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New Case of Anti-Synthetase Syndrome Associated with Cirrhosis Post HVB

机译:HVB后肝硬化相关的抗合成酶综合症新病例

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The anti-synthetase syndrome is represented by the association of in-flammatory myopathy (polymyositis/dermatomyositis), interstitial pneu-monitis, Raynaud’s syndrome, inflammatory polyarthritis, and characteristic skin abnormalities. We found also a clinical aspects of Mechanic’s hand with high level of anti-synthetases. We report the case of a 66-year-old patient with polyar- thralgias of large and small inflammatory joints, myositis, Raynaud’s phenomenon, and cutaneous involvement of the hands and feet, with fissure hyperkeratosis feet and an appearance of mechanic’s hands. The patient had an inflammatory syndrome, elevated muscle enzymes, positivity of anti-Jo1 antibodies, PID at chest CT and the presence of inflammatory mononuclear cell infiltrates at muscle biopsy. The evolution under immunosuppressive treatment combining corticosteroids and methotrexate was marked by an oedemato-ascitic decompensation of a post-HVB cirrhosis unknown.
机译:抗合成酶综合症表现为炎症性肌病(多发性肌炎/皮肌炎),间质性肺炎,雷诺氏综合症,炎性多关节炎和特征性皮肤异常。我们还发现了机械师的手具有高水平的抗合成酶的临床方面。我们报道了一名66岁的患者,该患者患有大小不一的炎性关节多发性关节痛,肌炎,雷诺现象以及手脚皮肤受累,脚出现角化性角化过度和机械手的外观。该患者患有炎症综合症,肌肉酶升高,抗Jo1抗体阳性,胸部CT上的PID和肌肉活检中存在炎性单核细胞浸润。联合应用皮质类固醇和甲氨蝶呤的免疫抑制治疗下的演变,其特征在于HVB后肝硬化的未知的水肿性腹水代偿失调。

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