Temporal arteritis with normal erythrocyte sedimentation rate

The erythrocyte sedimentation rate (ESR) is used to differentiate temporal arteritis from other forms of headache in the elderly. Though temporal arteritis can occur with a normal ESR, this is not generally appreciated in primary care. The case reported here is a 74-year-old woman with biopsy-proven temporal arteritis; her ESR was 22 mm/hr. Of note, her hematocrit was more than 40 percent, a condition previously reported to be associated with temporal arteritis and a normal ESR. This discussion focuses on the diagnosis of temporal arteritis and its associated symptoms.     

Aortic involvement in patients with temporal arteritis and polymyalgia rheumatica

In 4 patients with temporal arteritis or polymyalgia rheumatica, women aged 60, 57, 83 and 73 years respectively, signs of aortic involvement were established. The first patient presented with signs of systemic inflammation without signs of temporal arteritis or aortitis. In the second, an acute symptomatic thoracoabdominal aneurysm developed. In the third, temporal arteritis was associated with chronic progressive dilatation ofthe thoracic aorta. The fourth developed signs of intermittent claudication of the extremities. The clinical manifestations in all patients were attributed to chronic inflammation of the aorta caused by giant cell arteritis. Aortic giant cell arteritis frequently accompanies temporal arteritis, but is rarely diagnosed. Up to 75% of patients with temporal arteritis may have some degree of aortic involvement. Thoracic aneurysms, complicated by rupture or dissection, are the most serious complications. Aortic disease associated with signs of systemic inflammation should trigger the suspicion of giant cell arteritis. Corticosteroids are the most important part of treatment. Three patients recovered following treatment; the first two received an endoprosthesis; in the woman aged 83 years, this was not technically possible; she died after 1.5 years.     

Rare complication in a patient with giant cell arteritis

Giant cell arteritis (temporal arteritis) is a chronic vasculitis that usually affects older people. Although this is a systemic disease, it most often affects the cranial arteries. The most frequent complication of this disorder is visual loss. We report the case of a patient who suffered several rare complications, including tongue necrosis, as a result of being diagnosed with giant cell arteritis following the start of treatment.     

Temporal arteritis: an atypical presentation

Temporal (giant cell) arteritis is a systemic granulomatous vasculitis primarily involving branches of the carotid arteries in patients aged 50 years and greater. Its classic symptoms and signs are headache and elevated erythrocyte sedimentation rate (ESR), but this is not the only presentation. This case is the first reported in a Korean, whose chief complaint was pain in the tongue and headaches and whose ESR was normal. The diagnosis was confirmed by biopsy. Her symptoms abated when treated with prednisone, but she developed diabetes mellitus, osteoporosis, and compression fracture of lumbar vertebrae while being treated. Primary physicians should become aware of the atypical features of this disease, as well as the potential complications of treatment.     

Diagnosis and prognosis of polymyalgia rheumatica and giant cell arteritis

Retrospectively the data were examined of 69 patients with polymyalgia rheumatica (PMR) and giant-cell arteritis (GCA), of whom 62 were treated with corticosteroids. The clinical and laboratory data, and the outcome of temporal artery biopsy were compared. In addition the relation between the course of the disease and survival was investigated. PMR and GCA are closely related syndromes with a multiform clinical presentation. There even is a group of patients with merely systemic symptoms, without specific signs of PMR or GCA. Concerning the course of the disease patients can be divided into groups with a shorter and longer period of disease activity; patients with a smooth stable remission and a chronic disease. The former group shows a significantly better five-year survival. At the time of diagnosis no reliable prediction of the course of the disease is possible.     

Takayasu arteritis: a chronic vasculitis that is rare in children

Takayasu arteritis was diagnosed in two children, a 15-year-old girl and a 10-year-old boy. The girl had suffered from fatigue, malaise, abdominal pain and weight loss for several months, but no clear cause could be found. A few weeks later, when a blood pressure of 222/155 mmHg was measured, the possibility of renal artery stenosis was considered and imaging studies revealed indications for Takayasu's disease. The patient was given methylprednisolone followed by a combination of prednisone and, initially, cyclophosphamide, later methotrexate. This resulted in a clinical remission of the inflammatory process. The boy presented with increasing fatigue and variable episodes of fever. After 3 years, sarcoidosis or Castleman's disease were considered. Imaging studies revealed aortic stenosis. He underwent stenting of the involved vessel segment. Takayasu arteritis is a chronic vasculitis of unknown origin, affecting mainly the aorta and its main branches. As a result of the inflammation, stenosis, occlusion or dilatation of the involved vessels may occur and cause a wide range of symptoms. Especially in the early phase, the symptoms often are non-specific. One should look for hypertension, blood pressure differences between the two arms, decreased peripheral pulsation or bruits over the aorta and its major branches. Radiological examination may consist ofangiography, magnetic resonance imaging or CT-scans. Treatment consists of corticosteroids and other immunosuppressants, such as cyclophosphamide, methotrexate, azathioprine, and antagonists of tumour-necrosis factor alpha. In addition, balloon dilatation or stenting is often necessary.     

