Division of Rheumatology and Clinical Immunology, Department of Medicine, Jichi Medical University, Tochigi, Japan.
Department of Diagnostic Pathology, Jichi Medical University, Tochigi, Japan.
Rheumatol Int. 2019 May;39(5):901-909. doi: 10.1007/s00296-019-04255-2. Epub 2019 Feb 21.
The objective was to investigate the clinical and histological features of liver dysfunction in patients with polymyositis (PM) or dermatomyositis (DM).A total of 115 patients (38 with PM and 77 with DM), who were admitted to our hospital between 2001 and 2012, were retrospectively reviewed. Liver dysfunction was defined as an alanine transaminase (ALT) level ≥ 60 U/l and a disproportionate ALT elevation relative to the creatine kinase level. The histological findings from liver biopsies were also assessed.The frequencies of liver dysfunction were 3% and 17% in the patients with PM and DM, respectively. Liver dysfunction was not observed in the patients who had malignancies. Among the patients with DM with no malignancies (n = 50), 20% had liver dysfunction, and all of the patients with liver dysfunction were positive for the anti-melanoma differentiation-associated gene 5 (MDA5) antibody. Compared with those in the patients who did not have liver dysfunction, the ALT, alkaline phosphatase, γ-glutamyl transferase, and KL-6 levels were significantly elevated in the patients who had liver dysfunction. Six patients, comprising four with DM and two with PM, underwent liver biopsies, and the common histological findings associated with DM were steatosis, hepatocyte ballooning, increases in the pigmented macrophage numbers, and glycogenated nuclei. Hemophagocytosis was detected in two of three patients with DM who underwent liver biopsies and bone marrow aspirations. In conclusion, Liver dysfunction might be an extramuscular manifestation in patients with DM who are anti-MDA5 antibody-positive. Steatosis and hepatocyte ballooning could be common histological features.
目的是研究多发性肌炎（PM）或皮肌炎（DM）患者肝功能障碍的临床和组织学特征。回顾性分析 2001 年至 2012 年间我院收治的 115 例患者（38 例 PM 和 77 例 DM）。肝功能障碍定义为丙氨酸转氨酶（ALT）水平≥60U/L，且 ALT 升高与肌酸激酶水平不成比例。还评估了肝活检的组织学发现。PM 和 DM 患者的肝功能障碍发生率分别为 3%和 17%。无恶性肿瘤的 DM 患者中未观察到肝功能障碍（n=50），但 20%的患者存在肝功能障碍，且所有肝功能障碍患者均为抗黑色素瘤分化相关基因 5（MDA5）抗体阳性。与无肝功能障碍的患者相比，肝功能障碍患者的 ALT、碱性磷酸酶、γ-谷氨酰转移酶和 KL-6 水平显著升高。6 例患者（4 例 DM 和 2 例 PM）进行了肝活检，与 DM 相关的常见组织学发现为脂肪变性、肝细胞气球样变、色素性巨噬细胞数量增加和糖原核。3 例行肝活检和骨髓抽吸的 DM 患者中检测到噬血细胞现象。总之，肝功能障碍可能是抗 MDA5 抗体阳性的 DM 患者的肌肉外表现。脂肪变性和肝细胞气球样变可能是常见的组织学特征。
