Predictors of recurrent ischemic optic neuropathy in giant cell arteritis.

BACKGROUND: The competing interests of preventing recurrent ischemic optic neuropathy (ION) and minimizing medication side effects make corticosteroid dose reduction in giant cell arteritis (GCA) a difficult problem. The authors sought to determine whether any factors were predictive of recurrent ION. METHODS: Retrospective review of the records of 100 consecutive patients with biopsy-proven giant cell arteritis diagnosed in two Australian hospitals between 1988 and 1998. Among 67 patients who met inclusion criteria for ION in GCA, seven patients had recurrent ION. We compared the seven patients with recurrent ION to the 60 patients with nonrecurrent ION in terms of age, gender, mode of corticosteroid delivery, initial visual acuity in the affected eye, prevalence of bilateral ION, initial erythrocyte sedimentation rate (ESR) level, and rate of corticosteroid dose reduction. In the recurrent ION group, we documented the timing of the recurrence in relation to corticosteroid dose, elevation in acute phase reactants, and relapse of systemic symptoms. RESULTS: We found recurrent ION in GCA in 10% of our cohort, higher than has been previously reported. Recurrences, all of which were ipsilateral, occurred from 3 to 36 months (median 8 months) after the initial ION. None of the clinical indicators the authors examined differed between the two groups. Six of seven patients with recurrent ION had elevations in ESR or C-reactive protein or a new headache at the time of ION recurrence, but in only one of these patients were these features recognized as preceding the recurrent ION. One patient had neither an elevation in acute phase reactants nor a relapse in systemic symptoms of GCA at the time of ION recurrence. CONCLUSIONS: Recurrent ION in GCA is difficult to predict. Although elevated acute phase reactants or new systemic symptoms consistent with GCA were present in six (83%) of our patients with ION recurrence, in only one patient (17%) did these events occur with enough lead time to allow caregivers to act preemptively. Thus, even very close monitoring of GCA patients with ION may not predict ION recurrence.     

Giant cell arteritis (temporal arteritis): A report of four cases from north east India

Giant cell arteritis (GCA) is a common disease of the geriatric age group in the western world, with a prevalence of 0.2% in the fifty plus age group. It is an important cause of morbidity, with irreversible visual loss being the most ominous complication. This diagnosis is an important consideration in all cases of new onset headache in elderly subjects. Reports of giant cell arteritis are few and far between in the Indian subcontinent. In this report, we describe the clinical details of four cases of giant cell arteritis, detected at Guwahati, Assam. The four patients were in the 70-82 age group. Sex distribution was equal. All of them had polymyalgia rheumatica (PMR), with one case displaying an initial presentation as only PMR. Cardinal manifestation was a severe headache, frequently accompanied by scalp allodynia and abnormalities of the superficial temporal artery (STA) on examination. STA biopsy yielded histopathological confirmation in three patients. Permanent visual loss was noted in one patient. These cases highlight the importance of assessing the possibility of giant cell arteritis through appropriate clinical history, estimation of acute phase reactants and the judicious use of superficial temporal artery biopsy, to clinch the diagnosis.     

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.     

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.     

Giant cell (temporal) arteritis: a treatable cause of multi-infarct dementia

Dementia occurs infrequently in patients with giant cell (temporal) arteritis (GCA). Three elderly women with biopsy-proven GCA showed abrupt cognitive decline during periods of clinically active GCA, 1 to 6 months after diagnostic temporal artery biopsy, during periods of corticosteroid taper. One patient had additional clinical signs of cerebral infarction and other ischemic phenomena. Reinstitution of higher oral doses of corticosteroids successfully prevented further cognitive losses and permitted gradual but incomplete improvement of cognitive function in 1 patient. Neuropsychologic data from 2 patients 7 to 10 months after temporal artery biopsy suggested multifocal cognitive impairment, and the 3rd patient appeared clinically to be globally, severely demented. Neuroimaging studies revealed multiple areas of infarction, predominantly in the posterior circulation territory. One patient had bilateral vertebral artery occlusions (digital subtraction angiography) and bilaterally reduced carotid system perfusion pressures (oculoplethysmography). There were no associated cardiovascular risk factors or family history of dementia in these patients.     

Recent Advances in Diagnostic Strategies for Giant Cell Arteritis

Giant cell arteritis (GCA) is a systemic vasculitis that affects the aorta and its major branches. Involvement of the ciliary artery can result in ischemic optic neuropathy and subsequent blindness, which is typically irreversible. If GCA is suspected, treatment with glucocorticoids should be initiated promptly to prevent further vision loss. However, given the need for prolonged therapy with glucocorticoids and the morbidity associated with their use, diagnosis should be confirmed. Clinical features and laboratory findings are neither sensitive nor specific for GCA. The mainstay of diagnosis remains histopathologic examination of a section of the superficial temporal artery. Several imaging studies have been used to evaluate the temporal artery but, at present, their utility as alternatives to a temporal artery biopsy is limited. Recent advances in imaging modalities have allowed detailed noninvasive imaging of the large arteries and are a useful adjunct for the diagnosis of GCA, particularly in patients with primarily large-vessel involvement in whom temporal artery biopsy is often negative.     