Etiopathogenesis of giant cell arteritis

Giant cell arteritis is a vasculitis of large and medium size arteries, especially those of the aortic arch with an extracranial distribution, but also the aorta and some of its larger branches. It is characterised by the presence of mononuclear inflammatory infiltrates close to the internal elastic lamina formed by lymphocytes and macrophages, which in slightly more than 50% of the cases contain multinucleate giant cells. The morbidity associated with this disease is related to phenomena of distal ischemia to the luminal stenosis of the inflamed arteries, and to a lesser extent to the formation of aneurisms due to the weakening of the arterial wall. With an unknown aetiology, its pathogenesis is immune through the migration and location of gamma-INF -producing T cells in the adventitia of the inflamed arteries, it being assumed that this is the place of immune stimulation by a still unidentified antigen. The recruitment and activation of macrophages by this cytokine is one of the most important points of its pathogenesis. The destruction by these of the arterial elastic tissue is a relevant phenomenon, as is the production of other factors promoting neoangiogenesis and a proliferation of neointime, responsible through obliterating light for the ischemic manifestations of the disease. The process is accompanied by an important systemic repercussion characterised by a strong reaction of acute phase and general but barely specific symptoms of disease. On the other hand, an important percentage of patients show a clinical picture of polymyalgia rheumatica, an entity that has a historical and controversial relationship to this arteritis. In recent years important contributions have been made to the understanding of the immune mechanisms involved in its pathogenesis.     

A population-based case-control study of temporal arteritis: evidence for an association between temporal arteritis and degenerative vascular disease?

The first population-based incident case-control study of temporal arteritis (TA) in the US was conducted using the unique data resources of the Rochester Epidemiology Project. During the period 1950-1985, 88 newly diagnosed cases of biopsy-proven TA were identified among residents of Olmsted County, Minnesota. Cases were each matched to four Olmsted County community controls on age, sex and duration of community medical record. Odds ratios (OR) were calculated for marital status, education, Quetelet index, pregnancy, age at menopause, thyroid disease, diabetes, smoking, hypertension, angina, myocardial infarction, peripheral vascular disease, and stroke. Multivariable conditional logistic regression analysis identified statistically significant adjusted OR for smoking (2.3, 95% CI = 1.3-4.1). Elevated ORs which were not statistically significant were noted for angina, myocardial infarction, and peripheral vascular disease. These data suggest that TA and arteriosclerosis may share a common causal pathway. Alternatively, histopathological misclassification of temporal artery biopsies may have resulted in the observed association. Due to the limited power of this population-based study, multicentre collaboration should be encouraged to more precisely define the epidemiology of TA.     

儿童大动脉炎35例临床分析

目的:探讨儿童大动脉炎的临床特点,提高对该病的认识。方法:研究对象为1990年1月～2008年1月在吉林大学第一医院儿科住院并确诊为儿童多发性大动脉炎的患儿共35例,对其城乡分布、临床表现、病情活动性指标以及病情严重性等进行回顾性分析并与文献报道过的成人多发性大动脉炎进行比较。结果:35例患儿中男18例,女17例,发病年龄4～15岁,平均为(11.5±2.2)岁,确诊时病程7天～48个月,其中位数时间为2个月。临床分型以Ⅱ型为最多占57.1%,首发症状以头痛(51.4%)、抽搐(37.1%)等较为多见;在体征方面高血压94.3%、血管杂音61.4%、无脉或脉弱74.1%,共有22例患儿心脏受累所占的比例约为62.8%;仅有不到一半的患儿出现炎性指标升高,就诊时共有30例患儿处于病情活动期占85.7%,有94%以上的患儿出现一种或一种以上的并发症。结论:与文献报道中的成人发病情况比较,儿童多发性大动脉炎并非少见而且较成人大动脉炎起病急、病情重、预后差,在临床工作中应注意监测血压,以减少漏诊及误诊的几率。     

Two coronary artery fistulae in a patient with temporal arteritis and chest pain.

