The overlap syndrome is frequently encountered in rheumatology, but the coexistence of Behçet's disease and ankylosing spondylitis is considered to be rare. We present a case of this interesting coexistence in a 49-year-old male with positivity for HLA-B51 and negativity for HLA-B27.
Keywords: Ankylosing spondylitis, Behçet's disease, HLA-B27, HLA-B51, sacroiliitis
|How to cite this URL:|
Mounir A, Driouach S, El Khader S, Zinebi A, Moudden MK. Behcet's disease and ankylosing spondylitis: A rare association. Indian J Med Spec [Epub ahead of print] [cited 2019 Aug 19]. Available from: http://www.ijms.in/preprintarticle.asp?id=259812
| Introduction|| |
Behçet's disease (BD) is a multisystemic vasculitis, characterized by recurrent oral aphthae and other systemic manifestations. Ankylosing spondylitis (AS) is a chronic systemic inflammatory disorder of the enthesis and paravertebral structures. The coexistence of BD and AS has been rarely reported. On one hand, several authors suggested that BD falls into spondyloarthritides (SpA) group because of the presence of sacroiliitis in BD. On the other hand, some studies did not support this hypothesis based on the genetic characteristics of the two diseases concerning the HLA-B27 antigen for AS and HLA-B51 for BD. The coexistence of these two pathologies in the same person could be hazardous. The present case is a patient who fulfilled both the criteria for BD and AS.
| Case Report|| |
A 49-year-old patient, diabetic, follow-up case of BD evolving for 24 years with recurrent bipolar aphthae and positivity of HLA-B51, put under colchicine 1 mg/day with a good improvement. For 6 years, the patient had inflammatory dorsolumbalgia and fessalgia. On physical examination, his cervical and dorsal back motions were painful and limited, and the chest expansion was +2 cm. His Schober's index was 10 + 4 cm. The sacroiliac joints were painful to passive mobilization. Ophthalmologic examination was normal. There was no history of any rheumatic disease in his family. Furthermore, he did not describe any conjunctivitis, urethritis, psoriasis, severe diarrhea attacks, cardiovascular, or neurological symptoms. Routine laboratory tests were normal. HLA-B27 was negative. Erythrocyte sedimentation rate was 80 mm/h, and C-reactive protein value was 21.2 mg/L. Pelvis radiography revealed bilateral Grade 3 sacroiliitis [Figure 1]. Cervical spine X-rays showed flattening of the cervical lordosis [Figure 2]. A syndesmophyte bridging was observed on his thoracic spine radiographs [Figure 3]. Considering all the findings, the diagnosis of AS was made according to Assessment of Spondyloarthritis International Society (ASAS) criteria associated with BD. The BASDAI score of activity was 2.1. The patient was subjected to treatment with diclofenac 150 mg/day and rehabilitation program with a good evolution.
|Figure 1: Anteroposterior pelvis radiography showing bilateral Stage III sacroiliitis according to the classical New York criteria|
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| Discussion|| |
In 1974, Moll et al. included BD in the SpA. Since then, there have been many debates about the existence of a relationship between BD and SpA. However, it is known that BD has clinical features in common with SpA such as asymmetrical peripheral oligoarthritis, an involvement of distal interphalangeal joints, sacroiliac and spinal joints, intestinal and ocular inflammation, and the genomic features which share three risk loci with AS and psoriasis.,, The AS-associated uveitis follows a benign course and involves the anterior uveal track. However, the uveitis in BD involves both the posterior and the anterior uveal tracks, which causes the loss of sight in 25 of 100 patients. Furthermore, BD shares two loci with inflammatory bowel disease (IL23R and IL10) involving shared pathogenic pathways in BD and SpA. There has been recently a study which involved 27 countries, and the prevalence of AS in BD reported is 1.9%. Then again, Kotevoglu concluded that sacroiliitis in BD is not more frequent than normal population. There remains insufficient evidence that suggest sacroiliitis is an intrinsic feature of BD. In BD patients with arthritis, Lehner described an increased incidence of HLA-B27. On the other hand, no association with this antigen was reported in BD. According to a Korean series, the frequency of HLA-B51 in the BD group was significantly higher than in the SpA group. Due to the fact that BD seems to be associated with HLA-B51 rather than HLA-B27, this finding strongly opposes the assumption that BD is part of the SpA group. Further studies are needed to identify the genetic variants – other than HLA-B27 and HLA-B51 – in patients with BD and AS coexistence, and the shared remaining environmental factors contribute to both disease triggering.
| Conclusion|| |
Although BD has common clinical and genetic features with SpA, our case suggests that BD be considered a differential diagnosis of AS. Further investigations are needed to understand the etiopathogenesis of this coexistence, especially as the number of cases increases.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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Department of Internal Medicine, Military Hospital Moulay Ismail, Meknes
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2], [Figure 3]