Giant cell arteritis presenting as renal vasculitis.

Giant cell arteritis commonly presents with headache, polymyalgia, and visual signs and symptoms. Other neurological, respiratory or vascular symptoms occur in 10-30% of patients. It is extremely rare for giant cell arteritis to present initially with haematuria. Here we describe a case which presented with fever and haematuria, which emphasise the need to be vigilant about the diagnosis of giant cell arteritis as an underlying cause.     

Giant cell arteritis causing bilateral sequential anterior ischaemic optic neuropathy--a case report

Giant cell arteritis is a chronic granulomatous inflammation of unknown aetiology involving large and medium size arteries in the elderly. It causes acute visual loss from ischaemia to the optic nerves or central retinal artery occlusion. This is a rare cause of anterior ischaemic optic neuropathy in our local population. We present a patient who had bilateral loss of vision from sequential arteritic ischaemic optic neuropathy. She was treated with intravenous steroids immediately. Diagnosis was based on histopathological studies of temporal artery biopsies.     

Giant cell arteritis

Giant cell (senile, temporal or cranial) arteritis occurs three times more frequently in women and the majority of patients are over 70 years of age. This series of forty-seven patients presented with visual symptoms and most also had a constitutional upset. The ocular signs were of ischaemic papillopathy (in 80%), which was unilateral in 61% and bilateral in 17%; or central retinal artery occlusion (13%). The ESR was always elevated above 40 mm, and in 28% it was above 100 mm. The main ophthalmic differential diagnosis was to determine whether it was arteritic or atherosclerotic ischaemic papillopathy in this age group, and principal differences are outlined (Table 6). Following corticosteroid therapy, vision improved in 23%, remained unchanged in 64% and deteriorated in 13% of patients. Visual deterioration predominated in elderly women who received steroids late in the course of the disorder. Early steroid therapy is recommended in elderly patients with visual symptoms and significantly raised ESRs.     

Giant cell arteritis

Giant cell arteritis (GCA), temporal arteritis or Horton's arteritis, is a systemic vasculitis which involves large and medium sized vessels, especially the extracranial branches of the carotid arteries, in persons usually older than 50 years. Permanent visual loss, ischaemic strokes, and thoracic and abdominal aortic aneurysms are feared complications of GCA. The treatment consists of high dose steroids. Mortality, with a correct treatment, in patients with GCA seems to be similar that of controls.     

Giant cell arteritis with panocular involvement in an Indian male

A case of giant cell arteritis with systemic and panocular involvement is reported here. This elderly Indian male presented with symptoms of unilateral temporal headache and intermittent jaw claudication for a month followed by diplopia and blurring of vision and later loss of vision in the right eye. The right eye showed some limitation of ocular movements, presence of relative afferent pupil defect, anterior segment ischaemic changes and anterior ischaemic optic neuropathy. Visual evoked potential showed an absent P1 wave while the left eye with normal 6/6 vision sowed a prolonged P1 wave. Fundus fluoresceine angiography showed delay in choroidal perfusion. His erythrocyte sedimentation rate (ESR) was 120 mm/hr and he was started on oral prednisolone. Superficial temporal artery biopsy obtained one week after starting steroids was positive for giant cell arteritis. Steroids led to the resolution of optic disc swelling, disappearance of anterior segment signs, full recovery of right ocular movements and no further deterioration of the fellow eye. On steroids, he developed insomnia and progressive myopathy which resolved and is now symptom free at lower doses of steroids.     

