ARTHRITIS & RHEUMATISM Vol. 62, No. 9, September 2010, pp 2806–2812 DOI 10.1002/art.27568 © 2010, American College of Rheumatology Mortality in Behçet’s Disease D. Saadoun,1 B. Wechsler,1 K. Desseaux,2 D. Le Thi Huong,1 Z. Amoura,1 M. Resche-Rigon,2 and P. Cacoub1 Conclusion. The overall mortality in our BD cohort was 5% after a median followup of 7.7 years. Male sex, arterial involvement, and the number of flares were associated with mortality in BD. Objective. To report the long-term mortality in patients with Behçet’s disease (BD). Methods. A cohort of 817 patients fulfilling the international criteria for BD from a single center in France were analyzed for causes of death, the standardized mortality ratio (SMR), and the factors associated with mortality. Results. Among the 817 patients with BD, 41 (5%) died after a median followup of 7.7 years, of whom 95.1% were male. The mean ⴞ SD age at death was 34.8 ⴞ 11.9 years. Main causes of death included major vessel disease (mainly, arterial aneurysm and Budd-Chiari syndrome) (43.9%), cancer and malignant hemopathy (14.6%), central nervous system involvement (12.2%), and sepsis (12.2%). The mortality rate at 1 year and 5 years was 1.2% and 3.3%, respectively. There was an increased mortality among patients ages 15–24 years (SMR 2.99, 95% confidence interval [95% CI] 1.54– 5.39) and those ages 25–34 years (SMR 2.90, 95% CI 1.80–4.49) as compared with age-and sex-matched healthy controls. The mortality decreased in patients older than age 35 years (SMR 1.23, 95% CI 0.75–1.92). In multivariate analyses, male sex (hazard ratio [HR] 4.94, 95% CI 1.53–16.43), arterial involvement (HR 2.51, 95% CI 1.07–5.90), and a high number of BD flares (HR 2.37, 95% CI 1.09–5.14) were independently associated with the risk of mortality. Behçet’s disease (BD) is a chronic, relapsing vasculitis of unknown etiology that is characterized by mucocutaneous, ocular, articular, vascular, gastrointestinal, and central nervous system manifestations (1,2). BD significantly increases morbidity and mortality. The leading cause of morbidity in BD is eye involvement with the potential threat of visual loss. Few studies have addressed the mortality of Behçet’s syndrome. Among 2,031 patients from Japan, 31.7% experienced clinical deterioration, and 0.9% died during the course of a single year of followup. In Turkey, 42 of 428 patients died, mainly due to major vessel disease and neurologic involvement. However, the remaining studies were mostly short term or had a small cohort, with the focus being on mortality related to a single organ system (i.e., central nervous system, major vessels). Furthermore, most of the reports on mortality in patients with BD come from Japan or Turkey. BD shows considerable geographic variation in clinical expression. For instance, patients with BD from Asia exhibit a higher frequency of gastrointestinal involvement as compared with those from the Mediterranean basin (3,4). The present study was undertaken to report the long-term mortality in BD. We analyzed the main causes of death and the standardized mortality ratio (SMR) in a cohort of 817 patients with BD from a single university center in France. Factors associated with mortality were assessed by multivariate analysis. 