A 59-year-old woman with a history of cardiac sounding chest pain was investigated by coronary arteriography. Two unsuspected fistulae were found. The fistulae originated from the distal right coronary artery and the diagonal branch of the left coronary artery. Both drained into the left ventricle. She was also found to have temporal arteritis, treatment of which abolished the chest pain.     

Cannabis use and untreated HIV-infection: unknown risk factors for premature peripheral artery disease

A 32-year-old woman presented with a painful leg and a gangrenous big toe. Her medical history included HIV-infection that had remained untreated for 8 years. In addition, she had smoked about 10 cannabis-cigarettes daily during the previous 15 years. Physical examination and angiography confirmed the presence of severe peripheral artery disease in the left lower leg. She received a femorodistal bypass graft but the gangrene was progressive, ultimately necessitating a lower leg amputation. Histopathological examination revealed intimal fibrosis and thrombosis with recanalisation in combination with fragmentation of the internal elastic membrane. Peripheral artery disease is often associated with lower extremity ischaemia, mostly affecting elderly patients and almost always caused by atherosclerosis. When ischaemic symptoms manifest themselves in young individuals (<40 years), rare causes of obliterative arterial disease, such as inflammation or post-traumatic vascular injury, must be excluded. Use of cannabis and untreated HIV infection are both relatively unknown risk factors for the onset of premature non-atherosclerotic arterial disease. Stopping the smoking of cannabis appears to have a favourable effect on the ischaemic symptoms. Whether treatment of HIV-infection can affect the course of premature peripheral vascular disease is unknown. When deciding whether or not to give antiviral therapy, care providers should also consider the increased cardiovascular mortality rates associated with these treatment regimens. In the case described, the HIV-infection was considered the most likely cause of the peripheral artery disease, based on all the histopathological findings.     

Ocular involvement in giant cell arteritis

Giant cell arteritis (GCA) is a systemic disease with a wide spectrum of clinical manifestations. Severe visual loss is the most frequent serious complication. This article reviews current ideas about GCA and in particular its ocular manifestations.     

Takayasu's arteritis on steroid therapy. Seven years follow-up

The authors report a 7 year follow-up of Takayasu's arteritis (TA) type III, group 1, in a young Italian woman. At diagnosis, at the age of 25, the echotomographic and angiographic studies showed narrow subclavian arteries, narrow abdominal aorta (diameter of 0.6-0.8 cm) below the renal arteries, stenotic left common carotid and renal arteries, and occluded upper mesenteric artery. With steroid therapy, (prednisone 50 mg/day per os), the erythrocyte sedimentation rate (ESR) normalized within 12 days. With a maintenance dosage of 7.5 mg/day per os, the patient achieved remission as documented by the absence of symptoms, the persistent normalization of ESR, and the improving of the diameter of the abdominal aorta (1.3-1.4 cm). On steroid therapy, the patient had a normal pregnancy and delivered a healthy baby girl. The disease has been stable for seven years. Recently, diabetes mellitus occurred and it has been treated with insulin therapy. The rising of ESR after tapering of steroid therapy (prednisone 5 mg per os on alternate days) suggests an alternative treatment with a cytotoxic agent.     

Takayasu's arteritis associated with Crohn's disease. Report of a case

The authors report a rare case of Takayasu's arteritis with aortic arch and abdominal aorta involvement associated with Crohn's disease of the colon which both occurred in a young female patient. The coexistence of two immune mediated diseases in the same subject is unusual for they are generally considered to be independent of each other, however a hypothesis of their possible interrelationship is put forward.     