The diagnosis of giant cell arteritis

Giant cell arteritis (GCA) is the most common primary vasculitis in adults older than 50 years. The potential of GCA to cause bilateral, sequential vision loss makes it often a true neuro-ophthalmic emergency. Approximately one fifth of patients with GCA will present with ophthalmic complaints alone. The diagnosis of GCA requires a high index of suspicion and a systematic approach to diagnostic testing. The combination of abnormal laboratory markers of systemic inflammation and unilateral temporal artery biopsy is usually diagnostic. Additional testing with other diagnostic modalities may be required in cases in which clinical suspicion remains high despite a negative initial workup. We systematically review the diagnostic modalities used in suspected GCA patients who present with neuro-ophthalmic symptoms and signs.     

Giant cell arteritis of the cervical radicular vessels presenting with diaphragmatic weakness

The clinical and histopathological details of a patient who succumbed to giant cell arteritis (GCA) of the cervical radicular vessels are described. The initial clinical presentation, with diaphragmatic weakness, has not previously been reported. Normal inflammatory indices and the unusual presentation prevented diagnosis during life, but GCA should be considered in the differential diagnosis of any unexplained neuropathic or radiculopathic syndrome, as corticosteroid therapy may lead to recovery. This is the first account of the pathological findings in cervical radiculopathy associated with GCA.     

Visual function and quality of life among patients with giant cell (temporal) arteritis.

OBJECTIVE: To investigate patient perception of visual and systemic disability associated with giant cell arteritis (GCA) and whether the perceived disability can be correlated with visual performance measures. METHODS: We prospectively evaluated and compared the visual performance and quality of life survey for 20 patients with GCA after 4 to 5 weeks of corticosteroid therapy and after one year of therapy. We measured visual acuity, contrast sensitivity, and threshold perimetry and patients completed the Activities of Daily Vision Scale (ADVS) and the short-form of the Health Survey (SF-36). The results were grouped by GCA affected or unaffected eye or by better or worse eye and reported as a decimal and percent impairment for acuity, log units for contrast, mean deviation and the Advanced Glaucoma Intervention Study (AGIS) score for perimetry. The results for patients with and without visual loss were compared. Correlation analyses between ADVS categories and visual performance measures, SF-36 categories and the presence of visual loss, total corticosteroid dose, systemic symptoms, secondary hypertension or diabetes mellitus, the presence of vertebral fracture, and visual performance were performed. RESULTS: Day driving was the only ADVS category significantly reduced at baseline in patients with visual loss (62.5) compared with those without visual loss (96.3, P = 0.04). Modest to moderate correlations between ADVS categories were most frequent for percent binocular acuity impairment with day driving (r = -0.62, P = 0.017), with distance vision (r = -0.5, P = 0.02), and with glare (r = -0.59, P = 0.006); and the AGIS score of the worse eye with day driving (r = -0.66, P = 0.01), with near vision (r = -0.49, P = 0.03), and with glare (r = -0.48, P = 0.04). The baseline SF-36 scores did not correlate with the presence of vision loss at baseline or systemic complications. The ADVS and SF-36 scores were similar at one year. The total dose of corticosteroids only had a modest correlation with the one-year mental health score (r = -0.45, P = 0.05), but there was no correlation between SF-36 scores and other systemic side effects of steroid therapy. CONCLUSION: Except for the day driving score, the ADVS did not differ between patients with and without visual loss. The SF-36 did not distinguish between patients with and without visual loss and did not reveal significant trends. The ADVS and SF-36 did not reveal significant disability in GCA patients and there were no strong correlations with any visual performance or systemic measures.     

Giant cell arteritis

Giant cell arteritis (GCA) is an immune-mediated vasculitis affecting individuals over 50 years of age. It is characterised by granulomatous inflammation that affects medium-sized and large arteries. The wide spectrum of clinical manifestations can be divided into those related to tissue ischemia from vascular lesions and those related to a systemic inflammatory response. The pathogenesis of these groups also appears distinct, with vascular lesion formation thought to be an adaptive immune response, and the systemic inflammatory reaction an innate immune response. Clinical suspicion of GCA must remain especially high in those with neurological or visual symptoms and if warranted, prompt treatment with high-dose corticosteroids is invaluable in halting disease progression.     