1 D. Saadoun, MD, PhD, B. Wechsler, MD, D. Le Thi Huong, MD, Z. Amoura, MD, P. Cacoub, MD: Hôpital Pitié-Salpétrière, Assistance Publique-Hôpitaux de Paris, and Université Pierre et Marie Curie-Paris 6, Paris, France; 2K. Desseaux, MD, M. Resche-Rigon, MD, PhD: INSERM U717, Hôpital Saint-Louis, Assistance PubliqueHôpitaux de Paris, Paris, France. Dr. Cacoub has received consulting fees, speaking fees, and/or honoraria from AstraZeneca, Bristol-Myers Squibb, SanofiAventis, Gilead Sciences, and Schering-Plough (less than $10,000 each) and from Roche and Servier (more than $10,000 each). Address correspondence and reprint requests to D. Saadoun, MD, PhD, Service de Médecine Interne, AP-HP, Hôpital PitiéSalpêtrière, Service de Médecine Interne, 83 Boulevard de l’Hôpital, Paris F-75013, France. E-mail: email@example.com. Submitted for publication November 14, 2009; accepted in revised form May 11, 2010. PATIENTS AND METHODS Patients. Clinical records of 817 consecutive patients fulfilling the international criteria for the classification of BD (5) were analyzed. All patients were referred to, and regularly followed up in, the internal medicine department of the Pitié-Salpétrière university hospital in Paris, France between 2806 MORTALITY IN BD 2807 Table 1. Characteristics of the patients with Behçet’s disease (BD) and comparison between patients who died and those who remained alive* Parameter All (n ⫽ 817) Died (n ⫽ 41) Age at diagnosis, mean ⫾ SD years Male sex Ethnic origin Europe North Africa Africa Other HLA–B5 (n ⫽ 642) Oral ulceration Genital ulceration Ocular involvement CNS involvement Articular involvement Venous involvement Arterial involvement Number of BD flares, mean ⫾ SD BD flares ⱖ5 Immunosuppressants 31.5 ⫾ 10.5 541 (66.2) 34.8 ⫾ 11.9 38 (92.7) 365 (44.7) 336 (41.1) 73 (8.9) 43 (5.3) 259 (40.3) 812 (99.4) 568 (69.5) 514 (62.9) 220 (26.9) 354 (43.3) 301 (36.8) 114 (13.9) 3.7 ⫾ 2.6 179 (21.9) 431 (52.8) 15 (36.6) 19 (46.3) 4 (9.8) 3 (7.1) 11/31 (35.5) 41 (100) 18 (43.9) 25 (61) 15 (36.6) 20 (48.8) 18 (43.9) 15 (36.6) 4.0 ⫾ 2.7 12 (29.3) 31 (75.6) Alive (n ⫽ 776) 31.4 ⫾ 10.4 503 (64.8) 350 (45.1) 317 (40.8) 69 (8.9) 40 (5.1) 300/611 (49.1) 771 (99.3) 550 (70.8) 489 (63.0) 205 (26.4) 334 (43.0) 283 (36.5) 99 (12.8) 3.6 ⫾ 2.7 167 (21.5) 400 (51.6) * Except where indicated otherwise values are the number (%) of patients. CNS ⫽ central nervous system. 1974 and 2006. For each patient, the following data were collected: age at diagnosis of BD, sex, date of satisfaction of criteria for BD, geographic origin, and main features of BD, including mucocutaneous manifestations, ocular lesions, rheumatic disease manifestations (arthralgias, arthritis), neurologic involvement, and/or vascular involvement (venous and arterial lesions). The number of BD flares, treatment, outcome, and causes of death were recorded. Six hundred forty-two patients with BD were investigated for HLA–B5 typing. Assessment of the SMR. To compare the observed mortality with the expected mortality, the SMR was used. The SMR is the ratio of the observed mortality in patients to the mortality in the total French population of subjects with corresponding sex and year of birth. Only patients who were adults at diagnosis (age ⱖ15 years) were considered. Expected mortality rates were obtained from the French National Statistical Institute (http://www.ined.fr/fr/pop_chiffres/france/ mortalite_causes_deces/deces_sexe_age). Patients were divided into 3 age groups (ages 15–24 years, 25–34 years, and ⱖ35 years). Statistical analysis. Data are summarized as frequencies and percentages for categorical variables. Quantitative variables are presented as the median and 25th–75th percentiles (interquartile range [IQR]) or mean ⫾ SD. To take into account censored observations, the Kaplan-Meier method was used to estimate survival curves. The percentage of patients with a length of followup of ⬍6 months, 1 year, and 2 years was 5%, 10%, and 17%, respectively. Factors associated with the occurrence of death were assessed using a Cox proportional hazards model. Hazard ratios (HRs) with