巨细胞动脉炎的临床分析(附8例报告)

目的分析巨细胞动脉炎的临床表现、辅助检查结果及对不同治疗的效果和误诊的原因。方法对8例患者的首发症状、临床表现、局部检查、实验室检查、组织学检查和对不同治疗的效果进行分析。结果巨细胞动脉炎可以有不同的首发表现形式，但头痛是主要的临床症状（6例）。实验室检查：8例血沉均增快，平均86mm／h；5例血红蛋白减低，平均为6．1g／L；4例CRP增高，平均为132mg／L。颞动脉组织学检查是诊断的一种重要手段．3例行颞动脉检查（6条），5条内膜欠光滑、腔内回声不佳；1条未见异常。皮质激素有非常明显的治疗效果。结论对以头痛为首发症状的中老年人，特别是颞、枕和颈部胀痛和剧痛，伴或不伴发热、眼胀和复视的患者，要行颞动脉触诊检查．注意血红蛋白、ESR和CRP的变化，必要时行颞动脉超声检查，颞动脉分段活检是诊断巨细胞颞动脉炎的金标准．皮质激素是治疗的首选。     

Giant cell arteritis as a cause of death. Report of nine cases

Nine patients who have died of giant cell arteritis (GCA) are described. Two patients died of myocardial infarction caused by GCA in the coronary arteries. Another two patients died of dissecting aneurysm of the aorta where GCA were found in the lesions of the aorta wall. Giant cell arteritis causing cerebral stroke was the cause of death in five patients. None of the cases were receiving adequate corticosteroid treatment when symptoms of the ischemic catastrophies started. These cases illustrate that GCA is a generalized arteritis that can involve arteries of vital importance.     

Giant cell arteritis presenting as oculomotor nerve palsy with pupillary dilatation.

Acute complete oculomotor palsy with headache is a classical presentation of an extrinsic compression most commonly due to a posterior communicating artery aneurysm. We present a patient with such a presentation but with histologically proven giant cell arteritis. This possibility should be considered especially in the elderly to avoid complications and the need for angiography.     

颞动脉炎24例临床分析

目的:总结颞动脉炎的神经系统临床特征。方法:对诊断为颞动脉炎的24例患者进行回顾性分析,根据临床表现、颞动脉活检、糖皮质激素治疗效果作为诊断标准,以1990年美国风湿病学会(ACR)制定的颞动脉炎诊断标准作为参考。结果:24例患者被诊断为颞动脉炎,8例经颞动脉活检证实7例。其中,头痛为主要症状,本组24例中有22例(91.7%);视力障碍及眼痛较常见,本组有3例(12.5%),其中有1例失明;低至中度发热10例(41.7%),体温37.2～39℃;合并肌炎6例;颞动脉均增粗,呈条索状,搏动减弱。经糖皮质激素治疗后其症状或体征常在数小时至数天消失。结论:颞动脉炎的神经系统症状与体征较为常见,可见严重的并发症,早期诊断及治疗可得到有效控制。     

Scleritis and temporal arteritis.

Thirty consecutive patients with severe scleritis or episcleritis were admitted as in-patients to the Medical Ophthalmology Unit and assessed for systemic disease. There were seventeen women and thirteen men. The mean age was 53 with a median of 57 (range 23-83). Eighteen of the patients had scleritis: eleven of these had evidence of connective tissue disease and three of them had temporal arteritis. Twelve patients had episcleritis: six of them had a collagen disease and one of them developed temporal arteritis. This high incidence of temporal arteritis in association with scleritis has not been previously reported. It is important to diagnose and treat overt temporal arteritis early with parenteral steroids so that ischaemic papillopathy can be avoided. A higher incidence of collagen diseases than previously described is reported in episcleritis. It is thought that this is secondary to selection since patients with the usual self-limiting episcleritis are not normally referred for further in-patient investigation. In no patient was more than one significant diagnosis made. There was no significant medical illness in only 11% of patients with scleritis and 33% of patients with episcleritis. The majority of the non-collagen diseases (e.g. hypertension) were not previously recognized. In none of the patients with temporal arteritis was the diagnosis made before admission. It is concluded that full examination and investigation for underlying disease is indicated in both scleritis and severe episcleritis.     

Cranial diabetes insipidus caused by giant cell arteritis

A 78 year old woman was admitted with symptoms of diabetes insipidus and was found to have a high ESR, the cause of which was uncertain. Five months later she presented with blindness, and cranial arteritis was confirmed by temporal artery biopsy. The endocrine abnormality returned to normal after steroid therapy. The association between these two conditions is discussed.     

