Objective: To summarize the clinical characteristics and treatments of chronic non-bacterial osteomyelitis with autoimmune hepatitis in children. Methods: A child who had chronic non-bacterial osteomyelitis with autoimmune hepatitis was admitted to the Department of Gastroenterology of the Children's Hospital Capital Institute of Pediatrics at April 2022. The clinical data was retrospectively analyzed. Using the keywords of "chronic non-bacterial osteomyelitis""autoimmune hepatitis" in Chinese and English, the literature from database establishment to December 2022 in CNKI, Wanfang, China Biomedical Literature Database and Pubmed was searched. Combined with this case, the clinical characteristics and treatment of chronic non-bacterial osteomyelitis combined with autoimmune hepatitis were analyzed. Results: A 5 years and 3 months girl was admitted to the Department of Gastroenterology of Children's Hospital, Capital Institute of Pediatrics for "transaminase elevated for 1 year and swelling of right maxillofacial area for half a year". The physical examinations at admission found a 4.0 cm × 4.0 cm swelling area with tenderness before the right ear, abdominal distention with visible abdominal wall vein, firm and enlarged liver (10.0 cm below the xiphoid and 4.5 cm below the right ribs), and splenomegaly (Line Ⅰ 10.0 cm, Line Ⅱ 11.5 cm, and Line Ⅲ 25.0 cm). There was no redness, swelling or restriction of the limbs. Laboratory examination found abnormal liver function with alanine aminotransferase 118 U/L, aspartate aminotransferase 227 U/L, γ-glutamyltransferase 360 U/L, and positive direct anti-human globulin test; immunology test found immunoglobulin G 41.60 g/L and a homogeneous type of antinuclear antibody of 1∶1 000; the autoimmune hepatitis antibody test found a positive anti-smooth muscle antibody (1∶100). Liver biopsy showed moderate interfacial inflammation and the patient was diagnosed with autoimmune hepatitis (International Autoimmune Hepatitis Group 19). The imaging findings showed extensive involvement of the bilateral mandible, while the right side was severe. There were expansile bone changes, thinning of the bone cortex, and significant swelling of the surrounding soft tissue in the mandibular body, mandibular angle, and mandibular ramus. After treatment of glucocorticoid, the swelling of the right maxillofacial region disappeared and the transaminase returned to normal. Only one case was reported before in English and none in Chinese. The two cases were both girls whose main clinical features were joint pain and swelling. The previous case started with pain in both knee joints, and developed liver injury during treatment while this case had liver injury as the initial clinical presentation. Besides, the affected sites and degrees of arthritis in the 2 cases were different. After glucocorticoid treatment, the clinical symptoms were alleviated, and transaminases returned to normal. Conclusions: Chronic non bacterial osteomyelitis may involve the liver and manifest as autoimmune hepatitis. Glucocorticoids therapy is effective.
目的： 总结慢性非细菌性骨髓炎合并自身免疫性肝炎患儿的临床特点及治疗效果。 方法： 回顾性分析2022年4月首都儿科研究所附属儿童医院消化内科收治的1例慢性无菌性骨髓炎合并自身免疫性肝炎患儿的临床资料。以“慢性非细菌性骨髓炎”“自身免疫性肝炎”“chronic non-bacterial osteomyelitis”“autoimmune hepatitis”为关键词分别在中国知网、万方数据库、中国生物医学文献数据库、Pubmed数据库进行检索（建库至2022年12月），结合本例资料，对以肝损伤为首发表现，慢性非细菌性骨髓炎合并自身免疫性肝炎患儿的临床特点及药物治疗分析进行总结。 结果： 患儿，女，5岁3月龄，因“发现转氨酶升高1年，右侧颌面肿胀半年”入院。入院体格检查可见右耳前面部肿胀，大小约4.0 cm×4.0 cm，触痛阳性，腹部膨隆，见腹壁静脉，肝大，质硬，剑突下10.0 cm，右肋下4.5 cm，脾大，Ⅰ线10.0 cm，Ⅱ线11.5 cm，Ⅲ线25.0 cm，质硬。四肢各关节无红肿及活动受限。辅助检查可见肝损伤表现及免疫指标异常，丙氨酸转氨酶118 U/L，天冬氨酸转氨酶227 U/L，γ谷氨酰转移酶360 U/L，直接抗人球蛋白试验阳性；免疫球蛋白IgG 41.60 g/L；抗核抗体阳性（1∶1 000，均质型），余阴性，自身免疫性肝炎抗体示抗平滑肌抗体阳性（1∶100）；肝脏组织穿刺活检示中度界面炎，患儿转氨酶升高、肝脏肿大，自身抗体及抗平滑肌抗体阳性、肝脏组织病理存在界面性肝炎，除外其他可能的病毒性肝炎，符合自身免疫性肝炎的诊断标准（简化的国际自身免疫性肝炎评分系统 19分）；影像学示双侧下颌骨受累广泛，但右侧更重，下颌体、下颌角、下颌支均有病变，呈膨胀性骨改变，骨皮质变薄，周围软组织明显肿胀。本例患儿加用糖皮质激素治疗后右侧颌面肿胀消失，监测转氨酶指标恢复正常。文献复习符合检索条件中文文献0篇、英文文献1篇，共1例相关病例报道，结合本例患儿共2例，均为女性，临床特点均有关节疼痛、肿胀。本例为以肝损伤为首发表现，文献报道1例为以双膝关节疼痛起病，药物治疗后期发现肝损伤。2例患儿关节受累部位及程度各不相同，但加用糖皮质激素治疗，临床症状均缓解，转氨酶恢复正常。 结论： 慢性非细菌性骨髓炎可以合并肝脏受累，表现为自身免疫性肝炎，激素治疗有效。.