatric Behçet's disease: a UK tertiary centre experience.

169 : Paediatric Behçet's disease in Iran: report of 204 cases.

170 : A national cohort study on pediatric Behçet's disease: cross-sectional data from an Italian registry.

171 : Behçet's Disease in Children: Diagnostic and Management Challenges.

172 : Clinical profile and evolution of patients with juvenile-onset Behçet's syndrome over a 25-year period: insights from the AIDA network.

173 : Juvenile Behçet's disease: a tertiary center experience.

174 : Cluster analysis of paediatric Behçet's disease: Data from The Pediatric Rheumatology Academy-Research Group.

175 : Behçet syndrome in children and adults: discovering similarities and differences by a comparative study.

176 : Behçet's disease and pregnancy.

177 : The clinical course of Behçet's disease in pregnancy: a retrospective analysis and review of the literature.

178 : Behçet's disease and pregnancy.

179 : Behçet's disease and pregnancy: a retrospective analysis of course of disease and pregnancy outcome.

180 : Behcet's disease and pregnancy: what to expect?

181 : Behçet's disease: treatment of mucocutaneous lesions.

182 : Influence of age of onset and patient's sex on the prevalence and severity of manifestations of Behçet's syndrome.

183 : Sensitivity and specificity of different diagnostic criteria for Behçet's disease according to the latent class approach.

184 : The American College of Rheumatology criteria for the classification of systemic lupus erythematosus: strengths, weaknesses, and opportunities for improvement.

185 : The proportional Venn diagram of Behçet's disease-related manifestations among young adult men in Turkey.

186 : Validation of the International Study Group criteria for Behçet's disease.

187 : A reassessment of the International Study Group criteria for the diagnosis (classification) of Behçet's syndrome.

188 : A reassessment of the International Study Group criteria for the diagnosis (classification) of Behçet's syndrome.

189 : The International Criteria for Behçet's Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria.

190 : Diagnosis/Classification Criteria for Behcet's Disease.

191 : Evaluation of sensitivity and specificity of diagnostic criteria for Behçet's disease in the absence of a gold standard.

192 : Birmingham Behçet's service: classification of disease and application of the 2014 International Criteria for Behçet's Disease (ICBD) to a UK cohort.

193 : Classification Criteria for Behçet Disease Uveitis.

194 : Consensus classification criteria for paediatric Behçet's disease from a prospective observational cohort: PEDBD.

195 : The performance of different classification criteria in paediatric Behçet's disease.

196 : Validity and reliability of the BODI for assessing damage in Behcet's disease.

197 : Damage accrual in Behçet disease: Behçet's Syndrome Overall Damage Index (BODI) versus Behçet's Disease Damage Index (BDI).

198 : Behçet's syndrome.

199 : Differential diagnosis and management of Behçet syndrome.

200 : A20 haploinsufficiency (HA20): clinical phenotypes and disease course of patients with a newly recognised NF-kB-mediated autoinflammatory disease.

201 : Secukinumab induced Behçet's syndrome: a report of two cases.

202 : Report of two cases of Behçet's disease developed during treatment with secukinumab.

203 : Behçet's-like disease during secukinumab treatment: new paradoxical reaction?

204 : A case report of recalcitrant aphthous ulcers in two patients treated with interleukin-17 inhibitors.

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