IS TEMPORAL ARTERY BIOPSY A WORTHWHILE PROCEDURE?

BACKGROUND: Temporal artery biopsy (TAB) has been accepted as the gold standard for the diagnosis of giant cell arteritis (GCA) or temporal arteritis (TA) even though it is of low sensitivity and specificity. Current medical practice recommends commencing high dose steroids before performing a biopsy, and the continued use of long-term steroids even if biopsy is negative but clinical suspicion of the diagnosis is high. The aim of the present study is to determine if TAB results actually changes the management of patients suspected of GCA or TA. METHODS: Retrospective case note analysis of 70 consecutive patients with TAB over 5 years (1999-2003) from Royal Melbourne Hospital (RMH), Melbourne, Australia. RESULTS: Histology revealed five (7%) positive biopsies, five (7%) of 'healed arteritis', and 60 (86%) negative biopsies. After excluding 15 patients who were lost to follow up, management of 13 (23.6%) patients was influenced by the biopsy results: seven with negative biopsies had steroids discontinued while six patients with biopsies showing positive and healed arteritis continued on steroids. Management of 42 (76.4%) patients was not altered following biopsy results: 11 with negative biopsy continued on steroids, 19 never started because of low clinical suspicion and 12 ceased steroids some time after biopsy as there was no symptomatic improvement. CONCLUSION: With the management of 76.4% of patients unchanged following biopsy, some may argue that these patients underwent unnecessary surgery. However, TAB is a minor procedure that can yield important results for the management of GCA, which if untreated can lead to serious complications. We believe TAB should be performed where there is clinical suspicion of GCA.     

Giant cell arteritis presenting as a stroke in the internal carotid artery territory: a case-based review

Giant cell arteritis (GCA) is a large-vessel vasculitis, typically affecting the aorta and its branches. The involvement of vertebral and internal carotid arteries occurs in a limited number of cases, and stroke as a presenting symptom of GCA is extremely unusual: this subset of the disease has a poor prognosis and rarely responds to immunosuppression.We report the case of a 70-year-old woman, who presented to the Emergency Department for ischemic stroke, which appeared to be the first and only symptom of GCA. The prompt administration of steroids and tocilizumab (TCZ) led to clinical and radiological resolution, with no residual disability at 6-month follow-up.Our case-based review, highlighting the rarity of a large vessel vasculitis presenting only with a cerebrovascular accident, provides new evidence for the efficacy of TCZ even in more unusual varieties of GCA: in these cases, TCZ should be immediately prescribed, in order to prevent mortality and severe long-term morbidity.     

Temporal arteritis in the young

Temporal arteritis in the form of giant cell arteritis (GCA) is common in the elderly but is extremely rare in patients less than 50 years of age. We describe two male patients: one who presented at the age of 31 years with painful, nodular swellings of both temporal arteries and whose temporal artery biopsy demonstrated a non-giant cell panarteritis with mixed inflammatory cell infiltrate typical of juvenile temporal arteritis (JTA); another one, aged 40 years, who presented with headache and cerebral angiography consistent with an intracranial vasculitis and whose temporal artery biopsy confirmed an authentic multinucleated GCA. The first patient spontaneously improved after biopsy and the second patient has responded well to corticosteroid therapy. These two cases exemplify well two distinct but extremely rare forms of temporal arteritis in young patients. A 3rd subset is that associated with a systemic vasculitis. Few cases of JTA have been reported and, to our knowledge, we describe in this report one of the only cases of GCA with central nervous system involvement in the young.     

