Review Question - QID 211201

The patient presents with clinical symptoms and imaging consistent with C2 pannus formation consistent with Rheumatoid Arthritis. The most likely laboratory finding would be an elevated anti-cyclic citrullinated protein (anti-CCP), which is the most specific and sensitive serum marker for rheumatoid arthritis.

Rheumatoid arthritis is an inflammatory disease that affects many large joints and results in progressive deformity and instability due to soft tissue laxity and inflammatory pannus formation. The involvement of the cervical spine can present in three patterns of instability which include atlantoaxial subluxation, basilar invagination, and subaxial subluxation. Common presenting symptoms include progressive myelopathy, axial neck pain, and occipital headaches. Rheumatoid patients with suspected cervical spine involvement should undergo a thorough radiographic assessment of the cervical spine.

Del Grande et al. reported two cases of rheumatoid cervical spondylitis and a literature review of the diagnosis and treatment of cervical spine disease in rheumatoid arthritis patients. They reported that cervical spine involvement is a late finding of rheumatoid arthritis usually occurring 10 years after diagnosis and is characterized by boney erosions and subluxations on radiographs. They concluded that rheumatoid patients with neck pain and myelopathy should undergo a thorough neurological evaluation and imaging.

Steinberger et al. analyzed the morbidity and mortality in adult patients undergoing transoral pannus resection. They found a 21.4% postoperative complications rate with 2.4% mortalities. They concluded the transoral approach carries significant risks of morbidity and mortality.

Chieng et al. performed a systematic review of 17 studies reporting on the treatment of rheumatoid cervical spondylitis of the craniovertebral junction. They reported higher neurologic improvement in patients treated with posterior based surgery than with combined anterior-posterior procedures. The authors concluded that the choice for the surgical approach is based on the reducibility of the subluxation in traction but ultimately should be based on the individual patient's pathology.

Figure A is the sagittal CT scan of the craniovertebral junction with erosive changes of the odontoid and a large mass effect on the spinal cord at the from an inflammatory pannus. Figure B is the sagittal T2 MRI of the cervical spine with narrowing of the canal at the craniovertebral junction due to a large inflammatory pannus. Illustration A is a treatment algorithm for the treatment of rheumatoid cervical spondylitis.

Incorrect Answers:
Answer 2: While an elevated CRP and WBC count are nonspecific laboratory finding and may be elevated in RA, anti-CCP is a much more sensitive and specific marker for RA.
Answer 3: HLA-B27 is associated with ankylosing spondylitis which manifests as a bamboo spine rather than pannus formation at the dens.
Answer 4: An M spike of serum protein gel electrophoresis is diagnostic of multiple myeloma which is characterized by multiple lytic lesions throughout the axial and appendicular skeleton. It does not result in pannus formation and spinal cord compression.
Answer 5: EXT gene mutation is associated with multiple hereditary exostoses and is characterized by large osteochondromas that form at the physis of long bones.