Cases reported "Arteritis"

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1/32. A case of pulmonary arteritis with stenosis of the main pulmonary arteries with positive myeloperoxidase-antineutrophil cytoplasmic autoantibodies.

    A 53-year-old woman was referred to our hospital with the main symptoms of productive cough, fever and exertional dyspnoea. Chest X-ray revealed enlargement of the left hilar shadow and cavitary infiltration in the right upper lobe. 99mTechnetium-macroaggregated albumin (99mTc-MAA) perfusion scintigram showed complete hypoperfusion through the entire right lung. A pulmonary angiogram revealed stenotic lesions in the right and left main pulmonary arteries. Right cardiac catheterization showed an elevated right ventricular systolic pressure. There was no evidence of systemic arterial lesions nor vasculitis. The patient was positive for myeloperoxidase (MPO)-antineutrophil cytoplasmic autoantibodies (ANCA) (168 EU). The mycobacterium avium complex sputum culture was positive. The pulmonary stenotic lesions were surgically resected. The resected pulmonary arterial lesions were pathologically diagnosed as non-specific vasculitis. The cavitary lesion disappeared 6 months after the surgery. Two years after the surgery, although the MPO-ANCA level had decreased to 12 EU, stenosis of the pulmonary arteries reappeared. It is suggested that the patient became positive for MPO-ANCA in association with the mycobacterium avium complex infection, and that the presence of MPO-ANCA may not be related to the development of pulmonary stenosis of the main pulmonary arteries.
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2/32. Arteritis and brachial plexus neuropathy as delayed complications of radiation therapy.

    radiation-induced arteritis of large vessels and brachial plexus neuropathy are uncommon delayed complications of local radiation therapy. We describe a 66-year-old woman with right arm discomfort, weakness, and acrocyanosis that developed 21 years after local radiation for breast adenocarcinoma. Arteriography revealed arteritis, with ulcerated plaque formation at the subclavian-axillary artery junction, consistent with radiation-induced disease, and diffuse irregularity of the axillary artery. electromyography showed a chronic brachial plexopathy. The patient's acrocyanosis, thought to be due to digital embolization from her vascular disease, improved with antiplatelet therapy. The concurrent combination of radiation-induced arteritis and brachial plexopathy is uncommon but should be considered in patients presenting with upper extremity pain or weakness after radiation therapy.
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3/32. Fatal subarachnoid hemorrhage, with brainstem and cerebellar infarction, caused by aspergillus infection after cerebral aneurysm surgery: case report.

    OBJECTIVE AND IMPORTANCE: Intracranial aspergillosis has been reported to cause subarachnoid hemorrhage (SAH) attributable to ruptured mycotic aneurysms. We describe a case of aspergillus arteritis that caused SAH without aneurysm formation, followed by successive brainstem and cerebellar infarction. CLINICAL PRESENTATION: A 50-year-old woman experienced a sudden onset of headache. Computed tomography demonstrated SAH. After angiography revealed an aneurysm of the anterior communicating artery, a complete neck-clipping operation was performed, without neurological deterioration. However, the patient experienced another episode of SAH on the 26th postoperative day. INTERVENTION: We repeated the craniotomy and confirmed that the clip was still intact. A second angiographic evaluation did not reveal an aneurysm or any other cause of hemorrhage. On the 30th postoperative day, magnetic resonance imaging demonstrated cerebellar infarction in the territory of the anteroinferior cerebellar artery. The patient died on the 40th postoperative day, after another episode of SAH and progressive cerebellar and brainstem infarction. The postmortem examination revealed destruction of the basilar artery and occlusion of the basilar and vertebral arteries attributable to aspergillus arteritis. CONCLUSION: When a patient presents with SAH of unknown origin followed by cerebral infarction, aspergillus arteritis should be included in the differential diagnosis. Earlier recognition of this fungal infection improves the prognosis.
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4/32. Large artery involvement in giant cell (temporal) arteritis.

    Of 248 patients with giant cell arteritis, 34 had evidence that the disease affected the aorta or its major branches. Symptoms suggestive of large artery involvement were intermittent claudication of an extremity, paresthesias, and Raynaud's phenomenon. Physical findings included absent or decreased large artery pulses and bruits over large arteries. Four patients presented with decreased upper extremity pulses as the initial manifestation of their arteritis. Nine other patients under treatment for temporal arteritis or polymyalgia rheumatica first developed evidence of large artery involvement as corticosteroid therapy was tapered or discontinued. angiography, performed in 10 patients, was helpful in indicating arteritis rather than atherosclerosis as the cause of large artery disease. Three patients died with aortic rupture, and, at autopsy, widespread giant cell arteritis was found. However, when corticosteroids were given in adequate doses, the response was favorable in most patients; intermittent claudication decreased and the pulses improved.
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5/32. Colonic perforations in systemic lupus erythematosus.

