Session Information
Date: Monday, October 22, 2018
Title: Vasculitis Poster II: Behҫet’s Disease and IgG4-Related Disease
Session Type: ACR Poster Session B
Session Time: 9:00AM-11:00AM
Background/Purpose: There is some evidence that incident Behcet’s syndrome (BS) might be becoming less severe (1, 2). We compared clinical findings at presentation of BS patients registered in a large, long standing dedicated multidisciplinary outpatient clinic at 4 time points during a 40-years period.
Methods: There were 4 groups. Group 1 included patients registered in 1979- 1981, Group 2 those registered in 1990, Group 3 in 2000 and Group 4 in 2010. Only demographic and clinical findings at initial presentation were recorded on prepared forms.
Results: As shown in Table 1, over 4 decades, male/female ratio decreases gradually. While mean age at presentation does not change, the median disease duration got shorter. Almost all clinical manifestations except genital ulcers and neurological involvement tended to decrease in frequency. This was also true when genders were separately analyzed. Importantly the severity of vascular and eye disease decreased (Table 2). The slope of vascular disease was more obvious.
Conclusion: Our observations support the notion that incident BS might be getting milder. There might be a list of explanations for this observation. 1. It might be a true biological phenomenon due to changing environmental causes. In this line the significant decrease in papulopustular lesions could be due to a more sanitary environment while the rather unchanging frequency of neurologic involvement might be its possible independence from the environment. 2. It might be that the awareness of BS is increasing and we are recognizing less severe cases earlier. 3. Another explanation might be the more effective treatment these patients received before they were referred which was not specifically sought in this survey.
References:
1. Yoshida A, et al. Comparison of patients with Behçet’s disease in the 1980s and 1990s. Ophthalmology. 2004.
2. Cingu AK, et al. Comparison of presenting features and three-year disease course in Turkish patients with Behçet uveitis who presented in the early 1990s and the early 2000s. Ocul Immunol Inflamm. 2012.
Table 1. Initial demographic and clinical characteristics of cohorts
|
Group 1 1979-81 cohort n=211 |
Group 2 1990 cohort n=170 |
Group 3 2000 cohort n=225 |
Group 4 2010 cohort n= 270 |
P
|
|
|
Male /Female |
140/71 |
110/ 60 |
142/ 83 |
150/ 120 |
0.069 |
|
Male/Female ratio |
1.97 |
1.83 |
1.71 |
1.25 |
|
|
Mean age at disease onset |
31.5 ± 8.3 |
30.9 ± 9.0 |
30.7± 9.3 |
32.3 ± 9.6 |
NS |
|
Median disease duration |
2.5 [1.0-6.0] |
2.0 [1.0-5.0] |
1 [0.5-3] |
1 [0.6-4] |
0.06 |
|
Mucocutaneous inv. , n (%) |
|||||
|
Genital ulceration |
187 (88.6) |
137 (80.6) |
184 (81.7) |
220 (81.4) |
NS |
|
Papulopustular lesion |
174 (82.5) |
130 (76.5) |
187 (83) |
185 (68.5) |
<0.001 |
|
Erythema nodosum |
132 (62.6) |
88 (51.8) |
101 (44.8) |
112 (41.4) |
<0.001 |
|
Ocular involvement, n (%) |
107 (50.7) |
107 (62.4) |
97 (43.1) |
129 (47.7) |
0.001 |
|
Vascular involvement, n (%) |
49 (23.2) |
29 (17.0) |
41 (18.2) |
31 (11) |
0.008 |
|
Large vessel involvement, n (%) |
21 (42.9) |
11 (38.0) |
13 (31.7) |
7 (22.5) |
<0.001 |
|
Neurologic involvement, n (%) |
7 (3.3) |
6 (3.5) |
5 (2.2) |
10 (3.7) |
NS |
|
Arthritis, n (%) |
79 (37.4) |
37 (21.8) |
53 (23.6) |
56 (20.7) |
<0.001 |
Table 2. Severity of ocular involvement at presentation
|
Ocular involvement |
1979-81 cohort n =107 |
1990 cohort n=106 |
2000 cohort n=97 |
2010 cohort n= 129 |
p
|
|
Visual acuity < 0.1 in bilateral eyes, n (%) |
17 (15.7) |
8 (7.5) |
6 (6.5) |
9 (6.9) |
0.03*
|
|
Visual acuity < 0.1 in unilateral eye, n (%) |
23 (21.3) |
19 (17.9) |
18 (19.7) |
22 (17) |
0.39** |
|
Visual acuity > 0.5 in bilateral eyes |
39 (36.1) |
54(55.6) |
55 (60.4) |
73 (56.6) |
0.002**
|
*Cohort 1 vs 4 and Cohort 1 vs 3; **Cohort 1 vs 4
To cite this abstract in AMA style:
Dincses E, Ozguler Y, Ucar D, Ozyazgan Y, Ugurlu S, Hatemi G, Melikoglu M, Yurdakul S, Yazici H, Seyahi E. Retrospective Analysis of Initial Presentation Findings of Behcet’s Syndrome throughout 4 Decades [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/retrospective-analysis-of-initial-presentation-findings-of-behcets-syndrome-throughout-4-decades/. Accessed .« Back to 2018 ACR/ARHP Annual Meeting
ACR Meeting Abstracts - https://acrabstracts.org/abstract/retrospective-analysis-of-initial-presentation-findings-of-behcets-syndrome-throughout-4-decades/