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A case of undifferentiated connective tissue disease: is it a distinct clinical entity?

机译:一例未分化的结缔组织病:它是一个独特的临床实体吗?

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BACKGROUND: In November 2001, a 24-year-old woman with thrombocytopenia and Raynaud's phenomenon presented to our clinic. Her physical examination was unremarkable except for bruising on her legs and arms. INVESTIGATIONS: Laboratory assays detected the presence of antinuclear and anti-ribonucleoprotein antibodies. Tests for antibodies to double-stranded DNA and for antiphospholipid (lupus anticoagulant and anticardiolipin), anticentromere, anti-Scl-70, and antiplatelet antibodies were negative, as was a Coombs test. An echocardiogram, chest X-ray, and abdominal scan showed no abnormalities. Nailfold digital capillaroscopy revealed minor capillary changes not specific for scleroderma. DIAGNOSIS: Undifferentiated connective tissue disease. MANAGEMENT: The patient was successfully treated initially with high doses of corticosteroids and azathioprine, followed by rapid dose tapering. Therapy was continued for 2 years and then stopped. Over the next 4 years the patient's disease history was unremarkable, apart from mild Raynaud's phenomenon of the hands and the presence antinuclear and anti-ribonucleoprotein antibodies. The diagnosis of undifferentiated connective tissue disease was confirmed at her most-recent assessment, in October 2007.
机译:背景:2001年11月,一名24岁的患有血小板减少症和雷诺现象的妇女出现在我们的诊所。她的身体检查无异常,只是腿和胳膊上有瘀伤。调查:实验室检测发现存在抗核和抗核糖核蛋白抗体。抗双链DNA抗体和抗磷脂抗体(狼疮抗凝和抗心磷脂),抗着丝粒,抗Scl-70和抗血小板抗体的检测均为阴性,这与Coombs检测相同。超声心动图,胸部X线和腹部扫描未见异常。针刺数字毛细血管镜检查发现,毛细血管的变化不仅仅局限于硬皮病。诊断:未分化的结缔组织病。处理:该患者最初已成功接受高剂量的皮质类固醇和硫唑嘌呤治疗,随后迅速减量。继续治疗2年,然后停止。在接下来的4年中,除了轻微的雷诺氏手状现象以及存在抗核和抗核糖核蛋白抗体外,患者的病史并不明显。她最近一次评估是在2007年10月,证实了未分化结缔组织疾病的诊断。

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