their 95% confidence interval (95% CI) are presented as a measure of association. Occurrences of first oral ulcerations, genital ulcerations, eye involvement, articular involvement, venous involvement, and central nervous system involvement were considered as time-dependent covariates, as well as having ⱖ5 BD flares. All factors with a P value lower than 0.05 in the univariate analysis were included in a Cox proportional hazards multivar- iate model. All tests were 2-sided at the 0.05 significance level. Confidence intervals were calculated directly from the Poisson distribution (6,7). Analyses were performed using the R statistical package. RESULTS Characteristics of the patients. The main features of the 817 patients with BD are summarized in Table 1. The mean ⫾ SD age at diagnosis was 31.5 ⫾ 10.5 years, and 541 (66.2%) of the patients were male. Most of the patients originated from Europe (44.7%) and North Africa (41.1%). The HLA–B5 typing was positive in 40.3% of patients. The main clinical signs of BD included recurrent oral ulcerations (99.4%) and genital ulcerations (69.5%), skin lesions (mainly, pseudofolliculitis) (61.4%), eye involvement (62.9%), articular involvement (43.3%), venous involvement (36.8%), central nervous system involvement (26.9%), and arterial involvement (13.9%). The mean ⫾ SD number of BD flares was 3.7 ⫾ 2.6. In comparing the main characteristics between patients who died and those who remained alive, a higher frequency of male patients (92.7% versus 64.8%, respectively), a higher number of BD flares (mean ⫾ SD 4.0 ⫾ 2.7 versus 3.6 ⫾ 2.7, respectively), a higher frequency of arterial involvement (36.6% versus 12.8%, respectively), a higher proportion of patients receiving immunosuppressant drugs (75.6% versus 51.6%, respectively), and a lower frequency of genital ulcerations (43.9% versus 70.8%, respectively) were observed among the patients who died (Table 1). 2808 SAADOUN ET AL Table 2. Causes of death in 41 patients with Behçet’s disease (BD) Patient/sex/ age at death, years 1/M/57 2/M/43 3/M/36 4/M/40 5/M/32 6/M/32 7/M/40 8/M/30 9/M/44 10/M/21 11/M/27 12/M/18 13/M/34 14/M/22 15/M/24 16/M/22 17/M/39 18/M/22 19/M/24 20/M/57 21/M/48 22/M/37 23/M/33 24/M/29 25/M/48 26/M/28 27/M/30 28/M/29 29/M/58 30/M/40 31/M/48 32/F/29 33/M/34 34/F/22 35/M/27 36/M/61 37/M/33 38/M/27 39/M/17 40/M/27 41/M/49 Time between diagnosis of BD and death, months Number of BD flares Cause of death 3 4 4 4 4 7 7 9 9 12 14 14 20 29 34 36 36 37 37 41 47 50 50 51 52 54 57 74 77 77 82 82 92 98 119 136 156 159 183 228 290 3 4 1 2 3 1 1 3 6 2 3 2 2 1 3 14 4 7 3 2 3 7 2 6 4 2 1 9 3 4 6 3 1 3 6 4 6 8 8 8 2 Pseudomonas septicemia Pulmonary artery aneurysm (massive hemoptysis) Candidosis septicemia Thoracic aortic aneurysm (death after surgical repair) Cerebral aneurysm (sudden death by rupture) Pulmonary arterial aneurysm (massive hemoptysis) Ischemic heart disease Endocarditis Thoracic and abdominal aortic aneurysm (rupture of thoracic aneurysm) Budd-Chiari syndrome Pulmonary arterial aneurysm (Hughes Stovin syndrome) Budd-Chiari syndrome Budd-Chiari syndrome Pulmonary embolism Central nervous system disease with severe progressive course Acute renal failure (thrombotic microangiopathy during cyclosporine therapy) Ischemic heart disease Abdominal aortic aneurysm (sudden death by rupture) Unknown Carcinoma (lung) Carcinoma (tongue) Tuberculosis Ischemic heart disease Pulmonary embolism (during thalidomide therapy) Thoracic aortic aneurysm (death after surgical repair) Unknown Budd-Chiari syndrome (rupture of esophageal varice) Pancreatitis Acute leukemia Central nervous system disease with severe progressive course Central nervous system