Steroid sensitive systemic disease with anaemia in the elderly: a manifestation of giant cell arteritis?

Nine elderly patients presented with features of a multisystem disorder thought to be either a connective tissue disease of undefined type or disseminated malignancy. Associated features were a normochromic anaemia, raised erythrocyte sedimentation rate (ESR) (or plasma viscosity) and raised serum alkaline phosphatase levels. None had symptoms to suggest either giant cell arteritis or polymyalgia rheumatica. Temporal artery biopsy was performed before trial of corticosteroid therapy in four, and two showed giant cell arteritis. All nine responded dramatically to corticosteroids and the anaemias resolved. One died after 6 y, and the rest are well after 1 to 7 y.     

多发性大动脉炎的超声诊断价值

目的探讨多发性大动脉炎的二维及彩色多普勒超声图像特点,提高超声对本病诊断的准确性。方法对29例多发性大动脉炎患者病变段血管进行二维及彩色多普勒超声检查,分析病变段血管的声像图改变。结果29例多发性大动脉炎患者中头臂型15例,混合型12例,肾动脉型1例,胸腹主动脉型1例,病变段血管内壁增厚,回声减低,致管腔不同程度狭窄,为21%~95%。并发锁骨下动脉盗血综合征2例,颈总动脉闭塞8例。结论多发性大动脉炎累及血管最多为头臂型,其次为混合型。二维及彩色多普勒超声检查病变段血管的形态学及血流动力学改变,可作为临床诊断多发性大动脉炎的常规方法。     

Unusual variants in the presentation of temporal arteritis.

A retrospective study was made of 96 patients diagnosed as cranial arteritis of whom 32 were accepted using strict clinical criteria or a positive temporal artery biopsy. Unusual presentations of fever, psychiatric illness, headache-free patients and a ''normal'' ESR are described. The recognition of these variations is important in the early diagnosis of temporal arteritis.     

多发性大动脉炎8例临床分析

目的：探讨头臂型大动脉炎的诊断和治疗方法。方法：回顾性分析8例多发性大动脉炎的临床资料，在8例头臂型大动脉炎患者中重症头臂型5例，其中双侧颈总动脉、锁骨下动脉闭塞3例，行升主动脉-双侧颈总动脉-双侧锁骨下动脉与人造血管搭桥。一侧颈总动脉、双侧锁骨下动脉闭塞1例，行升主动脉-左侧颈总动脉-双侧锁骨下动脉与人工血管搭桥。左侧颈总动脉及左侧锁骨下动脉闭塞1例，行锁骨动脉-锁骨下动脉-左颈总动脉与人造血管搭桥。其余3例单侧锁骨下动脉闭塞行锁骨下动脉-锁骨动脉与人造血管旁路术。结果：8例大动脉炎患者随防5个月～9年，1例手术后第3年复发，行第二次手术，7例患者的脑缺血及上肢缺血症状消失，能进行正常生活和工作。结论：大动脉炎活动期最好用皮质激素，扩张血管，降低血液粘滞度抗血小板聚集等药物保守治疗，病情稳定后，根据病变的血管情况来选择血管重建术和术后继续抗凝治疗是治疗大动脉炎的有效方法。     

Acute myocardial infarction and subclavian artery occlusion in a 41-year-old woman with Beh?et’s disease: coronary and large vessel arteritis

We report the case of a 41-year-old Chinese woman with Behçet’s disease (BD) complicated by acute myocardial infarction, requiring inotropic and ventilatory support. Angiography showed critical left anterior descending coronary artery stenosis, a blocked left subclavian artery and left carotid artery stenosis. The patient was successfully treated with a high dose of immunosuppressants, standard anti-ischaemic therapy and percutaneous coronary intervention. Although life-threatening, coronary arteritis is a treatable manifestation of BD. We suggest that the diagnosis of coronary arteritis be considered in patients with BD who present with chest pain. Involvement of other arteries should also be looked out for in these patients.     