Early revascularization in a patient with perfusion computed tomography/diffusion-weighted magnetic resonance imaging mismatch secondary to acute vertebral artery occlusion. Case report

A 67-year-old woman with a recent history of recurrent ischemic stroke secondary to right vertebral artery stenosis suffered acute onset of left homonymous hemianopsia and the medial longitudinal fasciculus syndrome, which resolved with hyperdynamic therapy. However, consciousness deteriorated 6 hours later. Perfusion computed tomography (CT) revealed regions of prolonged mean transit time in the bilateral cerebellar hemispheres, vermis, brainstem, and right occipital lobe, which were more extensive than the ischemic lesions demonstrated by diffusion-weighted magnetic resonance (MR) imaging. Subsequent cerebral angiography showed occlusion of the right vertebral artery. The patient underwent emergent left superficial temporal artery to left superior cerebellar artery bypass. Postoperatively she demonstrated resolution of the preoperative perfusion CT/diffusion-weighted MR imaging mismatch and improved neurological deficits. Early revascularization in a patient with perfusion CT/diffusion-weighted MR imaging mismatch following acute vertebrobasilar stroke can lead to improvement in cerebral perfusion and neurological function.     

Superficial temporal artery-middle cerebral artery anastomosis in a patient of temporal arteritis with internal carotid artery occlusion: a case report

Temporal arteritis is a rare systemic autoimmune disease and the arteritic process in this case of temporal arteritis involved large and medium-size arteries. Temporal arteritis with internal carotid artery (ICA) occlusion is very rare. We report a case of temporal arteritis with ICA occlusion following superficial temporal artery (STA) -middle cerebral artery (MCA) anastomosis, together with steroid therapy. A 73-year-old female presented with a headache, visual disturbance of left side, and suppression of activity. Left STA was inflammatory and overswelling. Magnetic resonance angiography (MRA) and angiography revealed occlusion of the left internal carotid artery (ICA) at the cervical portion and lowering of vascular reserve on PAO SPECT. Diagnosis as temporal arteritis was conclusive due to the clinical presentation, laboratory studies, and left temporal artery biopsy, so steroid pulse therapy was initiated. Inflammation of left STA disappeared after steroid therapy, but left ICA occlusion on angiography and lowering of vascular reserve on SPECT remained for 3 months afterwards. Because of this, STA-MCA anastomosis was performed. There were no complications after the operation and the donor artery has been patent for two years. Temporal arteritis with ICA occlusion that requires extracranial-intracranial bypass (EC-IC bypass) is very rare. STA-MCA anastomosis with steroid therapy is effective for the prevention of cerebral infarction.     

Single-stage repair for ascending aortic aneurysm,artery stenosis and occlusion of neck vessels caused by Takayasu arteritis

Takayasu arteritis results in a variety of vascular symptoms, and there are some cases in which progressive vascular lesions require surgical intervention. We present a case with ascending aortic aneurysm, right common carotid artery stenosis, left common carotid artery occlusion and left subclavian artery stenosis caused by Takayasu arteritis that was successfully treated with total arch replacement and ascending aorta to right internal carotid artery bypass.     

Simultaneous bilateral vertebral artery occlusion in the lower cervical spine manifesting as bow hunter's syndrome

A 59-year-old male had suffered near-syncopal episodes for more than one year that occurred immediately on turning his head to the left. Cerebral magnetic resonance (MR) imaging did not detect any contributing lesions with well-developed posterior communicating arteries. Dynamic radiography of the cervical spine showed mild instability at C5-6 and concomitant intramedullary hyperintensity confirmed by T(2)-weighted MR imaging. Cervical computed tomography demonstrated an osseous protrusion in the right foramen transversarium at C5. Dynamic cerebral angiography revealed simultaneous vertebral artery (VA) occlusion at C6 on the right and C5 on the left associated with leftward head rotation. The VA showed an intact course at the C1 to C2 levels. The patient underwent anterior fusion followed by decompressive foraminotomy at C5-6 and C6-7 and meticulous resection of the markedly thickened fibroligamentous structure which was considered to contribute to the rotational VA occlusion. Postoperatively he no longer suffered near-syncope during head rotation. Bow hunter's syndrome is an infrequent type of vertebrobasilar insufficiency that may need surgical management for potential threat of ischemic stroke. Simultaneous VA occlusion at the lower cervical level can cause bow hunter's syndrome. Dynamic cerebral angiography is a useful modality for evaluating bow hunter's syndrome even if the patient cannot tolerate dynamic examination for the immediate symptom. Accurate anatomical knowledge of the uncovertebral foraminal region combined with intraoperative dynamic study is essential for safe surgery to the VA and favorable outcome.     