disease with severe progressive course Central nervous system disease with severe progressive course Unknown Pseudomonas septicemia AA amyloidosis (renal and digestive) Non-Hodgkin’s lymphoma, apsergillosis Carcinoma (bladder) Central nervous system disease with severe progressive course Pulmonary embolism (during thalidomide therapy) Unknown Non-Hodgkin’s lymphoma When we compared the characteristics of the male patients with those of female patients, we found differences in terms of geographic origin, in that male patients originated from either Europe (39.1%) or northern Africa (47.7%), whereas female patients were mostly from Europe (66.2%). There was a higher number of BD flares (mean ⫾ SD 3.7 ⫾ 2.6 versus 3.5 ⫾ 2.8, respectively) and a higher frequency of arterial involvement (90 [16.6%] of 540 versus 14 [5.1%] of 276, respectively) in male patients compared with female patients. However, there were no significant differences according to age at diagnosis, HLA–B5 status, frequency of main clinical features of BD, or immunosuppressant use. In the multivariate analysis, among male patients, arterial involvement (HR 2.5, 95% CI 1.2–5.6), genital ulceration (HR 0.5, 95% CI 0.3–1.1), and a high frequency (ⱖ5) of BD flares (HR 2.5, 95% CI 1.2–5.6) were independently associated with mortality. Causes of death. Table 2 summarizes the main causes of death in the 41 patients with BD who died over followup. The mean ⫾ SD age at death was 34.8 ⫾ 11.9 years, with a mean time between diagnosis of BD and death of 62.8 ⫾ 64.8 months. Main causes of death included arterial involvement (pulmonary arterial aneurysm [n ⫽ 3], thoracic aortic aneurysm [n ⫽ 3], myocardial infarction [n ⫽ 3], abdominal aortic aneurysm [n ⫽ MORTALITY IN BD 2809 Figure 1. Survival curve over followup of the 817 patients with Behçet’s disease, among all patients (A) and according to sex (B) and ethnic origin (C). 1], and cerebral aneurysm [n ⫽ 1]; 26.8% of patients who died), large venous involvement (Budd-Chiari syndrome [n ⫽ 4)] or pulmonary embolism [n ⫽ 3]; 17.1% of patients who died), cancer (lung, tongue, or bladder carcinoma [n ⫽ 1 each], malignant hemopathy [nonHodgkin’s lymphoma] [n ⫽ 2], and acute leukemia [n ⫽ 1]; 14.6% of patients who died), central nervous system involvement (n ⫽ 5; 12.2% of patients who died), and sepsis (pseudomonas septicemia [n ⫽ 2], candidosis septicemia [n ⫽ 1], tuberculosis [n ⫽ 1], and endocarditis [n ⫽ 1]; 12.2% of patients who died). Three patients who died from sepsis had an underlying disease, i.e., diabetes (n ⫽ 2) and bronchiectasis (n ⫽ 1). Other causes of death included pancreatitis (n ⫽ 1), AA amyloidosis (n ⫽ 1), thrombotic microangiopathy (n ⫽ 1), and unknown (n ⫽ 4). Mortality rates and SMRs. The cumulative rate of death was 1.2%, 2.1%, 3.3%, and 4.3% at 1, 3, 5, and 10 years, respectively (Figure 1). After a median followup of 7.7 years (IQR 3.4–13 years), 41 patients with BD (5%) died. The mortality as measured by the SMR (Figure 2) was divided into 3 age groups (15–24 years, 25–34 years, and ⱖ35 years) based on age at diagnosis of BD. In this analysis, 777 patients age 15 years or older were included. There was an increased mortality among the patients ages 15–24 years (SMR 2.99, 95% CI 1.54–5.39) and those ages 25–34 years (SMR 2.90, 95% CI 1.80–4.49) (Figure 2). The mortality decreased in patients older than age 35 years (SMR 1.23, 95% CI 0.75–1.92). Figure 2. Standardized mortality ratio (SMR) with 95% confidence intervals for 777 patients with Behçet’s disease. Only patients who were adults at diagnosis (age ⱖ15 years) were considered. Horizontal line indicates the cutoff. 