Arthritis & Rheumatism, Volume 63,
November 2011 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Chicago, Illinois November 4-9, 2011.

A Case Series of HLA B27 Patients with a Behet's Like Syndrome in a UK Population.

Ambrose1,  Nicola, Haskard2,  Dorian O.

Imperial College London, London, United Kingdom
Imperial College, London, United Kingdom


This review focuses on patients referred to a UK Behçet's Syndrome (BS) Specialist Clinic who were found to be HLA B27 positive.


Patient demographics, symptoms, signs, treatments and HLA markers were recorded for the 598 patients seen in clinic from 1999 to 2011. Updates to the datasheet were made at subsequent visits.


597 patients were seen between 1999 and 2011. Of these, 319 were classified as having definite BS (DBS), 184 of having possible BS, 59 were definitely not BS and 36 were found to be HLA B27 positive and were classified as B27 BS-like syndrome (B27 group). We compared the DBS (ie B27 negative) group with the B27 group. Age and male sex were comparable (median age 36 and 38, male sex 44% and 58%, respectively). In the DBS group, 97% fulfilled ISG criteria, versus 69% of the B27 Group (p<0.001). Oral ulcers were found >99% of the DBS group versus 81% of the B27 group (p=<0.001). Genital ulcers were found in 89% of the DBS group versus 58% of the B27 group (p<0.001). Fewer patients in the HLA B27 group had the combination of oral and genital ulceration (B27 58% versus BS 89%, p<0.001). Similar numbers in both groups had inflammatory eye lesions (BS 46%, B27 56%, (p=0.22). Similar numbers also had skin lesions (BS 80%, B27 70%, p=0.23), Only a minority in either group had a positive pathergy test (BS 12%, B27 11%, p=0.9). While some of this B27 group present with features clinically identical to DBS, most had features that led us to suspect their B27 positivity. Thus, anterior uveitis was the predominant ocular manifestation in B27 group (85% of all), whereas posterior or panuveitis is more typical of DBS. Only 9% of our non-B27 DBS group had a history of joint swelling at any stage in their illness versus 61% of the B27 group (p=0.001). The latter patients also had findings which are not usual features of DBS such as psoriasis, enthesitis or plantar fasciitis. Interestingly, neurological and vascular BS manifestations were equally common in the two groups, with neurological involvement in 12% of the DBS group and in 22% of the B27 group (p=0.06). Neurological involvement in the B27 group included meningoencephalitis, trigeminal neuralgia, hemiparesis, intracranial hypertension and seizures. Secondly, vascular involvement was found in 18% of DBS and 25% of B27 groups respectively (p=0.23). In the B27 group, findings included deep vein thrombosis, subclavian vein thrombosis, superficial thrombophlebitis, dural sinus thrombosis, pulmonary embolism, development of an ischaemic digit and a radial artery aneurysm.


Of the 598 patients seen in this clinic, 36 have been found to be HLA B27 positive. While some of this cohort present with features identical to a classical BS, most have features that led us to clinically suspect their B27 positivity. This group may represent an extreme end of the spondyloarthropathy spectrum of disease. Importantly, they experience serious neurological and vascular BS-like manifestations to a similar extent as their classic BS counterparts.

To cite this abstract, please use the following information:
Ambrose, Nicola, Haskard, Dorian O.; A Case Series of HLA B27 Patients with a Behet's Like Syndrome in a UK Population. [abstract]. Arthritis Rheum 2011;63 Suppl 10 :1038

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