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Giant cell arteritis (GCA) is a potentially fatal medium- to large-vessel vasculitis with ocular and systemic involvement. The most common ocular manifestation is anterior arteritic ischemic optic neuropathy (AAION), which can be blinding and may affect 1 or both eyes. Systemically, aortitis is the most serious potential complication of GCA, as it can lead to aortic dissection and aortic aneurysms.
Coronary arteritis has classically been associated with polyarteritis nodosa, Kawasaki disease, and Takayasu’s arteritis, but it can also be seen in the antineutrophil cytoplasm antibody–associated vasculitides such as granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis. Only few reports have been documented, however, in relation to GCA. Most of these cases were determined in the context of fatal myocardial infarctions (MIs), in which autopsy revealed pathologic findings of coronary GCA.
The following appears to be one of the first cases of a patient with temporal artery biopsy-proven GCA with ocular manifestations, who subsequently developed an MI, with evidence of coronary arteritis on autopsy.
A 76-year-old female with an ocular history of glaucoma and pseudophakia OU presented to the eye clinic with a superior visual field defect in her right eye. She reported a history of GCA 2 years prior, for which she had been treated with prednisone. GCA review of systems at this visit was significant for right temporal pain, scalp tenderness, jaw pain, and 5-pound weight loss.
Medical history was significant for diabetes mellitus, hypertension, hypercholesterolemia, cholecystectomy, and a remote history of breast cancer treated with mastectomy and chemotherapy approximately 30 years prior. She was an ex-smoker with a 25 pack-year history.
Vision was 20/40-1 OD and 20/30-2 OS. Intraocular pressures (IOPs) were 7 and 14 mm Hg. She had a grade 4 relative afferent pupillary defect of the right eye. Posterior segment examination revealed florid disc edema of the right optic nerve. Erythrocyte sedimentation rate was 63 mm/hour, C-reactive protein was 14.2 mg/L, and platelets were 401 × 109/L.
She was started on 80mg of oral prednisone for presumed AAION secondary to GCA, and temporal artery biopsy confirmed the diagnosis. Three days after presentation, vision in the right eye markedly declined to hand motions. She was started on intravenous (IV) methylprednisolone 250 mg every 6 hours. Examinations over the next several days revealed a cherry red spot consistent with central retinal artery occlusion secondary to GCA. The right eye became hypotonous, with an IOP of 3 mm Hg. The patient finished her course of IV methylprednisolone and was restarted on oral prednisone.
Three weeks after initial presentation, she was experiencing significant side effects from the steroids, including vomiting, diarrhea, and weakness. Her eye remained hypotonous, and she was now having right periorbital pain, which was attributed to likely ischemia of the ophthalmic artery.
The patient was admitted to hospital on the following day because increasing weakness, anorexia, and weight loss. The next morning, she died of cardiorespiratory arrest, shortly after developing epigastric pain and nausea. Her most recent electrocardiogram, performed 7 years earlier, revealed a septal infarct of undetermined age.
Postmortem autopsy revealed inflammatory infiltrate within the external elastic laminar and medial layers of the coronary arteries (Fig. 1). The internal elastic lamina was thinned and fragmented (Fig. 2). No giant cells were seen, but in the context of the positive temporal artery biopsy, the specimen was identified as evidence of unequivocal active arteritis.
In addition to aortitis, a general increased cardiovascular risk has been associated in patients with GCA.
A multitude of factors likely contributes to the increased cardiovascular mortality in patients with GCA. The presence of chronic inflammatory changes may compound pre-existing atherosclerosis, and steroid treatment itself may play a role.
This case report supports the existing hypothesis that coronary arteritis is a true entity that can cause mortality in GCA. Although atherosclerotic disease is still likely the most common cause of MI in patients with GCA, it is possible that a significant number of GCA-related MIs are actually secondary to coronary arteritis but simply have not been identified because of lack of autopsy.
A large retrospective study spanning 6 decades recently evaluated the risk for acute coronary syndrome (ACS) in patients with GCA compared with a cohort of patients without GCA.
The results suggested that there was no overall increased risk for ACS in patients with GCA and furthermore showed that certain cardiovascular risk factors were actually less frequent in patients with GCA.
However, this study did not include cases of sudden death as part of its documented ACS events; as such, the analysis may have inadvertently excluded patients with GCA who did indeed suffer MIs, similar to the patient in the present case. Moreover, even if ACS incidence is similar between the 2 groups, it does not mitigate the fact that coronary arteritis is a real disease entity with associated morbidity and mortality.
Although there is robust evidence of an association between GCA and aortic aneurysms, the incidence of aortic aneurysms is so low that screening with aortic computed tomography scans is not currently recommended.
Thus, it is unlikely that screening protocols for the much less recognized entity of coronary arteritis in patients with GCA will be implemented imminently. However, clinical inquiry into signs and symptoms of aortic arch aneurysms, such as upper limb claudication and asymmetrical pulses, can be assessed easily in clinic. Similarly, symptoms of angina suggestive of possible coronary arteritis can simply be screened for in patients with GCA. As further studies seek to establish screening guidelines, it is important to consider coronary arteritis among other causes of GCA-related cardiovascular morbidity.
Aortic involvement in recent-onset giant cell (temporal) arteritis: a case-control prospective study using helical aortic computed tomodensitometric scan.