FRI0230 Crowned dens syndrome, yet another rheumatic disease imposter

• Published in: Annals of the rheumatic diseases Vol. 77; no. Suppl 2; p. 656
• Main Authors: Awisat, A., Rozenbaum, M., Rimar, D., Boulman, N., Kaly, L., Slobodin, G., Jiries, N., Hussien, H., Rosner, I.
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group LTD 01.06.2018
• Subjects: Ankylosing spondylitis; Arteritis; Behcet's syndrome; Biopsy; C-reactive protein; Calcium; Colchicine; Computed tomography; Diagnosis; Erythrocyte sedimentation rate; Familial Mediterranean fever; Fever; Glucocorticoids; Headache; Headaches; Inflammation; Laboratories; Ligaments; Neck; Neck pain; Pain; Patients; Prednisone; Rheumatic diseases; Rheumatoid arthritis; Spine (cervical); Spondylitis
• ISSN: 0003-4967; 1468-2060
• DOI: 10.1136/annrheumdis-2018-eular.3544

BackgroundCrowned Dens Syndrome (CDS) is defined as acute cervical or occipital pain, usually associated with elevated acute phase reactants, due to local inflammatory reaction related to calcifications in the ligaments surrounding the odontoid process. Virtually all previous descriptions of CDS have been related to calcium pyrophosphate dehydrate (CPPD) arthropathy.ObjectivesTo evaluate patients admitted with acute neck pain and/or headacke for CDS not only in those with CPPD arthropathy but also in patients with other rheumatic diseasesMethodsTwenty four cases of CDS treated in Zion Medical Centre in 2016–2017 were prospectively reviewed. Patients were evaluated according to clinical and laboratory features, background rheumatic disease, response to treatment and invase investigational procedures undertaken.ResultsAll patients (age range 54 to 87 years, 67% females) presented with acute onset pain in upper neck and/or occiput accompanied with extreme neck stiffness. Nineteen of 24 patients (79%) had elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Five patients (21%) complained on concomitant severe diffuse headache. Four of those underwent temporal artery biopsy, which was negative for arteritis in all cases, and one was subjected to lumbar puncture, which was non-contributory.The diagnosis of CDS was based on computed tomography imaging in all patients, with precipitations of calcium pyrophosphate dehydrate seen most often in transverse ligament (figure 1) or in alar ligaments.Seventeen patients (71%) had known rheumatic disease on presentation: 10 patients had the diagnosis of CPPD arthropathy, 3 patients had ankylosing spondylitis, 2 patients had rheumatoid arthritis, 1 patient had Behcet’s disease and 1 suffered from Familial Mediterranean Fever (table 1). In 4 more patients CDS was the presenting symptom of CPPD disease, diagnosed during hospitalisation.All patients were treated with glucocorticoids as 0.5 mg/kg prednisone plus colchicine 0.5 mg bid resulting in dramatic improvement in both clinical (head/neck pain alleviated and cervical spinal mobility regained) and laboratory measures.Abstract FRI0230 – Table 1Clinical and laboratory feature of 24 patients with crowned dens Age, median(range)71(54–87 )Female (ratio)16/24 (66%)Presenting symptoms :Neck painHeadache FeverNo. (%)24 (100%)5 (20.6%) 1 (4%)Antecedent rheumatologic diagnosis:Calcium pyrophosphate dehydrateAnkylosing spondylitisRheumatoid arthritisBechet’s diseaseFamilial Mediterranean FeverNo. (%)14 (58%)3 (12.5%)2 (8.3%)1 (4%)1 (4%)Elevated CRP/ESR, (mean)19 (60 mg/l)Patient underwent temporal artery biopsy4 (16.6%)ConclusionsCDS should be considered and craniocervical junction exposed in the context of acute cervical or occipital pain with stiffness and elevated inflammation markers not only in patients previously diagnosed with CPPD, but rather in diverse clinical settings. Particularly, CDS should be recognised as a possible alternative diagnosis in older patients referred with suspicion to giant cell arteritis because of new headache and elevated ESR/CRP. While generally believed to be a rare phenomenon, CDS was seen in 24 patients in 400-bed general hospital within 2 years and is probably widely underdiagnosed.Disclosure of InterestNone declared