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Mortality in Beh┬зet's disease.

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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: david.saadoun@psl.aphp.fr.
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.
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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.
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