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.     

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.     

Widespread thrombosis and necrotizing arteritis of the liver in a 69-year-old woman with symptoms simulating those of temporal arteritis

Autopsy findings of widespread thrombosis and necrotizing arteritis of the liver in a 69-year-old woman who had clinical symptoms suggesting those of temporal (giant cell) arteritis were presented. The lesion of the temporal artery was not of temporal arteritis but of thrombosis, part of widespread thrombosis, which occurred in the small arteries having hypertensive arteriolopathy. This case also had an arteritis of the liver resembling polyarteritis nodosa (PN). The pathologic processes demonstrated in this case suggested that the temporal arteritis-like symptoms may be brought about by occlusion of the temporal artery. Temporal arteritis is a nonfatal and self-limited disease and little information has been obtained from autopsy material. Accordingly, it is necessary to collect autopsy cases of temporal arteritis to investigate this disease through observations of whole bodies. Recently we examined a case which appeared clinically having symptoms of temporal arteritis, but the autopsy revealed that the symptoms were not brought about by temporal arteritis but by thrombosis of the temporal artery. It is the purpose of this paper to present this case and to discuss some problems concerning genesis of symptoms of temporal arteritis.     

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.     

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.     

ANEMIA AND INFECTION IN SCHOOL-AGED EGYPTIAN CHILDREN

Iron-deficiency anemia and infection are deterrents to optimal child health in many developing countries. We investigated the prevalence of anemia and elevated erythrocyte sedimentation rate (ESR), which reflects chronic background infection, recent illness, and diet for 190 children aged 10 to 13 years in 1995 in a Nile Delta village. The children exhibited a high prevalence of anemia (61.6%) and elevated ESR (54.9%). One-third of the children reported a minor illness within the past week, and more than 75 per cent had one or more parasites evident in stool or urine samples. There was no relationship of socioeconomic or dietary indicators to anemia or elevated ESR. Mothers' evaluation of the child's health status had no relationship to anemia or elevated ESR, with 43.6 per cent of mothers reporting their child's health status as excellent or good. Maternal report of the child's health as poor was related to recent illness. We speculate that where anemia and chronic infection are hyperendemic, both children and mothers may become habituated to the associated symptoms and consider them normal.     

ANEMIA AND INFECTION IN SCHOOL-AGED EGYPTIAN CHILDREN

Iron-deficiency anemia and infection are deterrents to optimal child health in many developing countries. We investigated the prevalence of anemia and elevated erythrocyte sedimentation rate (ESR), which reflects chronic background infection, recent illness, and diet for 190 children aged 10 to 13 years in 1995 in a Nile Delta village. The children exhibited a high prevalence of anemia (61.6%) and elevated ESR (54.9%). One-third of the children reported a minor illness within the past week, and more than 75 per cent had one or more parasites evident in stool or urine samples. There was no relationship of socioeconomic or dietary indicators to anemia or elevated ESR. Mothers’ evaluation of the child’s health status had no relationship to anemia or elevated ESR, with 43.6 per cent of mothers reporting their child’s health status as excellent or good. Maternal report of the child’s health as poor was related to recent illness. We speculate that where anemia and chronic infection are hyperendemic, both children and mothers may become habituated to the associated symptoms and consider them normal.     

多发大性动脉炎1例报告

多发性大动脉炎是一病因尚不明确的少见动脉性疾病,本文我科就收治的一例患者结合文献报道进行讨论。     

Clinical thinking and decision making in practice. An elderly patient with vertigo and high sedimentation rate

A 77-year-old woman was admitted because of progressive vertigo, nausea and a dysarthric speech disorder. The patient's history of diabetes mellitus, hypertension and hypercholesterolaemia, and the finding of murmurs over peripheral arteries at physical examination led to a presumptive diagnosis of cerebellar ischaemia in the context of generalized atherosclerosis. However, the diagnosis was revised when bilateral cerebellar infarction was demonstrated radiologically, and a biopsy of a temporal artery revealed giant cell arteritis. Despite treatment with prednisone (60 mg daily) the patient's neurological condition deteriorated, and she succumbed several months later to pneumonia. The case illustrates the pitfalls in the diagnostic approach of elderly patients with multiple pathology and it also emphasizes that in an elderly person with high erythrocyte sedimentation rate (> 100 mm in the first hour) temporal arteritis should be ruled out as soon as possible to prevent further neurological damage.     

多发大性动脉炎1例报告

多发性大动脉炎是一病因尚不明确的少见动脉性疾病，本文我科就收治的一例患者结合文献报道进行讨论。     

Surgical Repair of Abdominal Aortic and Renal Artery Aneurysms in Takayasu's Arteritis

