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Adult Onset Stills Disease Associated with Mycoplasma pneumoniae Infection and Hemophagocytic Lymphohistiocytosis

机译:与肺炎支原体感染和吞噬细胞性淋巴组织细胞增多症相关的成人发作性静止病

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摘要

Adult Onset Still's Disease (AOSD) is a systemic inflammatory disorder that can be associated with hemophagocytic lymphohistiocytosis (HLH), a rare but potentially fatal disease of overactive histiocytes and lymphocytes. We present a unique case of AOSD complicated by Mycoplasma pneumonia infection and HLH. A 28-year-old female developed joint pains followed by a diffuse, erythematous, pruritic skin rash that quickly spread throughout the body. The patient deteriorated and developed fever, chills, cough, and dyspnea and had to be intubated. She had hypoalbuminemia, elevated liver enzymes, a very high serum ferritin level, positive anti-Mycoplasma pneumonia IgG and IgM antibodies, and normal rheumatoid factor and anti-nuclear antibodies. The chest X-ray showed diffuse bilateral infiltrates. Bone marrow biopsy revealed hemophagocytosis. The patient was treated with azithromycin, methylprednisolone, and anakinra and was discharged home on cyclosporine and prednisone. This case highlights that patients can develop features of both AOSD and HLH at the beginning of the disease and early diagnosis and treatment increase the likelihood of recovery.
机译:成人发作性静止疾病(AOSD)是一种全身性炎症性疾病,可能与吞噬性淋巴细胞组织细胞增生症(HLH)相关,后者是一种罕见但可能致命的过度活跃的组织细胞和淋巴细胞疾病。我们提出了并发支原体肺炎和HLH的AOSD的独特案例。一名28岁的女性出现关节痛,随后出现弥漫性,红斑,瘙痒性皮疹,并迅速扩散至全身。患者恶化并发展为发烧,发冷,咳嗽和呼吸困难,必须插管。她患有低白蛋白血症,肝酶升高,血清铁蛋白水平很高,抗支原体肺炎IgG和IgM抗体阳性,以及类风湿因子和抗核抗体正常。胸部X线片显示双侧弥漫性浸润。骨髓活检显示吞噬细胞。该患者接受了阿奇霉素,甲基泼尼松龙和烟碱的治疗,并因环孢素和泼尼松而出院。该病例表明,患者在疾病开始时可同时出现AOSD和HLH的特征,早期诊断和治疗增加了康复的可能性。

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