The treatment of giant cell arteritis

Although giant cell arteritis (GCA) is a well-known vasculitis sensitive to corticosteroid-mediated immunosuppression, numerous issues of long-term therapeutic management remain unresolved. Because GCA encompasses a broad spectrum of clinical subtypes, ranging from devastating visual loss and neurological deficits to isolated systemic symptoms, the treatment of GCA must be adjusted to each case, and recommendations vary widely in the literature. This article systematically reviews the treatment options for patients with neuro-ophthalmic and neurological complications of GCA, as well as the evidence for possible adjuvant therapies for patients with GCA. Although there is no randomized controlled clinical trial specifically evaluating GCA patients with ocular and neurological complications, we recommend that GCA patients with acute visual loss or brain ischemia be admitted to the hospital for high-dose intravenous methyl-prednisolone, close monitoring, and prevention of steroid-induced complications. Aspirin may also be helpful in these cases. The evidence supporting the use of steroid-sparing immunomodulatory agents such as methotrexate for long-term management remains debated.     

Intracranial giant cell arteritis

Summary A case of giant cell arteritis of intracranial vessels diagnosed by autopsy is described. Giant cell arteritis of the proximal basal brain arteries was exceptionally marked in a man of 60 years. The clinical course, laboratory findings and the pathological alterations of the brain and intracranial blood vessels are described. The case is discussed on the basis of the literature on giant cell arteritis with cerebral symptoms as well as on granulomatous giant cells arteritis of the brain. A separation of these two entities does not seem justified.

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Zusammenfassung Es wird ein Fall von Riesenzellarteriitis der intrakraniellen Gefäße beschrieben, welcher bei der Autopsie diagnostiziert wurde. Bei diesem 60jährigen Mann war der histologische Befund der Riesenzellarteriitis in den proximalen Abschnitten der Gefäße der Gehirnbasis ungewöhnlich ausgeprägt. Es werden der klinische Verlauf, die Laboratoriumsbefunde und die histologischen Veränderungen des Gehirnes und der basalen Hirngefäße beschrieben. Der Fall wird anhand der Literatur über Riesenzellarteriitis mit zerebralen Symptomen diskutiert unter Einbezug der Fälle über granulomatöse Riesenzellarteriitis des Gehirnes. Eine Trennung dieser beiden Krankheitsbilder erscheint den Autoren nicht berechtigt.

     

Neurologic manifestations of giant cell arteritis

Journal of Neurology - Giant cell arteritis, the most frequent form of vasculitis in persons over 50 years of age, is a granulomatous chronic vasculitis involving large and medium-sized...     

Internal carotid stenosis and giant cell arteritis

We report on the case of a 69-year-old man admitted with a transient ischemic attack preceded by a two months history of severe headache. Giant cell arteritis was diagnosed by means of temporal artery biopsy. Angiography showed an intra- and extracranial stenosis of the left internal carotid artery. The possible relationship between this stenosis and vasculitis is discussed and stroke as a clinical manifestation of the giant cell arteritis is reviewed.     

Siegrist streaks in giant cell arteritis.

A patient who presented with symptoms of giant cell arteritis was found to have a right ophthalmic artery occlusion. One month after initial evaluation, the peripheral retina demonstrated multiple linear bands of chorioretinal atrophy known as Siegrist streaks. Although most commonly described in the setting of acute hypertension, Siegrist streaks also occur in patients with giant cell arteritis.     