Takayasu''s arteritis is a large vessel vasculitis that can be a challenging diagnosis to make and has a varied clinical presentation. Management largely depends on affected vessel disease severity and individual patient considerations. The diagnosis must be considered in a young patient with large vessel aneurysms. We present a case of a 30 year-old woman of Pacific Islander descent who presented to the Tripler Army medical Center Vascular Surgery Department in Honolulu, Hawai‘i seeking repair of her abdominal aortic and renal artery aneurysms prior to conception.A 30 year-old Pacific Islander woman with a history of a saccular abdominal aortic aneurysm and renal artery aneurysms presented to our clinic seeking vascular surgery consultation prior to a planned pregnancy. She had a renal artery stent placed at an outside institution for hypertension. She met the diagnosis of Takayasu''s arteritis by Sharma''s criteria. Physical exam was significant for a palpable, pulsatile, abdominal mass and CT angiography revealed a saccular irregular-appearing infra-renal abdominal aortic aneurysm, extending to the aortic bifurcation, with a maximum diameter of 3.3 cm. A right renal artery aneurysm was also identified proximally, contiguous with the aorta, with a maximal transverse diameter of 1.7 cm. The patient underwent a supraceliac bypass to the right renal artery with a 7mm Dacron graft, as well as excision of the right renal artery aneurysm. The abdominal aortic aneurysm was replaced using a Hemashield Dacron bifurcated 14mm x 7mm bypass graft. Intraoperative measurements of the renal artery aneurysm were 1.5 x 1.5 cm and the saccular appearing distal abdominal aortic aneurysm measured 3.6 x 3.3cm. The patient was discharged from the hospital 7 days post-operatively. At 1-year follow up, CT scan of the abdominal aorta revealed the repair was without any evidence of aneurysm formation, anastomotic pseudoaneurysm formation, or areas of stenosis. She has remained normotensive with a normal serum creatinine 18 months after her repair. She has since delivered her second child.It is rare for Takayasu''s arteritis to present with aneurysmal disease. It is much more common to present with stenosis or occlusion. It has yet to be proven that Takayasu''s truly has a higher incidence in those of Asian descent. Takayasu''s can be a difficult diagnosis to make but can be aided with the use of Sharma''s criteria. Our particular patient posed unique considerations on the effects of the physiology of pregnancy on her aneurysms and repair.Managing the active phases of disease is imperative, and though medical management is first line, surgical intervention may be necessary. Surgical intervention should be performed in a quiescent period of disease if possible given that biological inflammation at the time of intervention increases the complication rate. Repair of aneurysmal disease in a young female should also be considered prior to pregnancy.     

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.     

Three cases of macroprolactinaemia

A woman with hirsutism but otherwise symptom-free was found to have a raised serum prolactin and a pituitary microadenoma. The hyperprolactinaemia persisted despite bromocriptine therapy and subsequent pituitary surgery, which yielded a non-functioning adenoma. After a further 15 years with persistent hyperprolactinaemia but no symptoms, macroprolactinaemia was diagnosed. Such cases might account for part of the failure rate of pituitary microsurgery for prolactinoma. Testing for macroprolactinaemia is advisable in a woman with hyperprolactinaemia, especially if her ovulatory cycle is normal. Two other cases are reported in which macroprolactinaemia was associated with menstrual disturbances and other hormonal effects: in these, treatment with dopamine agonists suppressed the hyperprolactinaemia and restored normal menstrual cycles.     

A woman with fever and a painful hip and lower back

A 66-year-old woman presented with fever and acute pain in her lower back and left hip. She used prednison for arteritis temporalis. Abdominal radiography revealed no signs of free intraperitoneal gas, but instead a radiolucent area below the spleen suggesting free retroperitoneal gas. This was confirmed by CT-scan of the abdomen and surgery, when a diverticulitis of the colon descendens with retroperitoneal perforation was diagnosed.     

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.     

Kawasaki disease in Malta

Kawasaki disease is an acute febrile illness of childhood of unknown origin which may cause coronary arteritis and death. The Maltese incidence of 3.2/100,000 population <5 years of age is similar to that reported in non-Asiatic communities. None of the patients had coronary arteritis or other complications. A significant decline in delay to diagnosis was found, attributed to increased awareness of the disease.     

Diagnostic image (71). A woman with obesity, diabetes mellitus and use of corticosteroids. Mediastinal lipomatosis

In a 69-year-old obese woman on prednisone treatment for temporal arteritis a widened mediastinum on chest X-ray proved to be mediastinal lipomatosis on CT-scan.     

Temporal arteritis. A 14-year epidemiological, clinical and prognostic study.

A total of 136 patients with histologically proven temporal arteritis were seen in the Lothian Region of Scotland in the 14-year period, 1964-77. This study is a review of these cases with particular reference to incidence, age, sex, seasonal occurrence, along with clinical features, general and visual prognosis. It also comments on treatment, the adverse effects thereof, and the differential diagnosis. A seasonal effect significant at the 1 per cent level was established with a peak occurrence in January.     

The significance of body temperature, sedimentation, C-reactive protein, leukocyte count and differential for the diagnosis of infections in an internal medicine emergency department

The value of body temperature, erythrocyte sedimentation rate (ESR), C-reactive protein, white blood cell count and differentiation for the diagnosis of infections in patients in the first aid department of internal medicine, was investigated. Forty-six infections were observed in 260 patients (prevalence 18%). Patients with fever had a fourfold increased chance to have an infection. In a patient with fever and an increased ESR the chance to have an infection was 91%, with fever and a normal ESR 33%. In the absence of fever the chance to have an infection was 14% if the ESR was increased and 7% if normal. White blood cell count and differentiation, like C-reactive protein in addition to body temperature were of no use for the diagnosis of infections.     

Recurrent attacks of angioedema ascribed to the use of estrogen preparations and a pregnancy (hereditary angioedema type 3)

A woman experienced recurrent attacks of angioedema from the age of 17 to 21 years and these appeared to be associated with the use of oestrogens. After stopping the medication her complaints disappeared, but they returned during her first pregnancy. Angioedema is a serious condition, which can lead to acute abdominal symptoms, oedema of the upper respiratory tract and death by asphyxiation. The most well-known cause is hereditary angioedema, an autosomal dominant disorder that is characterized by deficiency of C1 esterase inhibitor (C1-INH). Recently, a new type of hereditary angioedema (type 3) has been reported that occurs exclusively in women and is characterised by oestrogen dependency (both endogenous and exogenous), normal C1-INH concentrations and severe attacks of angioedema, which are clinically indistinguishable from the classic form.