Low diagnostic yield with second biopsies in suspected giant cell arteritis.

OBJECTIVES: The clinical diagnosis of giant cell arteritis may be confirmed with a biopsy of the superficial temporal artery. Because of "skip lesions," a histologic diagnosis of giant cell arteritis may be missed with a unilateral biopsy. The authors report a study that investigates whether a biopsy of the contralateral superficial temporal artery provides any additional information for confirmation of a diagnosis of giant cell arteritis. METHODS: Available medical records of 91 consecutive patients who underwent bilateral superficial temporal artery biopsy procedures were reviewed. Information that was abstracted included sequence of biopsy procedures, length specimens, and histologic diagnosis. Microslides from all biopsy specimens were retrieved and reexamined in a masked fashion by the ocular pathologist (RCE) who had made the original diagnoses. RESULTS: Seventy-two bilateral simultaneous superficial temporal artery biopsies and 19 bilateral sequential biopsies were performed. The mean length of biopsy specimens was 23 mm, and the mean length of the total artery removed from each patient was 33 mm. The pathologist's original diagnosis and the diagnosis at reexamination were in 100% agreement. In 90 (99%) of the 91 patients, the histologic diagnoses in the left and right superficial temporal arteries were the same. This is a concordance rate of 98.9% (38 of 39 positive biopsy results) among the positive biopsy results. CONCLUSION: There is a low yield of information from a second temporal artery biopsy in patients with suspected giant cell arteritis. This suggests that patients who present to the ophthalmologist with possible giant cell arteritis will, in most cases, have a similar diagnosis on both temporal artery biopsies if the specimens are adequate.     

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.     

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.     

Giant cell arteritis of the occipital arteries

CONTEXT: Occipital headache and nuchal pain may indicate the involvement of the occipital arteries (OCCA) in temporal arteritis (TA). Recently high resolution color coded sonography (CCDS) has greatly improved the imaging of small lumen arteries. OBJECTIVE: The aim of the present study was the demonstration of TA, of the OCCA in comparison with the superficial temporal artery (STA) by means of CCDS in patients with nuchal and occipital pain suspected of suffering from TA. DESIGN: Prospective study of 78 patients comparing CCDS findings of the OCCA and of the STA with the clinical diagnosis and the biopsy results. RESULTS: 27 patients received the diagnosis TA; there were 51 other diagnoses. CCDS of the OCCA reached a sensitivity in diagnosis of 63 % and a specificity of 100 % and in histology of 65 % and 100% respectively. CCDS of the STA reached a diagnostic sensitivity of 78 % and a specificity of 94% and of a histological sensitivity of 77 % and specificity of 82 %. Reversibility of CCDS abnormalities was monitored in 5 patients over a period of 13 to 42 days. CONCLUSIONS: Involvement of the OCCA in TA patients is a frequent finding and may be the only pathological phenomenon in some patients with nuchal pain, occipital headache and occipital scalp tenderness. CCDS of the STA and OCCA contributed to the diagnosis of TA with a high rate of perivascular hypoechogenic abnormalities (stenoses and occlusions) and a low rate of these abnormalities in the control patients. However, CCDS cannot differentiate between inflammatory and degenerative artery disease and has limitations concerning spatial resolution. Before CCDS may replace biopsy in clinical practice the accuracy of the criteria recommended above should be tested in larger groups of patients.     

Brain vascular disease presenting as first manifestation of temporal arteritis: report of two cases]

Brain vascular disease as the first presentation of temporal arteritis is unusual. We present two cases in which the diagnosis emerged from the anamnesis. A 54 years old woman has had a left cerebral infarct 3 months ago. She was getting better when severe visual loss occurred and the family decided to get a second opinion. The patient have had right hemiparesis, aphasia and a left visual disturbance in the first episode; now she had severe bilateral visual disturbance. We suspected temporal arteritis was the etiology. HSR was 97 mm and fundoscopy disclosed severe ischemic optic neuritis. A 75 years old man presented Wallenberg syndrome. The history disclosed temporal headache and the examination showed inflammation in temporal artery. HSR was 70 mm and biopsy confirmed the diagnosis. "Tecnolatry" is affecting medical practice; it's necessary to put back in the center the clinical sovereignty.     