Deterioration of giant cell arteritis with corticosteroid therapy

BACKGROUND: Failure of response of giant cell arteritis (GCA) to corticosteroid therapy has invariably been attributed to the delay in diagnosing the disease or the use of inadequate corticosteroid dosage. Following our observation of progressive deterioration following the introduction of prednisolone use in a patient, we examined the possibility that worsening of the condition might be due to corticosteroid therapy rather than coincidence. OBJECTIVE: To determine whether corticosteroid therapy may exacerbate GCA. DESLGN: Case report and an analysis of similar cases reported in the medical literature. PATIENT: A 64-year-old man had a 3-month history of headache, night sweats, malaise and general weakness, and anorexia and weight loss and a more recent history of jaw claudication, dysphagia, and hoarseness. Clinical findings included prominent temporal arteries with absent pulsation, abnormal saccades to the right, and eyelid retraction. Laboratory findings included an elevated erythrocyte sedimentation rate and platelet count. Results of a biopsy of the temporal artery confirmed GCA. Magnetic resonance imaging scans showed ischemic cerebellar lesions and a mature infarct in the left anterior occipital, posteroparietal region. Following corticosteroid therapy commencement, the patient's condition deteriorated steadily for 5 days with clinical signs suggestive of an evolving vertebrobasilar stroke. Following treatment with high-dose intravenous dexamethasone sodium phosphate and heparin sodium, his symptoms improved. DATA SOURCES: The review included analysis of autopsy-based reports in which clinical details are provided and clinical reports in which major visual or cerebral complications are described. Significant complications occurred in many cases shortly following the introduction of corticosteroid therapy. In many of these cases, the symptoms indicated that GCA had been present for a significant period prior to corticosteroid therapy. CONCLUSIONS: Progressively evolving occlusive strokes may occur following corticosteroid therapy in patients with GCA. In cerebrovascular complications, vascular occlusion occurs at sites of active vasculitis, usually within the vertebrobasilar system. It is not certain that the worsening of the condition following corticosteroid therapy is always coincidental, and an alternative possibility, namely a functional relationship between the initiation of corticosteroid therapy and clinical deterioration, should be borne in mind.     

Recurrent brachial plexus neuropathy and giant cell arteritis

A 73-year-old women presented with a recurrent form of sporadic brachial plexus neuropathy, the so-called Parsonage and Turner syndrome. This diagnosis is based on clinical and electromyographic findings. Interestingly a biopsy of the temporal artery demonstrated a giant cell arteritis. The clinical picture started 2 weeks after an upper respiratory tract illness. The possible viral etiology of giant cell arteritis is considered. We think an immunological rather than ischemic disturbance may have caused the recurrent brachial plexus neuropathy. This case report suggests that giant cell arteritis be considered in the investigation of the Parsonage and Turner syndrome.     

Ophthalmic artery microembolism in giant cell arteritis.

A 70-year-old man presented with a history of headache and sudden loss of vision of the left eye. Funduscopic examination showed sector retinal edema and hemorrhage as well as optic disc swelling consistent with anterior ischemic optic neuropathy. The Westergren sedimentation rate was 66 mm/h. Temporal artery biopsy was consistent with giant cell arteritis. Routine transcranial Doppler testing performed on a Pioneer 2020 instrument (Nicolet Vascular, Inc., Golden, CO) equipped with special software for microembolus detection showed a microembolic signal in the left ophthalmic artery. During a subsequent monitoring study, microembolic signals were detected in the anterior and middle cerebral arteries, bilaterally. Microembolism can occur in giant cell arteritis. Ophthalmic artery microembolism can be detected in vivo by transcranial Doppler ultrasonography. This new imaging capability can potentially be useful when evaluating patients with vascular disorders of the eye.     

Headache in giant cell arteritis and other arteritides

Neurological Sciences - Giant cell arteritis remains the most common systemic vasculitis in patients over the age of 50. Headache is the most common symptom, but is not invariably present. The...     

Biopsy negative giant cell arteritis–Revised diagnostic criteria: Giant cell arteritis diagnostic criteria

BackgroundDiagnosis of giant cell arteritis has traditionally relied on demonstration of pathologic changes on temporal artery biopsy.MethodTo highlight recent advances in large vessel imaging resulting in revised diagnostic criteria for giant cell arteritis.ConclusionWe call attention to the revised diagnostic criteria imaging evidence of extracranial large vessel thickening as an alternative to temporal artery biopsy in diagnosis of giant cell arteritis in a patient with heralding anterior fornix infarct.