Peripheral neuropathic syndromes in giant cell (temporal) arteritis

Of 166 consecutive patients with histologically confirmed giant cell (temporal) arteritis (GCA) seen during a 3-year period, 23 (14%) had clinically diagnosed peripheral neuropathic syndromes temporally coincident with clinically active GCA. Electromyography and nerve conduction studies were performed in 16, confirming abnormalities in all. Of the 23 patients, 11 had a generalized peripheral neuropathy, nine had multiple mononeuropathies, and three had a mononeuropathy. The nerves affected as mononeuropathies were the median, ulnar, peroneal, tibial, and sural nerves, and the C-5 and L-5 nerve roots. Angiography, performed in two patients, demonstrated widespread arteritis involving the lower limbs and, after 3 months of oral corticosteroid treatment in one of these patients, an amputation specimen showed chronic arteritis.     

The management of acute visual failure.

This review of acute visual failure covers the clinical manifestations and management of ocular strokes CRA occlusion, BRA occlusion and AION. The diagnostic process for each patient requires meticulous attention to: 1. Blood pressure, heart rate and rhythm, palpation of the temporal arteries, and auscultation of the heart, neck, eyes and head. 2. Dilated funduscopic examination. 3. Immediate blood tests: complete blood count, PT, PTT, platelet count, ESR, fibrinogen level, fasting blood sugar, cholesterol, triglyceride and blood lipids. A test for antiphospholipid antibodies (ACLA and LA) is recommended in unexplained cases of CRA occlusion. Non-invasive investigations should utilise a battery of tests: 1. Carotid non-invasive studies; the useful tests give information about the presence of a haemodynamic lesion (Dopper ultrasonography and oculoplethysmography), analyse the bruit to determine the residual lumen diameter (phonoangiography), or image the artery with ultrasound (B-Scan ultrasonography). 2. Two-dimensional echocardiogram Invasive investigations are required in selected patients: 1. A temporal artery biopsy 2. A carotid arteriogram if the patient is a candidate for endarterectomy. The patient can be screened first with a non-invasive MRA of the neck and brain. 3. A timed FFA, particularly in cases of CRA occlusion when occlusion of the ophthalmic artery is suspected, in cases of AION of possible embolic origin or in AION to document the position of the watershed zone of the choroidal circulation and its relation to the optic nerve head. Emergency treatment in CRA occlusion is designed to lower intra-ocular pressure and dislodge the embolus. In impending CRA occlusion heparin is useful. Urgent systemic corticosteroids are needed when CRA occlusion, or AION are due to arteritis. In other situations treatment is directed towards preventing recurrence or involvement of the other eye by reducing or eliminating identified risk factors.     

Risk factors for early visual deterioration in temporal arteritis

Background

Despite corticosteroid treatment, patients with temporal arteritis may continue to lose vision. However, predictors of progressive visual loss are not known.

Methods

We retrospectively reviewed 341 consecutive patients with suspected temporal arteritis who underwent temporal artery biopsy. 90 patients with biopsy proven temporal arteritis were included in our study.

Results

Twenty‐one patients (23%) experienced continuous visual symptoms despite steroid therapy and 14 among these suffered persistent visual deterioration. Based on univariate analysis, visual loss on presentation was associated with disc swelling and a history of hypertension. Risk factors for progressive visual loss included older age, elevated C reactive protein and disc swelling.

Conclusion

Although corticosteroid therapy improves the visual prognosis in temporal arteritis, steroids may not stop the progression of visual loss. Our study reliably establishes the risk factors for visual loss in this serious condition. Whether addressing these risk factors early in their presentation can alter the visual outcome remains unknown. Individual risk anticipating treatment regimens and strategies might improve the visual prognosis in temporal arteritis in the future.Left untreated, temporal arteritis (TA) frequently results in blindness. Treatment requires immediate high dose steroids. Risk factors for initial and progressive visual loss, despite appropriate treatment, have not been extensively characterised, making selection of patients at risk for progressive visual loss difficult.We retrospectively reviewed clinical findings in patients with biopsy proven TA in order to assess risk factors for visual loss during the first days of steroid therapy.     

巨细胞动脉炎:颞动脉活检光镜研究

目的 探讨中国人巨细胞动脉炎(GCA)颞动脉活检的病理学特征和意义。方法 诊断为GCA的患者20例，非GCA患者7例为对照；以临床症状、颞动脉活检、类固醇激素治疗及随访结果作为诊断标准。结果 20例患者被诊为GCA，其中16例符合美国风湿病学会(ACR)的GCA诊断标准，18例显示活跃期血管炎，14例显示跳跃性损害，灵敏度、特异度和阳性预告值分别为90．0％、83．3％～94．8％。结论 提示颞动脉活检对GCA的诊断和治疗都具有重要意义。     

