PDF(Pubmed)
Abstract：
BACKGROUND: Macrophage activation syndrome is a rare disorder leading to unregulated immune activity manifesting with nonspecific constitutional symptoms, laboratory abnormalities, and multiorgan involvement. We report the case of a patient who presented with acute hepatitis secondary to macrophage activation syndrome diagnosed by liver biopsy and successfully treated with intravenous immune globulin, anakinra, and rituximab.
METHODS: A 42-year-old Laotian woman with adult-onset immunodeficiency with anti-interferon gamma antibodies presented with a fever, headache, generalized myalgia, dark urine, and reduced appetite in the setting of family members at home with similar symptoms. Her laboratory workup was notable for evidence of acute hepatitis without acute liver failure. After an unrevealing comprehensive infectious and noninvasive rheumatologic workup was completed, a liver biopsy was performed ultimately revealing the diagnosis of macrophage activation syndrome. She was successfully treated with intravenous immune globulin, anakinra, and rituximab.
CONCLUSIONS: This case highlights the importance of maintaining macrophage activation syndrome on the differential of a patient with acute hepatitis of unknown etiology in the correct clinical context and the value of a liver biopsy in making a diagnosis when noninvasive testing is unrevealing.
METHODS: A 42-year-old Laotian woman with adult-onset immunodeficiency with anti-interferon gamma antibodies presented with a fever, headache, generalized myalgia, dark urine, and reduced appetite in the setting of family members at home with similar symptoms. Her laboratory workup was notable for evidence of acute hepatitis without acute liver failure. After an unrevealing comprehensive infectious and noninvasive rheumatologic workup was completed, a liver biopsy was performed ultimately revealing the diagnosis of macrophage activation syndrome. She was successfully treated with intravenous immune globulin, anakinra, and rituximab.
CONCLUSIONS: This case highlights the importance of maintaining macrophage activation syndrome on the differential of a patient with acute hepatitis of unknown etiology in the correct clinical context and the value of a liver biopsy in making a diagnosis when noninvasive testing is unrevealing.
摘要：
背景：巨噬细胞激活综合征是一种罕见的疾病，导致免疫活性失调，表现为非特异性体质症状，实验室异常，和多器官参与。我们报告了一例患者，该患者通过肝活检诊断为继发于巨噬细胞活化综合征的急性肝炎，并成功地用静脉免疫球蛋白治疗。anakinra,还有利妥昔单抗.
方法：一名42岁的老挝妇女，患有成年免疫缺陷，抗干扰素γ抗体，并伴有发烧，头痛,全身肌痛,深色尿液,在家中有类似症状的家庭成员的情况下，食欲下降。她的实验室检查值得注意的是没有急性肝功能衰竭的急性肝炎的证据。在完成未揭示的全面感染性和非侵入性风湿病检查后，进行肝活检,最终诊断为巨噬细胞活化综合征.她成功地用静脉注射免疫球蛋白治疗,anakinra,还有利妥昔单抗.
结论：该病例强调了在正确的临床背景下，维持巨噬细胞活化综合征对鉴别病因不明的急性肝炎患者的重要性，以及在无创性检测未发现时，肝活检在诊断中的价值。
方法：一名42岁的老挝妇女，患有成年免疫缺陷，抗干扰素γ抗体，并伴有发烧，头痛,全身肌痛,深色尿液,在家中有类似症状的家庭成员的情况下，食欲下降。她的实验室检查值得注意的是没有急性肝功能衰竭的急性肝炎的证据。在完成未揭示的全面感染性和非侵入性风湿病检查后，进行肝活检,最终诊断为巨噬细胞活化综合征.她成功地用静脉注射免疫球蛋白治疗,anakinra,还有利妥昔单抗.
结论：该病例强调了在正确的临床背景下，维持巨噬细胞活化综合征对鉴别病因不明的急性肝炎患者的重要性，以及在无创性检测未发现时，肝活检在诊断中的价值。
