INTRODUCTION

Giant Cell Arteritis (GCA) is a chronic non-granulomatous vasculitis most commonly affecting medium and large sized arteries, with headache as its predominant symptom.¹ It affects 0.2% of adults >50 years of age.² Conversely, migraines are estimated to occur in 12% of the general population.³ Given this disparity in incidence and a shared presentation of a headache, patients with GCA may be misdiagnosed with having a migraine attack. This case report elucidates the presentation, differentials, workup, and lessons learned from a patient with a history of migraines who developed GCA.

CASE

A 74 y/o Caucasian female with a history of migraines for almost 40 years, cataracts, anxiety, and depression initially presented to her outpatient clinic for a worsening headache that was qualitatively different from her previous migraines. Of note, the patient had recently fallen two months prior. Given the change in the character of the headache and the recent fall, she was referred to the emergency department for further evaluation.

At the ED, she reported that this new type of headache has lasted a week and was initially intermittent but became constant in the last three days before her presentation. She described this headache as sharp and moderate in severity. Since her fall two months prior, the patient also noticed that her temporal arteries have gradually become more prominent and tender to palpation. Review of systems is positive for myalgias, fatigue, and nausea and was negative for fever, cough, chest pain, shortness of breath, visual changes, difficulty with speech, weakness, facial droop, and photosensitivity. Of note, the patient was recently diagnosed with acute cystitis with hematuria and was on trimethoprim/sulfamethoxazole and promethazine. Surgical history was noncontributory. Family history included a diagnosis of heart failure in her father. There was no family history of headaches, other neurologic or rheumatologic disorders. The patient denied the use of tobacco, ethanol, or illicit drugs. On exam, the patient was oriented to person, place, and time. She had tenderness over the bilateral temporal arteries and posterior scalp. There was no lymphadenopathy, erythema, warmth, or swelling noted in the temporal regions. No visual changes were observed. Pupils were equal, round, and reactive to light. Cranial nerves, motor, sensory, and coordination were all intact. The patient had no dysarthria or aphasia. Imaging was unremarkable for any acute intracranial process, and the patient was discharged to follow up at outpatient.

She was then seen at the neurology clinic for further evaluation. History revealed daily use of ibuprofen, aspirin/acetaminophen/caffeine, and zolmitriptan with relief. However, this new headache was not relieved by her usual therapy. Physical exam and review of systems were unchanged from the ED visit.

Differentials at that point include medication overuse headache (MOH) and temporal arteritis. Additional workup of CBC, CMP, ESR, and CRP was ordered. ESR was elevated at 69 mm/Hr (normal range 0-30 mm/hr). She was sent for a biopsy of her bilateral temporal arteries. Pathology report revealed transmural lymphohistiocytic infiltrate with abundant giant cells, consistent with temporal arteritis.

DISCUSSION

Giant Cell Arteritis (GCA) is the most common of all the vasculitides, affecting medium and large-sized arteries. Headache is the most common symptom occurring in 90% of patients with GCA¹.  It is often described as generalized, throbbing, with a predilection to occur in the temporal area and less likely, the occipital region.⁴  Complications from GCA include permanent vision loss, stenosis, aortic aneurysms, and rarely, stroke, tongue and scalp necrosis.⁵  Abnormal laboratory values often include anemia, leukocytosis, thrombocytosis, and elevated liver function tests.⁴ Additionally, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) are diagnostic indicators for GCA with a limited sensitivity of 86.9% and 84.1% respectively; however, combined, specificity can be as high as 97%.⁶  Cases of patients having biopsy-proven GCA with only mild elevations to normal values in their ESR^7,8 have also been reported. Corticosteroids remain the treatment of choice for GCA. If given promptly can result in in complete resolution of symptoms and prevention of vision loss.

The headaches experienced in migraines and GCA can share a similar clinical description. This similarity can cause a delay in diagnosis with up to 7.7 weeks¹⁰ and cause permanent vision loss in 15-25% of cases.¹¹  Fast-tracking diagnostic pathways can be facilitated by having a high index of suspicion in patients > 50 years of age who present with new-onset headaches in the temporal region with associated visual disturbances.

Our patient had maximal medical therapy and multiple confounding etiologies for her headache. Her history of migraines, and medication overuse can present as moderate to severe headaches. Choice of treatment can also mask the correct diagnosis in this case. Short-course corticosteroid therapy is an effective abortive treatment for migraines; however, it is also the mainstay medication for GCA. Sumatriptan has also been reported to improve GCA.¹²  Ruling out the most serious conditions associated with headaches requires the clinician to be aware of the “red flags” that we diligently ask our patients. Utilizing available screening tools, such as SNOOP (Systemic symptoms, Neurologic symptoms, Onset, Older, Previous headache), are helpful and efficient.⁹

CONCLUSION

Individuals with a known diagnosis of chronic headaches, as in our patient with chronic migraine and medication overuse headache (MOH) for almost 4 decades, can easily be masked with a different etiology of headache. We want to emphasize that a change in character and region of headache should prompt the physician to further investigate, as previous headaches can steer away from a new and worrisome diagnosis. CBC, ESR, CRP, and in acute settings, CT head, are an appropriate initial work-up. Elderly patients with chronic comorbidities would greatly benefit from a more comprehensive workup as rare diseases like GCA are time-sensitive and if not acted on promptly, can cause debilitation and a poor quality of life.

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