Giant Cell Arteritis
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Title: Giant Cell Arteritis
Author: Michael Jensen, MSIV
Keywords/Main Subjects: Giant Cell Arteritis, AAION
Diagnosis: Giant Cell Arteritis
Description of Case: An 80-year-old male recently diagnosed with polymyalgia rheumatica presented to his primary ophthalmologist with complaints of 1 month of intermittent diplopia, greying of vision, severe bitemporal headache, scalp tenderness, malaise, and night sweats but denied jaw claudication. Eye exam was at baseline upon initial presentation with BCVA of 20/40 OD and 20/50 OS, no diplopia, and normal fundus exam. History and exam were most consistent a diagnosis of giant cell arteritis and the patient was prescribed 80mg oral prednisone. ESR, CRP, CBC were ordered and the patient was referred to an oculoplastic surgeon for a temporal artery biopsy the next morning. Overnight, patient experienced a sudden decrease in vision after taking his prednisone. On exam BCVA is now CF in both eyes and labs show elevation of ESR, CRP, and platelets. Retinal exam was significant for 1+ edema and 4+ pallor of the optic nerve. The patient was admitted to the hospital and started on 1000mg/day IV steroid treatment as well as adjuvant therapy with heparin, pentoxifylline, and brimonidine. Despite three days of maximal therapy, the patients vision declined to NLP OU.
Giant cell arteritis is a large-medium vessel vasculitis strongly associated with polymyalgia rheumatica. It primarily effects adults age 70 years and above and often presents with a temporal headache, scalp tenderness, jaw claudication, and vision changes. Other symptoms include throat, ear, or tongue pain, constitutional symptoms including fever, malaise, night sweats, fatigue, and proximal muscle soreness and weakness. Work up includes sensitive markers of inflammation like ESR, CRP, and platelets. Temporal artery biopsy is the gold standard for diagnosis but temporal artery ultrasound can also be performed by an experienced practitioner. Treatment includes immediate administration of steroids once the diagnosis is suspected, without waiting for biopsy results. Neuro-Ophthalmology and Rheumatology should be consulted in the in-patient setting. There is no strong evidence in favor of IV or oral steroids but common practice to treat with 1000 mg IV steroids for three days if there are accompanying vision changes. Regardless of route, early treatment has the largest impact on vision outcomes and delays in treatment can lead to permanent vision loss, like the patient described above. Adjuvant therapies like heparin are based on limited evidence and their use should be decided on a case-by-case basis as part of a multi-disciplinary team.
Summary of the Case: Classic presentation of giant cell arteritis is and older adult with history of PMR, temporal headache, vision changes, claudication within the distribution of the carotid arteries, and constitutional symptoms. Standard workup includes inflammatory markers and temporal artery biopsy. Steroids should be started immediately if there is a strong clinical suspicion. If exam is positive for visual changes, the patient should be admitted for IV steroids along with emergent neuro-ophthalmology and rheumatology consultation.
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References:
- Salvarani, C., Pipitone, N., Versari, A., & Hunder, G. G. (2012). Clinical features of polymyalgia rheumatica and giant cell arteritis. Nature Reviews.Rheumatology, 8(9), 509-521. https://doi.org/10.1038/nrrheum.2012.97
- Gonzalez-Gay, M. A. , Barros, S. , Lopez-Diaz, M. J. , Garcia-Porrua, C. , Sanchez-Andrade, A. & Llorca, J. (2005). Giant Cell Arteritis. Medicine, 84 (5), 269-276. doi: 10.1097/01.md.0000180042.42156.d1.
- Hayreh SS, Zimmerman B. Management of giant cell arteritis. Our 27-year clinical study: new light on old controversies. Ophthalmologica. 2003 Jul-Aug;217(4):239-59. doi: 10.1159/000070631. PMID: 12792130
- Buono LM, Foroozan R, de Virgiliis M, Savino PJ. Heparin therapy in giant cell arteritis. Br J Ophthalmol. 2004 Feb;88(2):298-301. doi: 10.1136/bjo.2003.021592. PMID: 14736795; PMCID: PMC1772017
Faculty Approval by: Griffin Jardine, MD
Copyright statement: Copyright Michael Jensen, ©2024. For further information regarding the rights to this collection, please visit: http://morancore.utah.edu/terms-of-use/