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.     

The significance of color duplex ultrasonography for the diagnosis of temporal arteritis]

We examined the usefulness of color duplex ultrasonography in patients suspected of having temporal arteritis. Five patients, who were all aged 70 or older, developed a new onset of localized headache with temporal artery abnormalities, and had an elevated erythrocyte sedimentation rate of > 100 mm/hour. The final diagnoses were temporal arteritis in three patients, polymyalgia rheumatica in one, and probable healed temporal arteritis in one. Color duplex ultrasonography showed stenoses, which were confirmed histologically as well, in the superficial temporal artery of all patients. The characteristic findings of active temporal arteritis were, however, demonstrated in only three biopsy specimens, and in the remaining two the stenoses were thought to be related to previous arteritis. The hypoechoic halo, which has been reported to be a characteristic finding of color duplex ultrasonography in active temporal arteritis, was detected in only one patient with active temporal arteritis and another one with probable healed temporal arteritis. No stenoses were demonstrated in the superficial temporal arteries of 30 control subjects (20 with at least one risk factor of atherosclerosis and 10 without it). Color duplex ultrasonography can therefore be considered a powerful method for detecting stenoses in the superficial temporal artery. Its ability to identify their etiology is, however, unsatisfactory, so that temporal artery biopsy remains undoubtedly the most reliable test for etiological evaluation. We thus recommend color duplex ultrasonography as a supplementary method for the diagnosis of temporal arteritis, because it can provide useful information concerning the appropriate site of temporal artery biopsy.     

Thrombocytosis in temporal arteritis rising platelet counts: a red flag for giant cell arteritis.

OBJECTIVES: To determine whether early recognition and detection of thrombocytosis in patients with giant cell arteritis can help secure an earlier diagnosis, and whether it can help differentiate cases of arteritic optic neuropathy from other forms of optic neuropathy. METHODS: Medical and ophthalmologic records from 1993 to 1998 of the authors' patients with biopsy-proven temporal arteritis versus the authors' patients with nonarteritic anterior ischemic optic neuropathy and idiopathic demyelinating optic neuritis were prospectively collected. Past and present blood analyses were collected, and platelet counts were compared between patients with giant cell arteritis and control populations. This was done to determine whether thrombocytosis could help with the diagnosis and differentiation of these different disease states. RESULTS: There was a significant difference in the frequency of thrombocytosis in patients with giant cell arteritis (13 out of 19 patients), with or without arteritic ischemic optic neuropathy, as compared with those with nonarteritic anterior ischemic optic neuropathy (zero out of 30 patients), idiopathic optic neuritis (zero out of 26 patients), and healthy age-matched controls (one out of 22 control subjects). This difference was especially helpful in patients whose sedimentation rates were within the normal range (adjusting for age). Also noted was the finding that the rise in the platelet counts was not acute, but rather it was a slow gradual increase for approximately 12 months before the onset of significant systemic or visual symptoms. CONCLUSION: Thrombocytosis should be considered an important marker in patients being referred for evaluation of ischemic optic neuropathy, diplopia, amaurosis fugax, headache, or even generalized malaise. Westegren sedimentation rates <50 mm/hr are often erroneously viewed as nondiagnostic or equivocal in the elderly and just followed. An over-the-phone review of patients' sedimentation rates, complete blood counts, and platelet counts can lead to expedited evaluation and treatment of patients who may be at high risk of visual loss from temporal arteritis. Thrombocytosis should lower a physician's threshold to acutely treat patients for possible arteritic ischemic optic neuropathy until the disease is definitely ruled out.     

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。     

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.     

Temporal arteritis presenting as an extrapyramidal disorder

A case of temporal arteritis presenting with extrapyramidal symptoms (tremor, rigidity and extrapyramidal hypertonus) unresponsive to conventional treatment is here described. The onset of headache and laboratory abnormalities suggestive of temporal arteritis prompted a temporal artery biopsy which confirmed the diagnosis; the administration of corticosteroids led to the resolution of all symptoms.     

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.     

