Background/Purpose
Behcet’s Syndrome (BS) is a systemic vasculitis which may involve multiple organ systems simultaneously. Most frequently, clinical findings in BS fit into well recognized patterns such as the association between papulopustular skin lesions and arthritis. Neurological involvement in BS is a serious condition which could lead to significant disability. It could either be parenchymal or vascular. The pathogenesis of these two types of Neuro-Behcet’s Syndrome (NBS) are suggested to be different. The purpose of this study is to evaluate the association between the parenchymal Neuro-Behcet’s Syndrome and panuveitis.
Methods
We retrospectively reviewed the clinical records of 288 patients with BS, who met the international classification criteria for BS, diagnosed at two major rheumatology clinics from 2000 to 2014. Patient demographics, ophthalmic examinations, clinical and radiologic patterns of neurological involvement were recorded. Pearson’s Chi-square test was used for analysis.
Results
In this cohort of a total of 288 patients, 93 developed panuveitis and 38 had NBS (28 men and 10 women, median age 33 (28-54)). Of the 38 patients with neurological involvement, 28 had parenchymal and 10 had vascular disease. Venous sinus thrombosis was the only vascular involvement in NBS patients. Those with panuveitis were significantly more likely to have parenchymal neurological involvement compared to those without panuveitis (22.6% vs 3.6 %, p =0.001) (Table). 21 of the 28 parenchymal NBS patients had panuveitis either prior to or during the course of neurological involvement. Panuveitis was significantly associated with parenchymal-NBS (OR 7.83 95% CI 3.19-19.21).
Table. The association between panuveitis and parenchymal neurological involvement in patients with BS.
|
BS Patients |
|
Parenchymal neurological involvement |
P-value |
|
|
|
Present |
Absent |
||
|
Panuveitis |
Present |
21 |
72 |
0.001 |
|
Absent |
7 |
188 |
||
Conclusion
Among our 288 patients with BS, over one out of five cases with panuveitis had neurological involvement during their disease course. Our findings suggest a significant association between these two major organ involvements. This association might also be defined as a recognized clinical pattern, similar to the association of papulopustular skin lesions with arthritis. BS patients with panuveitis should be educated about possible signs and symptoms of neurological involvement which could progress quite rapidly and early initiation of treatment is the key for success.
Disclosure:
B. Bitik,
None;
B. Goker,
None;
K. Sahin,
None;
Y. S. Karadag,
None;
R. Mercan,
None;
A. Tufan,
None;
M. A. Ozturk,
None;
F. Ak,
None;
Y. Karaaslan,
None;
S. Haznedaroglu,
None.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/increased-risk-of-parenchymal-neurological-involvement-in-behcets-syndrome-patients-with-panuveitis/