Simultaneous Dissection of Intra- and Extracranial Vertebral Artery. Report of two Cases and Review of Literature

Summary.  Two patients who developed subarachnoid haemorrhage are presented. The first patient was a 41-year-old woman whose angiograms showed right extracranial vertebral artery (VA) dissection starting at the C2 level extending to the intracranial VA near the VA union. Proximal occlusion of the right VA by the endovascular approach was performed. The second patient was a 57-year-old man whose angiograms showed the left intracranial VA dissection distal to the posterior inferior cerebellar artery and an extracranial aneurysmal dilatation of the left VA at the C1 level and extracranial VA dissection in the V3 portion of the right VA. Left intracranial VA dissection was surgically trapped, and the remaining lesions were conservatively treated.  Simultaneous dissection of the intracranial and extracranial portions of the VA is rare. Such lesions usually cause brain ischaemia, but may cause intracranial subarachnoid haemorrhage. Published online July 18, 2002     

Atteinte rénale au cours de la maladie de Takayasu

Renal involvement in Takayasu's arteritis is frequent and worsens the progression of the disease. This is primarily a renal artery stenosis causing renovascular hypertension. The glomerular disease is exceptional. This study was undertaken to determine the clinical, radiological, biological features and therapeutic response in patients with kidney disease associated with Takayasu arteritis. A retrospective chart review was conducted on 11 patients (five men and six females), with a mean age of 31.1 years (19–40 years). The discovery of kidney disease preceded the diagnosis of Takayasu's arteritis in eight cases. Ten patients developed hypertension. Laboratory finding showed proteinuria in five cases of which one case was due to nephrotic syndrome. Renal failure was found in six cases including four cases in stage of terminal chronic renal failure. Impairment of the renal artery was present in nine patients, proximal in seven cases and distal in two cases, bilateral in five cases and unilateral in four cases. Narrowing renal artery was found in seven cases. The renal biopsy revealed membranoproliferative glomerulonephritis in one case and nephrosclerosis in another case. Eleven patients were followed for an average period of 155 months (3–335 months). Remission of nephrotic syndrome was concomitant with the remission of the disease. Seven patients developed outbreaks of Takayasu's arteritis of which six were in care. Relapse of nephrotic syndrome was concomitant with the outbreak of the disease followed by spontaneous remission of both diseases. Improved pressure was obtained in 5 cases and worsening renal function in seven cases. Death was observed in two cases.     

Arm ischemia secondary to giant cell arteritis

Patients with severe ischemic symptoms that fail to respond to steroid therapy, despite a dramatic and continuous drop in the erythrocyte sedimentation rate, may require arterial bypass surgery to overcome arterial ischemia caused by arteritis. We report the case of a patient with bilateral subclavian artery occlusion secondary to giant cell arteritis who responded well to steroid therapy and arterial reconstructive surgery. The patient has remained well for five years.     

Fatal hemorrhage from rupture of the intracranial internal carotid artery caused by aspergillus arteritis

To the best of our knowledge, this is the first reported case of combined intracranial and extracranial hemorrhage due to aspergillus cerebral arteritis. Knowledge of the imaging and the importance of early diagnosis and treatment are emphasized. A 78 year old man developed progressive right-sided visual impairment and diplopia. Magnetic resonance imaging demonstrated a mass lesion located in the right orbital apex, with extension to the cavernous sinus and the right middle cranial fossa. Cerebral angiography showed no aneurysmal dilatation. He was scheduled for transnasal biopsy. However, the patient died of massive epistaxis and intracranial hemorrhage. Postmortem examination revealed an aspergillus granuloma of the orbit and the skull base involving the intracranial and extracranial internal carotid artery. Aspergillus fumigatus was identified by culture. The characteristic feature of the fungal infection is a low-intensity signal on T2-weighted magnetic resonance images. This finding may be useful in diagnosing fungal infection.     