    Five patients with systemic lupus erythematosus (SLE), four of whom died with colonic perforations, are reported. Perforation of the colon constituted the most frequent cause of death among 107 patients with SLE admitted to the Rheumatic disease Unit during a three year period. All five patients with colonic perforation had clinical and laboratory manifestations of active SLE in addition to the abdominal syndrome. Most striking was evidence of active arteritis in all patients with either central nervous system involvement and/or peripheral arteritis, in addition to that found in the gastrointestinal tract. Hyperglobulinemia and rheumatoid factor as well as antinuclear antibodies were present at some time in all patients. The abdominal syndrome was characterized by the insidious onset of lower quadrant pain which was intermittent and colicky. Although direct abdominal tenderness was eventually present in all patients, rebound tenderness and hypoactive bowel sounds were variable and abdominal rigidity occurred only in one patient and late in the course. The differential diagnosis of abdominal pain in SLE is reviewed and possible mechanisms for the production of colonic perforations are discussed. It is suggested that the presence of rheumatoid factors in conjunction with circulating immune complexes may be the pathogenetic mechanism via the production of a mesenteric arteritis.
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6/32. Lethal subarachnoid bleeding under immunosuppressive therapy due to mycotic arteritis.

    A subarachnoid haemorrhage (SAH) occurred 67 days after cardiac transplantation in a 10-year-old girl with consecutive immunocompromising therapy. Neither digital subtraction angiography (DSA) nor computed tomographic angiography showed signs of intracranial vascular malformations. One month before the lethal SAH occurred, she had developed arterial hypertension and attacks of severe headache with cerebrospinal fluid (CSF) pleocytosis while CT scans showed an infarct of the left thalamus. Pathologic findings established the rare diagnosis of SAH due to aspergillosis-related mycotic arteritis. Imaging characteristics are presented.
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7/32. Segmental mediolytic arteritis. Clinicopathologic study and three-dimensional analysis.

    We describe the first recorded case of segmental mediolytic arteritis (SMA) in japan. A 71-year-old Japanese woman developed sudden abdominal pain and went into shock during hospitalization for treatment of valvular heart disease. laparotomy revealed a ruptured pseudoaneurysm of the left gastric artery. Histological examination of the resected artery revealed multiple extensive areas of the dilated wall where the media had partially or totally disappeared with or without the intima. These features were typical of those described previously for SMA (1,2). Three-dimensional analysis of the arterial lesions revealed that the residual media formed branches and cavities down the long axis of the artery, indicating that the major cause of the medial disappearance was arterial dissection. Three months later, the patient died of sepsis. autopsy revealed pseudoaneurysm formation at three different locations in the splenic artery. Histologically, these pseudoaneurysms showed destructive changes in the arterial wall similar to those described above, with organization and thrombosis, and were considered to be the end-stage lesion of SMA. This case is considered to be the fifth adult case of SMA in the literature and the first one showing chronic changes.
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keywords = abdominal pain
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8/32. Berry splenic artery aneurysm rupture in association with segmental arterial mediolysis and portal hypertension.

    A rare case of berry splenic artery aneurysm (SAA) rupture associated with segmental arterial mediolysis (SAM) and portal hypertension is reported. A 66-year-old woman, diagnosed as having liver cirrhosis and portal hypertension 6 years earlier, suddenly developed a lancinating pain in the upper abdomen and lost consciousness. She recovered consciousness while being transferred to hospital by ambulance. During the investigations, her level of consciousness suddenly deteriorated. ultrasonography showed a massive intraperitoneal hemorrhage, and she died 5(1/2) h after admission. On gross examination at autopsy it was not possible to find the rupture point of the vessel because the pancreas was embedded in a massive hematoma. However, careful dissection of the pancreatic tail after fixation revealed a berry aneurysm measuring 0.8 cm in diameter in a branch adjacent to the bifurcation in the distal third of the main splenic artery. Microscopic examination detected a rupture of the aneurysm. The histology of the arterial wall proximal to the aneurysm showed typical SAM. In general, berry SAA caused by SAM is rare and unlikely to rupture. The SAA in the present case likely occurred and ruptured due to the combination of SAM and portal hypertension.
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9/32. An unusual presentation of rheumatoid meningitis.

    BACKGROUND: central nervous system involvement in rheumatoid arthritis can rarely occur in the absence of systemic disease. Rheumatoid meningitis has not been reported to present as spells of neurologic dysfunction. PATIENT AND methods: The authors describe a woman with a history of well-controlled rheumatoid arthritis who presented with headaches and spells of focal neurological dysfunction. brain magnetic resonance imaging, brain biopsy, and temporal artery biopsy were required to make the diagnosis of rheumatoid meningitis with arteritis. RESULTS: neuroimaging revealed abnormal leptomeningeal enhancement. Necrotizing granulomatous inflammation was seen on meningeal and brain biopsy. A temporal artery biopsy showed evidence of arteritis without giant cells. CONCLUSIONS: The possibility of central nervous system involvement by rheumatoid arthritis should be considered in patients with a history of rheumatoid arthritis even in the absence of systemic symptoms. Making the diagnosis may require meningeal and brain biopsy. The condition may be steroid responsive.
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keywords = headache
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10/32. 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.
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