An Unusual Presentation of Rheumatoid Meningitis

BACKGROUND: Central nervous system involvement in rheumatoid arthritis can rarely occur in the absence of systemic disease. Rheumatoid meningitis has not been reported to present as spells of neurologic dysfunction. PATIENT AND METHODS: The authors describe a woman with a history of well-controlled rheumatoid arthritis who presented with headaches and spells of focal neurological dysfunction. Brain magnetic resonance imaging, brain biopsy, and temporal artery biopsy were required to make the diagnosis of rheumatoid meningitis with arteritis. RESULTS: Neuroimaging revealed abnormal leptomeningeal enhancement. Necrotizing granulomatous inflammation was seen on meningeal and brain biopsy. A temporal artery biopsy showed evidence of arteritis without giant cells. CONCLUSIONS: The possibility of central nervous system involvement by rheumatoid arthritis should be considered in patients with a history of rheumatoid arthritis even in the absence of systemic symptoms. Making the diagnosis may require meningeal and brain biopsy. The condition may be steroid responsive.     

The involvement of the peripheral nervous system in biopsy proven active giant cell arteritis

Abstract Background Peripheral nervous system (PNS) affection is an uncommon, sometimes life-threatening manifestation of giant cell arteritis (GCA). Objective To describe characteristics of neurological abnormalities of the PNS in GCA patients. Methods Eighty consecutive cases of biopsy proven GCA were studied. Results Three patients presented with subacute sensorimotor deficits abnormalities in the distribution of the arm plexus. In all cases PNS affection was the leading clinical symptom in addition to a typical clinical syndrome of cranial arteriitis. In one case MRI demonstrated diffuse signal abnormalities surrounding the brachial nerve plexus. In another patient, who died from pulmonary embolism 10 weeks after beginning of therapy, autopsy demonstrated residual arteritis in an artery supplying the brachial nerve plexus. Conclusions Involvement of the PNS is more uncommon than cerebral ischemia and neuroophthalmological complications in patients suffering from GCA. Severe PNS involvement has an affinity to the midcervical nerve roots and the brachial nerve plexus.     

Radiosensitive orbital inflammation associated with temporal arteritis.

A 75-year-old woman developed acute loss of vision in the OD, ipsilateral periocular pain, an afferent pupillary defect, sectoral optic disc edema, and later ipsilateral proptosis and an intraconal mass. She denied any symptoms of temporal arteritis, and a sedimentation rate was normal. Orbital biopsy demonstrated chronic granulomatous inflammation with perivasculitis. A temporal artery biopsy disclosed findings consistent with temporal arteritis. Following 2000 cGy of external beam radiation, her visual function and orbitopathy completely resolved. This unusual presentation of orbital inflammation in association with temporal arteritis demonstrates that pathologic findings of temporal arteritis may be clinically nonspecific and that external beam radiation may be an effective therapy in this setting.     

Giant cell arteritis as a cause of first-ever stroke

The aims of this study were to assess how frequently giant cell arteritis (GCA) was a cause of first-ever stroke in 4,086 patients in the Lausanne Stroke Registry and to determine the risk factors, patterns, latency and current therapy at onset in patients with GCA plus stroke. GCA was recognized using the criteria of the American College of Rheumatology. We report on 6 patients (0.15%) with a histologically proven diagnosis of temporal arteritis and clinical and neuroradiological evidence of cerebral ischemia. The CT and MRI scans showed lacunar infarction in 3 patients, territorial infarction in 2 and were normal in 1. Stroke latency ranged from 0 to 2 months. All patients suffered from headache. We conclude that stroke is a rare, but dangerous, complication of GCA and that a combination of antiplatelet drugs and corticosteroids may be advisable for preventing stroke occurrence.     

Pathologic findings in a steroid-responsive optic nerve infarct in giant-cell arteritis.

OBJECTIVE: To investigate the pathophysiologic mechanism of optic nerve infarction in giant-cell arteritis (GCA). BACKGROUND: Previous pathologic reports of optic nerve infarction in GCA involved patients who were blind at the time of death. The optic nerve infarcts were primarily retrolaminar in localization. Simultaneous short ciliary and ophthalmic artery vasculitis was found in all patients. METHODS: Clinical neurologic and ophthalmologic examination, temporal artery biopsy, and neuroimaging tests were performed in a patient with an anterior ischemic optic neuropathy secondary to GCA. Pathologic examination of the viscera, eye, and brain were performed at autopsy 1 month later. RESULTS: A prelaminar/retrolaminar infarct was found in this patient. Subsiding vasculitis was limited to the short ciliary arteries, sparing the central retinal, pial, and ophthalmic arteries. CONCLUSIONS: The authors believe that the visual improvement observed in this patient was the result of preserved, anterior optic nerve collateral circulation, as well as the neuroprotective and anti-inflammatory effect of the corticosteroids.