Treatment Approach for Coexisting Giant Cell Arteritis, Syrinx, and Transverse Myelitis
For patients with coexisting giant cell arteritis (GCA), syrinx, and transverse myelitis, high-dose glucocorticoids combined with tocilizumab should be initiated promptly, with consideration for IV pulse glucocorticoids if there are signs of threatened neurological function.
Initial Management of GCA with Neurological Complications
Immediate Treatment
- Initial therapy:
- High-dose oral glucocorticoids (1mg/kg/day, typically 40-60mg prednisone daily) 1
- Consider IV pulse glucocorticoids (typically methylprednisolone 500-1000mg daily for 3 days) if there are signs of threatened neurological function or rapid progression 1
- Add tocilizumab (162mg subcutaneously weekly) to the glucocorticoid regimen 1
Diagnostic Approach
- Temporal artery biopsy within 2 weeks of starting glucocorticoids 1, 2
- Long-segment biopsy (>1 cm) is preferred for better diagnostic yield
- Noninvasive vascular imaging to evaluate large vessel involvement 1
- MRI is particularly valuable for assessing both cranial and extracranial vessels 2
- High-resolution MRI of the spinal cord to evaluate the syrinx and transverse myelitis
Management of Neurological Complications
Transverse Myelitis Management
- IV pulse glucocorticoids are conditionally recommended for GCA with threatened neurological function 1, 3
- Monitor for clinical improvement of neurological symptoms
- Consider plasma exchange if no improvement with high-dose glucocorticoids
Syrinx Management
- Regular MRI monitoring of syrinx size and extension
- Neurosurgical consultation for potential surgical intervention if syrinx is expanding or causing progressive symptoms despite immunosuppressive therapy
Maintenance Therapy
Glucocorticoid Tapering
- Begin tapering glucocorticoids after 2-4 weeks of clinical improvement
- Slow taper schedule over 12-18 months while monitoring for disease activity
- Daily dosing is preferred over alternate-day scheduling 1
Immunosuppressive Therapy
- Continue tocilizumab for at least 12 months 1
- Consider adding aspirin if there is critical or flow-limiting involvement of vertebral or carotid arteries 1
- For patients who relapse while on moderate-to-high-dose glucocorticoids, add or switch to a non-glucocorticoid immunosuppressive agent 1
Monitoring and Follow-up
- Regular clinical monitoring is strongly recommended 1
- Monitor inflammatory markers (ESR, CRP) but do not escalate therapy based solely on elevated markers without clinical symptoms 1
- Regular MRI of the spine to assess syrinx and transverse myelitis
- Vascular imaging to monitor for disease progression in large vessels
Special Considerations
- Transverse myelitis in GCA is rare but has been reported 3 and requires aggressive treatment to prevent irreversible neurological damage
- The presence of a syrinx may complicate management and require multidisciplinary approach with neurosurgery
- Tocilizumab has shown superior efficacy in maintaining remission and reducing glucocorticoid requirements compared to glucocorticoids alone 1, 4
Common Pitfalls and Caveats
- Delaying treatment while awaiting biopsy results can lead to irreversible neurological damage
- Tapering glucocorticoids too quickly increases risk of relapse
- Monitoring disease activity can be challenging when using tocilizumab as it suppresses inflammatory markers
- Neurological symptoms may not respond as quickly as other GCA manifestations to treatment
- Failure to recognize that GCA can affect any artery in the body, including spinal arteries, potentially contributing to transverse myelitis 3