2810 SAADOUN ET AL Table 3. Factors associated with mortality in Behçet’s disease (BD)* Univariate analysis Multivariate analysis Parameter HR (95% CI) P HR (95% CI) P Age at diagnosis Male sex Geographic origin Europe North Africa Africa Other Number of BD flares HLA–B5 Oral ulcerations Genital ulcerations Ocular involvement CNS involvement Articular involvement Venous involvement Arterial involvement Immunosuppressants 1.38 (0.6–3.3) 6.87 (2.1–22.3) 0.49 0.001 4.94 (1.5–16.4) 0.007 1 2.48 (0.8–7.4) 1.71 (0.8–3.3) 2.45 (0.7–8.5) 2.47 (0.1–1.6) 0.51 (0.2–1.1) 0.54 (0.1–2.2) 0.41 (0.2–0.7) 0.78 (0.3–1.6) 1.42 (0.8–3.3) 1.02 (0.5–1.9) 1.06 (0.1–2.2) 3.3 (1.4–7.8) 2.39 (1.1–4.8) 0.11 0.12 0.16 0.018 0.07 0.39 0.006 0.51 0.42 0.96 0.88 0.005 0.017 2.37 (1.1–5.1) 0.029 0.49 (0.2–0.9) 0.044 2.51 (1.1–5.9) 0.034 * HR ⫽ hazard ratio; 95% CI ⫽ 95% confidence interval; CNS ⫽ central nervous system. Factors associated with mortality. Results of the logistic regression analysis of factors associated with mortality are summarized in Table 3. In the univariate analysis, there was no significant association between mortality and age, HLA–B5 status, ethnic origin, oral ulceration, articular involvement, central nervous system involvement, or ocular and venous involvement. Among the patients who died, patients were predominantly male and had a significantly higher frequency of BD flares, a significantly higher frequency of corticosteroid and immunosuppressant use, a significantly higher frequency of arterial involvement, and a significantly lower frequency of genital ulceration. In the multivariate analysis, male sex (HR 4.94, 95% CI 1.53–16.43), arterial involvement (HR 2.51, 95% CI 1.07–5.90), genital ulceration (HR 0.49, 95% CI 0.25–0.98), and a high frequency (ⱖ5) of BD flares (HR 2.37, 95% CI 1.09–5.14) were independently and significantly associated with mortality (Table 3). We next tested the impact of being nonEuropean versus being European on the risk of death (results not shown). Among European patients with BD (n ⫽ 365), there were 15 deaths (4.1%), while among non-European patients (n ⫽ 452), there were 26 deaths (5.7%). In the univariate analysis, the HR for the risk of mortality in non-European patients was 1.85 (95% CI 0.98–3.57) (P ⫽ 0.06). In the multivariate analysis, male sex (HR 4.6, 95% CI 1.4–15.2), arterial involvement (HR 2.4, 95% CI 1.1–5.3), genital ulceration (HR 0.5, 95% CI 0.2–0.9), and a high frequency (ⱖ5) of BD flares (HR 2.4, 95% CI 1.1–5.3) were independently and significantly associated with mortality. The HR for the risk of mortality in non-European patients was 1.3 (95% CI 0.7–2.5) (P ⫽ 0.45). DISCUSSION In the present study, we assessed the long-term mortality in a cohort of 817 patients with BD. We analyzed the main causes of death and the SMR in our BD cohort. Factors associated with mortality were assessed by multivariate analysis. The most striking findings from this study are 1) the overall mortality of 5% among patients with BD after a median followup of 7.7 years, 2) the 3 times higher mortality in BD patients younger than age 35 years, 3) the independent association of mortality with male sex, arterial involvement, and a high number of BD flares, and 4) the specific causes of death in our patients with BD. Among 817 patients with BD, 41 (5%) died after a median followup of 7.7 years. This was consistent with the results of previous studies from Morocco (10 deaths [3.2%] in 316 patients]) and from Turkey (6 deaths [3.9%] in 152 patients) (8,9). However, the investigators in Turkey reported the long-term mortality in their cohort after surveying the data over 2 decades, which yielded a mortality rate of 9.8% (10). In the present