著者

仁科 有美子 土屋 貴彦 青木 正紀 山上 賢治 早川 純子 金子 菜穂 西成田 進

出版者

一般社団法人 日本臨床リウマチ学会

雑誌

臨床リウマチ (ISSN:09148760)

巻号頁・発行日

vol.21, no.2, pp.169-173, 2009-06-30 (Released:2016-03-31)

参考文献数

11

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We report a case of 79-year-old woman with an inflammatory aneurysm occurred in a thoracic aorta. The patient was admitted to our medical center because of continuous pyrexia of unknown origin. A marked elevation of serum C-reactive protein level was observed. Infections, collagen vascular diseases and neoplastic disease including malignant lymphoma were found to be unlikely by hematologic, serologic and radiographic examinations. Autoantibodies including antineutrophil antibodies were undetectable. Only enhanced-computed tomography revealed an abnormal finding, so-called mantle core sign, in thoracic aorta, which is specific for inflammatory aortic aneurysm. In general, the disease occurs in abdominal aorta, and the involvement of thoracic aorta like this case is rare. Usually, an aortic aneurysm occurs based on atherosclerotic change of blood vessels, and the aspects of an inflammation of large vessels have been focused recently at the pathological findings, the cytokine profiles and the immunological abnormalities. Thus, the differential diagnosis of the disease from Takayasu arteritis, a prototype of a large vessel vasculitis, is often difficult. The diagnostic procedure to differentiate from the other large vessel vasculitis, Takayasu arteritis and atherosclerotic diseases in abdominal aorta, is discussed. The patient was given oral prednisolone which lead to favorable outcome.

著者

齊藤 美佐子 青木 正紀 金子 菜穂 早川 純子 西成田 進

出版者

一般社団法人 日本臨床リウマチ学会

雑誌

臨床リウマチ (ISSN:09148760)

巻号頁・発行日

vol.19, no.1, pp.61-67, 2007-03-30 (Released:2016-12-30)

参考文献数

17

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A 64-year-old man was diagnosed with MPO-ANCA-related systemic vasculitis in March, 2004, because of multiple mononeuropathies, an interstitial pneumonia, a rapidly-progressive glomerulonephritis which was demonstrated by a biopsied-specimen from kidney. High dose intraveneous methylprednisolne bolus-therapy following an oral prednisolone were transiently effective. In August, 2004, the serum level of MPO-ANCA elevated again, and a macular purpura appeared in both arms. A hundred mg/day of oral cyclophosphamide was added to the prednisolone. In February, 2005, he was re-admitted to our hospital because of muscle weakness of extremities mainly in the left arm and leg. Laboratory examination showed a pancytopenia, high serum levels of ferritin (more than 3000μg/ml) and of MPO-ANCA. Bone marrow-aspirated smear showed a number of hemophagocytosis by macrophages. His general status worsened with continuous pyrexia and a decreased level of consciousness. He died in April, 2005, in spite of the treatments including several antibiotics and anti-fungal agents. An abscess formation in the right frontal lobe demonstrated by the autopsy seemed to be responsible for his final outcome. Histologically, the narrowing of lumen and thickening of small vessels in several organs such as kidneys and lungs were observed. In this case, the serum levels of cytokines such as INF-γ, TNF-α, which play an important role for the pathogenesis of hemophagocytic syndrome were undetectable. On the other hand, the serum levels of IL-6, IL-10 and soluble IL-2 receptor were high. These cytokine profiles were compatible with those in compensatory anti-inflammatory response syndrome (CARS) which reflects a cachexic condition but not systemic vasculitis nor hemophagocytic syndrome.