多发性大动脉炎颈动脉病变的超声诊断价值

目的总结多发性大动脉炎颈动脉病变的超声表现特点,提高其诊断价值。方法回顾性分析25例经临床诊断为多发性大动脉炎的颈动脉超声声像图表现。用彩色多普勒超声检查患者的颈总动脉、颈内动脉和颈外动脉,观察并测量颈动脉管腔内径、内膜—中膜（IMT）厚度、彩色多普勒血流显像（CDFI）和脉冲多普勒,分析颈动脉血流变化的频谱及彩色多普勒声像图指标;鉴别颈动脉狭窄与闭塞,估测狭窄程度以及评估颈动脉闭塞的范围;并与数字减影血管造影（DSA）检查结果进行比较。结果 25例患者中共有50条颈动脉受累,其中39条（78%）颈总动脉受累,4条（8%）颈外动脉受累,7条（14%）颈内动脉受累。在39条受累的颈总动脉中,37条表现为动脉壁IMT弥漫性增厚;2例局限性增厚。1例颈总动脉内膜剥脱伴夹层动脉瘤形成,IMT厚度1.8～5.7mm,管腔都有不同程度的狭窄和闭塞。超声检查血管狭窄或闭塞的诊断符合率与DSA结果比较差异无统计学意义（χ2=0.4224,P〉0.05）。结论应用彩色多普勒超声能显示颈动脉管壁的特征性变化,并可显示管腔内血流动力学异常,为临床提供重要的诊断信息。     

多发性大动脉炎合并脑梗死研究进展

在中国脑梗死已成为第一大致死性疾病,具有高发病率、高死亡率、高致残率、高复发率等特点。近年来脑梗死的致病因素已成为研究热点,如高血压、高血脂等危险因素已被广泛接受并在一定程度上得到控制,然而15~45岁的青年脑梗死发病率仍逐年增加,对年轻患者身体、精神以及家庭造成致命打击,这迫切要求我们更深入地探讨脑梗死的发病机制。据报道约10%~20%大动脉炎患者可并发脑缺血发作,以引起多发性脑梗死以及中青年脑梗死为特点,是引起长期病态和过早死亡的主要因素。研究发现,大动脉炎合并脑梗死的早期诊断可有效降低青年脑梗死死亡率同时提高患者生活质量,相较于普通脑梗死患者治疗手段,多发性大动脉炎脑梗死更应该慎重选择精准治疗方案。本文参考近年来国内外大动脉炎并发脑梗死的研究进展,就该疾病的病理、诊断以及治疗进行深入探讨,以期为临床治疗提供依据。     

48例大动脉炎临床分析

目的 探讨大动脉炎(TA)的临床特点,加深对大动脉炎的认识.方法 回顾性分析我院1990-2010年收治的48例大动脉炎患者的临床表现,总结发病年龄、性别、病变特征及治疗方法等.结果 男女患者比例为1∶2.7,发病年龄＞40岁者5例,占10.4％;临床分型头臂动脉型32例,广泛型6例;首发症状中最常见的是头晕(27例,...     

Polymyalgia rheumatica/giant cell arteritis in a Cambridge general practice

The aim of this study was to establish the incidence and prevalence of polymyalgia rheumatica/giant cell arteritis in general practice. Patients with this disorder, whether previously diagnosed or not, were ascertained by using a questionnaire administered by interview, and all received full clinical and laboratory assessment. A total of 579 patients aged 65 and over was seen, and 19 (33/1000) had been diagnosed or developed symptoms within the previous eight years. Thus the calculated annual incidence in those aged 65 and over was about 4/1000. The figures from this first large scale study of polymyalgia rheumatica/giant cell arteritis in general practice are much higher than those from studies carried out in hospital. The questionnaire was effective in both identifying known cases of polymyalgia rheumatica/giant cell arteritis and detecting new cases. As this is a treatable disorder, it is important that doctors become aware of how common it is in elderly people.