study, most deaths occurred 5 years after the diagnosis of BD. The main causes of death included major vessel disease (i.e., arterial aneurysm and Budd-Chiari syndrome) and central nervous system involvement. Although not independently associated with mortality, central nervous system involvement accounted for 12% MORTALITY IN BD of deaths in our study. In large studies specifically addressing neurologic disease in BD, the mortality rate ranged between 5.5% and 20% (11,12). The median period until death was 4 years after onset of neurologic signs (11). The frequency of arterial involvement was 3 times higher among the patients who died. In the multivariate analysis, patients with BD who developed arterial involvement had a 2.5 higher likelihood of dying. Thoracic aorta and pulmonary arterial aneurysms were associated with the higher mortality. The rate of death in patients with aneurysm rupture has been found to be as high as 60% (13). We previously reported that the survival rate of patients with BD complicated by arterial lesions was 66% at 15 years (14). The most severe complication of pulmonary vasculitis is pulmonary arterial aneurysms (13,15,16). In a study by Hamuryudan et al involving 24 patients with BD who experienced pulmonary arterial aneurysm (16), 12 (50%) of the patients died 9.5 months after the onset of hemoptysis, despite receiving therapy. In a more recent report on patients with pulmonary arterial aneurysm, the same group observed an overall 5-year survival rate of 63%; patients diagnosed since 1992 had a 5-year survival rate of 80%. The improved prognosis was believed to be due to earlier diagnosis and rational use of immunosuppressive agents (17). Pulmonary arterial vasculitis almost exclusively affects male patients and has a strong association with a systemic pattern of vessel involvement at other sites (13,16). In addition to arterial involvement, large venous lesions were associated with an important increase in mortality, especially in those with Budd-Chiari syndrome. In the present study, Budd-Chiari syndrome accounted for 10% of deaths. Hepatic vein thrombosis was diagnosed in 12 patients, of whom 4 (33.3%) died. Behçet’s syndrome is a common cause of Budd-Chiari syndrome. In a series of BD patients with large-vessel thrombosis (18), hepatic vein thrombosis was reported in 14 (26.4%) of 53 patients, and after a mean followup of 3 years, 10 (71.2%) of the patients died. In a study by Kural-Seyahi et al, among 42 patients with BD who died, Budd-Chiari syndrome was diagnosed in 3 patients and superior vena cava occlusion in 4 patients (10). In another study, involvement of the large vessels was found to be present in 15–35% of patients with BD (19). The prevalence of large vessel involvement, involving either the venous or arterial systems or both, in Turkish patients with BD was reported to be 49.2% in male patients and in 5.6% in female patients (10). All of the 2811 studies on BD confirm the predominance of vascular disease in male patients (13). Male sex was the main factor associated with mortality in our cohort of 817 patients with BD. Thirtyeight (92.7%) of the 41 patients with BD who died were male. In multivariate analyses, the likelihood of death was increased almost 5 times when male sex was added as a covariate. Compared with female patients, the proportion of arterial involvement was 3 times higher in male patients. Male sex and a younger age at onset have been previously reported to markedly influence disease expression and the course of BD (3,10,20). Male patients tended to have more flares of BD compared with female patients. We found an independent association between a high number of