The results of coronary arteriography in young men after myocardial infarction in north-east Scotland

The case records of 50 consecutive male patients aged 40 years or under who were investigated by selective coronary arteriography after myocardial infarction were reviewed. Fourteen patients had normal coronary vessels and 36 patients had significant occlusive disease. Eighteen were considered to be in need of surgical treatment. The features of myocardial infarction on the ECG were less marked in the group of patients with normal coronary arteriograms. Many of these patients were asymptomatic and had complete resolution of the ECG changes. As well as having normal coronary arteries, many also had normal left ventricular angiograms. Cigarette smoking was very common in the whole group, 86 per cent of patients being moderately heavy cigarette smokers. Five of the 14 patients in the 'non-occlusive' group were non-smokers and only two of the 36 patients in the 'occlusive' group were non-smokers (P less than 0.01). The fasting serum cholesterol was significantly lower in the 'non-occlusive' group than in the 'occlusive' group. There was no significant difference between the two groups regarding blood pressure, family history of ischaemic heart disease, obesity or alcohol consumption. There was, however, a high incidence of heavy alcohol consumption amongst patients who subsequently required coronary artery surgery. Many of the patients in the 'non-occlusive' group were considered to have had smaller, more localised myocardial infarctions and a lesser degree of coronary disease which may not be detected by coronary arteriography. Others, with more widespread cardiac damage, could be explained on the basis of thrombosis and subsequent recanalisation.     

Risk-benefit considerations in the management of polymyalgia rheumatica

Management of the polymyalgia rheumatica syndrome (PMR) is controversial. Gratifying symptomatic response usually occurs after treatment with nonsteroidal antiinflammatory drugs or low doses of corticosteroids. However, some PMR patients are at risk of sudden blindness from an associated disease, giant cell arteritis (GCA). Blindness can be prevented by using higher and more toxic doses of corticosteroids. Temporal artery biopsy can be an aid in diagnosis, though it is not a completely sensitive test for GCA. This study employs decision analysis and derived risk-benefit ratios (equivalent to utility ratios) to evaluate five possible PMR management strategies. The incremental risk-benefit analysis provides a means for weighing intangible trade-offs without a formal utility analysis. Given base case assumptions derived from the literature, empirical treatment with high-dose steroids cannot be justified for PMR patients who have no cranial arteritic symptoms, because the acceptable risk-benefit ratio associated with this strategy is more than 90 cases of severe medication side effects per case of monocular blindness averted.     

A case of multiple peripheral hepatic artery aneurysms after chemoembolization

A case of 57-year-old man with multiple peripheral hepatic artery aneurysms is reported. The patient was admitted with liver dysfunction and detected hepatoma in the right lobe. Chemoembolization was performed for the treatment of hepatoma. After 35 days, reangiography revealed multiple peripheral hepatic artery aneurysms in the region of chemoembolization. The etiology of aneurysms were presumably drug induced arteritis.     

Polymyalgia rheumatica and temporal arteritis

Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) are closely related and frequently occurring inflammatory diseases with an incidence of 50 and 18 per 100,000 per year, respectively, in people aged 50 years or over. The most frequent symptom of PMR is aching and morning stiffness lasting more than 1 month and exacerbated by movement, occurring in the shoulder and pelvic girdles and in the neck region. GCA is vasculitis of the large and medium-sized arteries that originate from the aortic arch, causing new and marked headache localised over the temporal or occipital areas, jaw claudication, visual impairment or claudication of the arms. GCA is characterised by histopathological panarteritis with a predominantly lymphohistiocytic cell infiltrate. Activation of macrophages is central to the arteritis. Standard treatment for PMR and GCA is glucocorticoids, which may consist of prednisone 10-20 mg/day or its equivalent for PMR patients and prednisone 30-40 mg to 1 mg/kg body weight for GCA patients. For GCA patients with recently impaired vision, treatment should start with high doses of intravenously administered glucocorticoids, such as methylprednisolone 1 g/day for 3 consecutive days. A treatment duration of 1-2 years is often required for patients with PMR or GCA; because of the side effects associated with long-term use of glucocorticoids, osteoporosis prophylaxis with oral calcium supplementation, vitamin D and bisphosphonates is appropriate.     

How to perform a temporal artery biopsy

Temporal artery biopsy is performed to confirm the diagnosis of giant cell arteritis. The technique is described together with the anatomy of the superficial temporal artery.     

An autopsy case of well differentiated leiomyosarcoma of the jejunum

A 54-year-old man had a leiomyosarcoma of the jejunum, 8 X 6 X 5 cm in size resected, on December 17, 1982 and died of liver metastases with hemorrhagic ascites (4,000 ml) on July 5, 1984. The malignant potential of a smooth muscle tumor based on the mitotic index of tumor cells has been stressed for many years. However, our biologically malignant autopsy case showed not only an exceptionally low mitotic index in tumor cells of the surgical specimen but also in those of the autopsy specimen. In this report, the fact that the degree of mitotic figures sometimes fail to indicate biological behavior is discussed.