Crowned dens syndrome, yet another rheumatic disease imposter

Area of Science:

• Rheumatology
• Orthopedics
• Inflammatory Diseases

Background:

• Crowned dens syndrome (CDS) involves inflammation around the odontoid process, typically associated with calcium pyrophosphate dehydrate (CPPD) arthropathy.
• Acute cervical or occipital pain and stiffness are characteristic symptoms of CDS.

Purpose of the Study:

• To investigate the clinical presentation and management of Crowned dens syndrome (CDS) in a diverse patient cohort.
• To broaden the understanding of conditions associated with CDS beyond CPPD arthropathy.

Main Methods:

• Prospective identification of 24 inpatients with CDS over two years.
• Clinical assessment, inflammatory marker evaluation, and review of associated rheumatic diseases.
• Treatment with glucocorticoids and colchicine, with outcomes assessed for clinical and laboratory measures.

Main Results:

• Patients (54-87 years, 67% female) presented with acute neck pain/stiffness and elevated inflammatory markers.
• While 71% had known rheumatic diseases (including CPPD, ankylosing spondylitis, rheumatoid arthritis), CDS also presented as an initial symptom of CPPD in some.
• All patients showed dramatic improvement with glucocorticoids and colchicine, with pain alleviated and mobility regained.

Conclusions:

• Crowned dens syndrome (CDS) should be considered in acute cervical pain with stiffness and inflammation, even outside of known CPPD arthropathy.
• Imaging the craniocervical junction is crucial for diagnosing CDS in various clinical settings.
• Early recognition of CDS can lead to prompt treatment and potentially avoid unnecessary invasive diagnostic procedures.