BD flares and mortality. Patients who experienced ⱖ5 flares of BD had a 2 times increased likelihood of dying. This has never been previously reported. In a recent study of BD patients with neurologic involvement, the number of attacks (⬎2) and relapse during steroid dose tapering was associated with a poor prognosis (11). A high number of BD flares resulted in exposure of patients to repeated treatment with corticosteroids and immunosuppressants, which might account for the deaths due to sepsis, drug toxicity, or neoplasia. Alternatively, persistent inflammation may lead to complications such as AA amyloidosis. Mortality, as measured by the SMR, was significantly higher in patients with BD as compared with age-and sex-matched healthy controls. The mortality rate was 3 times higher in BD patients younger than age 35 years as compared with healthy controls. In patients older than age 35 years, the mortality rate tended to reach that in healthy controls. Interestingly, previous studies have reported that the severity of this disease abates with time (3,10,20). Thus, the overall mortality in our cohort of patients with BD was 5% after a median followup of 7.7 years. The mortality rate at 1 year and 5 years was 1.2% and 3.3%, respectively. Male sex, arterial involvement, and a high number of flares were independently associated with mortality in BD. When compared with age-and sex-matched healthy controls, the mortality rate was 3 times higher in patients with BD younger than age 35 years. AUTHOR CONTRIBUTIONS All authors were involved in drafting the article or revising it critically for important intellectual content, and all authors approved the final version to be published. Dr. Saadoun had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. 2812 SAADOUN ET AL Study conception and design. Saadoun, Wechsler. Acquisition of data. Saadoun, Wechsler, Le Thi Huong, Amoura, Cacoub. Analysis and interpretation of data. Saadoun, Wechsler, Desseaux, Le Thi Huong, Amoura, Resche-Rigon, Cacoub. REFERENCES 1. Sakane T, Takeno M, Suzuki N, Inaba G. Behçet’s disease. N Engl J Med 1999;341:1284–91. 2. Yazici H, Yurdakul S, Hamuryudan V. Behçet disease. Curr Opin Rheumatol 2001;13:18–22. 3. Shimizu T, Ehrlich GE, Inaba G, Hayashi K. Behçet disease (Behçet syndrome). Semin Arthritis Rheum 1979;8:223–60. 4. Yurdakul S, Tuzuner N, Yurdakul I, Hamuryudan V, Yazici H. Gastrointestinal involvement in Behçet’s syndrome: a controlled study. Ann Rheum Dis 1996;55:208–10. 5. International Study Group for Behçet’s Disease. Criteria for diagnosis of Behçet’s disease. Lancet 1990;335:1078–80. 6. Liddell FD. Simple exact analysis of the standardised mortality ratio. J Epidemiol Community Health 1984;38:85–8. 7. Daly L. Simple SAS macros for the calculation of exact binomial and Poisson confidence limits. Comput Biol Med 1992;22:351–61. 8. Benamour S, Zeroual B, Bennis R, Amraoui A, Bettal S. Behçet’s disease: 316 cases. Presse Med 1990;19:1485–9. In French. 9. Yazici H, Basaran G, Hamuryudan V, Hizli N, Yurdakul S, Mat C, et al. The ten-year mortality in Behçet’s syndrome. Br J Rheumatol 1996;35:139–41. 10. Kural-Seyahi E, Fresko I, Seyahi N, Ozyazgan Y, Mat C, Hamuryudan V, et al. The long-term mortality and morbidity of Behçet syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine (Baltimore) 2003;82:60–76. 11. Akman-Demir G, Serdaroglu P, Tasci B, the Neuro-Behçet Study Group. Clinical patterns of neurological involvement in Behçet’s disease: evaluation of 200 patients. Brain 1999;122:2171–82. 12. Siva A, Kantarci OH, Saip S, Altintas A, Hamuryudan V, Islak C, et al. Behçet’s disease: diagnostic and prognostic aspects of neurological involvement. J Neurol 2001;248:95–103. 