Combined medical,surgical and endovascular treatment of a giant cell arteritis case manifesting as upper limbs acute ischemia

We report a case of giant cell arteritis manifesting as upper limbs ischemia due to a complete occlusion of the left subclavian artery and a high grade stenosis of the right subclavian artery. We decided to use a combined medical, surgical and endovascular treatment followed by long term treatment with methotrexate. After 4 years the patient had no signs or symptoms of relapse. In our personal experience long term treatment with Methotrexate demonstrated a certain efficacy in avoiding relapse of the inflammatory phase and in maintaining stability of results in this kind of disease.     

Usefulness of V3-Radial Artery Graft-V4 Bypass in Bilateral Fusiform Aneurysms of Vertebral Artery: Case Report

A 55-year-old woman with bilateral vertebral artery (VA) aneurysms was transferred to our hospital. She suffered from a minor stroke. Magnetic resonance imaging (MRI) for the stroke incidentally revealed bilateral VA aneurysms. Due to its size, more observation was recommended, and the patient was found eager to be treated. Both side surgeries were found inappropriate because of severe lower cranial nerve disturbances. The right aneurysm involved the posterior inferior cerebellar artery (PICA) and the V4 segment was deviated to the right side. Therefore, the smaller right aneurysm was treated first with an occipital artery (OA)-PICA bypass and a V3-radial artery graft (RAG)-V4 bypass followed by proximal clipping of the PICA and the right VA. The right VA was successfully remade by RAG and the right aneurysm was not revealed on postoperative examination. By doing so, the opposite aneurysm was able to be eliminated by the parent artery occlusion even by using an interventional radiology (IVR). The V3-RAG-V4 bypass is a useful method for treating bilateral VA aneurysms. This is a new bypass which has not been reported so far to the best of our knowledge.     

Giant cell arteritis

Opinion statement Patients with a suspected diagnosis of giant cell arteritis (GCA) should be started on high-dose corticosteroid therapy without delay. A temporal artery biopsy should be performed after initiation of therapy to confirm the diagnosis. Patients with acute visual or neurologic symptoms present a neuro-ophthalmic emergency. Therapy should be initiated immediately with high-dose intravenous methylprednisolone sodium succinate and followed by high-dose oral prednisone. Treatment may begin with highdose oral prednisone in patients without visual or neurologic symptoms. Calcium, vitamin D, and peptic ulcer prophylaxis should accompany steroid therapy, as indicated. The following treatments should be considered for patients with suspected GCA and acute visual or neurologic signs or symptoms: intravenous methylprednisolone sodium succinate (250 mg intravenously every 6 hours) should be given for 3 days, followed by oral prednisone (80 mg per day or 1 mg/kg) for 4 to 6 weeks. Prednisone should then be tapered by 10 mg per day every month. Most patients require 1 year of therapy to avoid relapse. Taper and duration should be modified according to erythrocyte sedimentation rate, C-reactive protein, and signs and symptoms of GCA. Rheumatologic consultation and follow-up is often helpful for these patients. For patients with suspected GCA and no acute visual or neurologic signs or symptoms, therapy may begin directly with oral prednisone (80 mg per day or 1 mg/kg) with same taper and duration based on laboratory values and clinical signs and symptoms.     

Mega-aorta syndrome development in giant cell arteritis. A same entity?

Giant cell arteritis (GCA) is the most common form of large vessel arteritis. GCA typically involves the branches of the external carotid artery, but is the leading cause of inflammatory aortitis. However, involvement of the aorta often goes undetected. We present a case of an 81-year-old man, with headache and intense chest pain, who was previously given a diagnosis of GCA with a temporal artery biopsy 6 years ago. Owing to the suspicion of acute aortic syndrome, an emergent computed tomography (CT) was performed. CT showed the development of mega-aorta syndrome, with a diameter of 75.2 mm in the ascending aorta, 61.8 mm in the aortic arch, 76.1 mm in the descending thoracic aorta, and 45.1 mm in the abdominal aorta, presenting a chronic type B aortic dissection. Although there are reported cases secondary to Takayasu arteritis, this is the first case reported in the literature of mega-aorta syndrome associated with GCA in a patient previously diagnosed using temporal artery biopsy.     