拟诊为颞动脉炎的纤维肌性发育不良3例分析并文献回顾

目的 探讨纤维肌性发育不良的特点,提高神经内科医生对纤维肌性发育不良的认识。方法 收集3例被拟诊为颞动脉炎而行颞动脉活检并经病理诊断为纤维肌性发育不良的病例,复习并分析国内外相关病例报道。结果 3例均表现为头痛,其中2例仅累及颞动脉,另外1例合并双侧股浅动脉病变; 病理检查均表现为内膜纤维组织病变。结论 纤维肌性发育不良是一种血管病,几乎可累及全身血管床,可以“头痛、高血压病、腹痛、癫痫、神经功能缺损等”为首发症状; 神经内科医师应警惕此病,正确诊断及治疗。     

Late ipsilateral recurrence of ischemic optic neuropathy in giant cell arteritis.

A patient with arteriosclerosis, diabetes mellitus, and giant cell arteritis (GCA) treated continuously with low-dose prednisone developed anterior ischemic optic neuropathy (AION) at 5 and 13 months after clinical diagnosis of GCA. At the time of late recurrent AION, there were no systemic symptoms or elevations in acute phase reactants to signal active arteritis, yet temporal artery biopsy disclosed dramatic inflammation, forcing the presumption that the infarct was arteritic. Recurrent systemic symptoms and elevation of acute phase reactants are not reliable warning signs of reactivated GCA. In patients at high risk for corticosteroid complications, late biopsy may be a reasonable guide to corticosteroid weaning.     

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.     

Temporal arteritis as a cause of a terrible headache]

Two patients with temporal arteritis are reported in whom terrible headache was the predominant symptom of the disease. Both of them improved after prednisone 60 mg daily in the initial dose. The diagnosis and the treatment of giant cell arteritis are discussed.     

Concordance of bilateral temporal artery biopsy in giant cell arteritis.

OBJECTIVE: To determine whether the diagnostic sensitivity of bilateral temporal artery biopsy is superior to that of unilateral biopsy in cases of suspected temporal arteritis. MATERIALS AND METHODs: A retrospective analysis of the results of 60 bilateral temporal artery biopsies examined in an ophthalmic pathology laboratory. RESULTS: The histopathologic diagnosis in 13% of the biopsy pairs was discordant. There was a 5% chance of obtaining a positive biopsy result on the side opposite an initially negative biopsy result. CONCLUSIONS: Bilateral temporal artery biopsy is 5% more likely than unilateral biopsy to detect the characteristic histopathologic findings in patients with temporal arteritis.     

Temporal arteritis: A case series from south India and an update of the Indian scenario

Objective:

To study the clinical, pathological and prognostic profile of patients with temporal arteritis in India.

Materials and Methods:

The study was conducted in a tertiary care center from south India from 2005 to 2010 in the departments of neurology and medicine. The details of all patients that satisfied the ACR 1990 criteria for diagnosis of temporal arteritis were reviewed. The clinical presentation, laboratory parameters and biopsy findings of the patients were analyzed and compared with other studies from India done over the last decade.

Results:

A total of 15 patients were diagnosed with temporal arteritis. The male:female ratio was 1.5:1. The mean age of onset was 67.58 years. Mean time for detection after onset of symptoms was 2.56 months. Typical manifestations included headache (100%), temporal artery tenderness (100%), jaw claudication (20%), polymyalgia rheumatica (53%) and visual manifestations (20%). The erythrocyte sedimentation rate was elevated in all patients. Biopsy was done in 13 patients, with 11 of them being positive. All patients responded to steroids well, with most patients being symptom-free within the first 48 h of treatment.

Conclusions:

Temporal arteritis seems to be underdiagnosed in India, with all patients previously misdiagnosed, and with a mean time from symptom onset to diagnosis of 2.5 months. The clinical presentation of temporal arteritis in India appears to be similar to that of the West, with no gender preference and a slightly younger age group.     

Internal carotid artery occlusion caused by giant cell arteritis.

A case of hemiplegia in a 46 year old woman is described. Total occlusion of the right internal carotid artery was discovered at angiography. Because of persistent elevation of the ESR, and characteristic plasma protein abnormalities, biopsy of the temporal artery was carried out and demonstrated the typical features of 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.