13. Calamia KT, Schirmer M, Melikoglu M. Major vessel involvement in Behçet disease. Curr Opin Rheumatol 2005;17:1–8. 14. Le Thi Huong D, Wechsler B, Papo T, Piette JC, Bletry O, Vitoux JM, et al. Arterial lesions in Behçet’s disease: a study in 25 patients. J Rheumatol 1995;22:2103–13. 15. Grenier P, Bletry O, Cornud F, Godeau P, Nahum H. Pulmonary involvement in Behcet disease. AJR Am J Roentgenol 1981;137: 565–9. 16. Hamuryudan V, Yurdakul S, Moral F, Numan F, Tuzun H, Tuzuner N, et al. Pulmonary arterial aneurysms in Behçet’s syndrome: a report of 24 cases. Br J Rheumatol 1994;33:48–51. 17. Hamuryudan V, Er T, Seyahi E, Akman C, Tuzun H, Fresko I, et al. Pulmonary artery aneurysms in Behçet syndrome. Am J Med 2004;117:867–70. 18. Bayraktar Y, Balkanci F, Bayraktar M, Calguneri M. Budd-Chiari syndrome: a common complication of Behçet’s disease. Am J Gastroenterol 1997;92:858–62. 19. Espinosa G, Font J, Tassies D, Vidaller A, Deulofeu R, LopezSoto A, et al. Vascular involvement in Behçet’s disease: relation with thrombophilic factors, coagulation activation, and thrombomodulin. Am J Med 2002;112:37–43. 20. Yazici H, Tuzun Y, Pazarli H, Yurdakul S, Ozyazgan Y, Ozdogan H, et al. Influence of age of onset and patient’s sex on the prevalence and severity of manifestations of Behçet’s syndrome. Ann Rheum Dis 1984;43:783–9. DOI 10.1002/art.27705 Erratum In the article by Emery et al in the August 2009 issue of Arthritis & Rheumatism (pages 2272–2283), there were several errors in the text and in Tables 1 and 2, resulting from an unintentional systematic programming error pertaining to CRP units used in the DAS28-CRP calculations. In Table 1, the mean ⫾ SD (median) DAS28 using CRP level in group 1 (placebo plus MTX), group 2 (golimumab 100 mg plus placebo), group 3 (golimumab 50 mg plus MTX), group 4 (golimumab 100 mg plus MTX), and groups 3 and 4 combined, respectively, should have been reported as 5.6 ⫾ 1.06 (5.6), 5.8 ⫾ 1.06 (5.8), 5.7 ⫾ 1.05 (5.6), 5.7 ⫾ 1.06 (5.7), and 5.7 ⫾ 1.05 (5.7). In Table 2, the number (%) of patients in group 1, group 2, group 3, group 4, and groups 3 and 4, respectively, experiencing good or moderate response according to the DAS28 using CRP level should have been reported as 97 (60.6), 105 (66.0), 119 (74.8) [P ⫽ 0.007], 120 (75.5) [P ⫽ 0.004], and 239 (75.2) [P ⫽ 0.001]; the number (%) of patients in group 1, group 2, group 3, group 4, and groups 3 and 4, respectively, experiencing remission according to the DAS28 using CRP level should have been reported as 30 (18.8), 27 (17.0), 41 (25.8) [P ⫽ 0.129], 44 (27.7) [P ⫽ 0.059], and 85 (26.7) [P ⫽ 0.054]. The second sentence under the heading “Other measures of arthritis” on page 2277 should have read “Statistical comparisons of the combined group versus group 1 were significant for these parameters, with the exception of DAS28 (using the CRP) remission, which approached significance (P ⫽ 0.054) (Table 2). The last sentence in the left column of page 2281 (continuing onto the right column) should have read “The benefit of golimumab plus MTX versus placebo plus MTX was also observed when evaluated by the ACR-N (only golimumab 50 mg plus MTX), the proportions of patients in both dose groups achieving a DAS28 response (using CRP or ESR) or remission (using ESR), and the ACR core components, with statistically significant improvements between golimumab plus MTX versus placebo plus MTX observed for the tender joint count (only 50 mg plus MTX), patient’s assessment of pain, patient’s assessment of disease activity, and the CRP level (both doses).” The errors did not affect the overall conclusions from the study. We regret the errors.