The association of gout with incident giant cell arteritis in older adults

Objectives

To assess whether gout is associated with a higher or lower risk of a new diagnosis of giant cell arteritis (GCA) in older adults, adjusting for known risk factors of GCA.

Long-term immunosuppressive treatment of a child with Takayasu's arteritis and high IgE immunoglobulins

A 7-year-old child presented with a severe form of Takayasu's arteritis, with two consecutive episodes involving the right testis and then the left kidney 6 months later. The renal artery obstruction was accompanied by severe hypertension. An aortography showed a complete obstruction of the left renal artery and a narrowing of the right subclavian artery. Plasma renin activity was high. Serum immunoglobulins were within the normal range, except for an increase in IgE (880 /l). Despite 4 months', treatment with antihypertensive drugs, prednisone, cyclophosphamide, and anticoagulant, the blood pressure never returned to normal and the left renal function remained completely absent. A nephrectomy was performed which immediately normalized plasma renin activity and blood pressure. The child was subsequently treated with alternateday prednisone for 3 months, alternating with 3 months of cyclophosphamide or, later, azathioprine. Persantine (dipyridamole) and acetylsalicylic acid were administered continuously. The right radial pulse returned to normal within 2 years. An 8-year follow-up failed to detect any new episode of arteritis. The right kidney showed signs of compensatory hypertrophy. Finally, a recent arteriography demonstrated not only a normal right renal artery blood flow but almost total disappearance of the right subclavian artery obstruction. However, the IgE remained abnormally high (2,023 g/l).     

The length of superficial temporal artery biopsies

BACKGROUND: To compare temporal artery biopsy specimen lengths from a tertiary care and a community hospital in New South Wales to recommended clinical guidelines in suspected giant cell arteritis. DESIGN: A retrospective observational study of all patients who underwent temporal artery biopsy at Bathurst Base Hospital (BBH) and Royal Prince Alfred Hospital (RPAH) over a 5-year period. METHODS: Patients who underwent temporal artery biopsy during the 5-year period were identified using computerized hospital databases. A retrospective chart review was carried out on all cases. Data were collected regarding patient age, patient sex, length of biopsy specimen, histopathological results and surgical team carrying out the biopsy. RESULTS: During the 5-year period, 157 temporal artery biopsies were carried out at both hospitals, with 38/157(24%) at BBH and 119/157 (76%) at RPAH. There was no significant difference in biopsy length at the two hospitals. The mean specimen length at BBH was 12.1 mm compared with 11.7 mm at RPAH (t=0.35; P=0.73). At RPAH, there was no significant difference in specimen length between the surgical specialties carrying out the biopsy (ANOVA F=1.37; P=0.26). Specimens of length 20 mm or greater were 2.8 times more likely to show features of giant cell arteritis than those less than 20 mm. CONCLUSION: The mean length of temporal artery biopsy specimens at both hospitals was substantially shorter than recommended guidelines of a minimum 20 mm. We recommend all surgeons carrying out temporal artery biopsies ensure a specimen of sufficient length is obtained.     

A case of lupus nephritis coexisting with podocytic infolding associated with Takayasu’s arteritis

A 61-year-old Japanese woman with both Takayasu’s arteritis (TA) and systemic lupus erythematosus (SLE) presented with proteinuria due to glomerulopathy associated with podocytic infolding. She presented with unequal pulses in the upper extremities at 38 years old. TA was diagnosed on the basis of angiographic identification of stenosis in the left subclavian artery. Eight years after onset of TA, SLE was diagnosed on the basis of clinical and laboratory findings, including proteinuria, hematological and immunological abnormalities, high titer of antinuclear antibody, and a positive lupus band test on the skin. A renal biopsy showed lupus nephritis coexisting with podocytic infolding associated with TA, which has rarely been reported. After low-dose prednisolone therapy and immunosuppressive therapy by cyclosporine for 14 years, proteinuria has persisted without deterioration of serum creatinine levels.