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Characteristics of 99mTc-MDP Whole-body Bone Scintigraphy in Erdheim-Chester Disease
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摘要:
目的 对Erdheim-Chester病(Erdheim-Chester disease, ECD)的99mTc-亚甲基二膦酸盐(methylene diphosphonate, MDP)全身骨显像特点进行总结, 以期为临床诊疗提供参考。 方法 本研究为描述性分析, 研究对象为2015年1月-2022年12月北京协和医院99mTc-MDP全身骨显像示骨受累的ECD患者, 总结病变累及部位、病变特征并分析骨骼病变评分与常见骨代谢分子标志物的相关性。 结果 共入选44例出现骨受累的ECD患者。99mTc-MDP全身骨显像示, 全身骨骼均可受累, 其中四肢骨(100%, 44/44)、颅骨与颌面骨(84.1%, 37/44)、肢带骨(65.9%, 29/44)受累最为常见, 肋骨与胸骨(38.6%, 17/44)、脊柱(29.5%, 13/44)受累相对少见。除累及部位具有特征性外, 病灶呈双侧对称性分布是ECD患者99mTc-MDP全身骨显像的另一重要特征。其中以四肢骨、颌面骨、锁骨病变呈双侧对称性分布的患者比例较高, 尤其下肢骨受累, 病变均为双侧对称(100%)。四肢骨病变中, 多为骨干和干骺端共同受累, 81.8%(36/44)的患者下肢骨病变形成了以膝关节为中心的对称性干骺端和骨干高摄取表现; 颌面骨病变主要表现为副鼻窦区域局灶性摄取增高, 伴或不伴下颌骨弥漫性摄取增高, 并可出现"林肯征"(61.1%, 22/36)。Spearman相关性分析显示, 骨骼病变评分与血钙、血磷、碱性磷酸酶、甲状旁腺激素等骨代谢分子标志物均无线性相关(P均>0.05)。 结论 ECD可出现全身多处骨骼受累, 其中以四肢骨尤其下肢骨受累最为常见, 并常伴颅骨和颌面骨病变; 膝关节周围干骺端和骨干对称性摄取增高是99mTc-MDP骨显像最常见的特征, 对ECD的诊断及鉴别诊断具有重要参考价值。 -
关键词:
- Erdheim-Chester病 /
- 全身骨显像 /
- 亚甲基二膦酸盐 /
- 病变特征
Abstract:Objective To summarize the features of 99mTc-methylene diphosphonate (MDP) whole-body bone scintigraphy (WBBS) in Erdheim-Chester disease (ECD) patients and to provide insights for clinical diagnosis and treatment. Methods Retrospective analysis was performed in ECD patients with bone lesion who underwent 99mTc-MDP WBBS at Peking Union Medical College Hospital from January 2015 to December 2022.The bone involvement sites and characteristic features in99mTc-MDP WBBS were summarized, and the correlation between bone lesion scores and bone metabolic biomarkers was analyzed. Results A total of 44ECD patients with bone involvement were included in the study.According to the 99mTc-MDP WBBS, the most commonly affected sites were the bones of the extremities (100%, 44/44), skull and maxillofacial bones (84.1%, 37/44), and appendicular bones (65.9%, 29/44), while involvement of the ribs and sternum (38.6%, 17/44) and spine (29.5%, 13/44) were less frequent.Bilateral and symmetric change of the lesions was an important feature of 99mTc-MDP WBBS in ECD.The proportion of patients with bilateral and symmetric bone lesions in the extremities, maxillofacial area, and clavicle was relatively high, particularly in the lower extremities (100%).In extremity bone lesions, there was mostly joint involvement of diaphysis and metaphysis, and 81.8%(36/44) patients demonstrated the typical scintigraphic characteristic: bilateral and symmetrical increased uptake in the distal femur and proximal tibia.The maxillofacial lesions mainly presented with focal uptake increase in the region of the paranasal sinuses, with or without diffuse increased uptake in the mandible-Lincoln sign (61.1%, 22/36).Spearman correlation analysis revealed that the bone metabolic biomarkers such as blood calcium, blood phosphate, alkaline phosphatase, and parathyroid hormone were not linearly correlated with bone lesion scores (all P>0.05). Conclusions ECD can cause multiple bone lesions, with involvement typically observed in the extremities, particularly in the lower extremities.Lesions in the skull and maxillofacial area are also frequently observed.Bilateral and symmetric uptake increase in the distal femur and proximal tibia is the most pathognomonic manifestation of 99mTc-MDP WBBS, providing valuable information for diagnosing and differentiating ECD. 作者贡献：马艳茹负责论文设计及论文撰写；牛娜负责论文修订；曹欣欣、李轶、景红丽、刘宇、陈黎波负责资料收集与影像学阅片。利益冲突：所有作者均声明不存在利益冲突 -
图 1 1例42岁男性ECD患者多饮、多尿7年，言语不清、下肢无力1年余，基线99mTc-MDP全身骨显像示颌面骨弥漫性异常摄取增高，双侧肱骨头、尺骨近段、右侧股骨大转子、双侧股骨远段、胫骨近段和远段异常摄取增高(箭头)，可见多处病灶呈双侧对称性分布
ECD：同表 1；MDP：亚甲基二膦酸盐
表 1 44例ECD患者骨代谢相关实验室检查结果
指标 数值(正常范围) 异常比例[%(n/N)] 血钙(x±s，mmol/L，n=44) 2.18±0.16(2.13~2.7) 9.1(4/44) 血磷(x±s，mmol/L，n=44) 1.31±0.21(0.81~1.45) 0(0/44) 碱性磷酸酶[M(P25, P75), U/L，n=44] 85(66.25，106.8)(35~135)* 9.1(4/44) 甲状旁腺激素[M(P25, P75), ng/L，n=25] 30.80(23.55，41.65)(15.0~65.0) 4.0(1/25) β-胶原降解蛋白[M(P25, P75), μg/L，n=25] 0.93(0.53，1.24)(0.21~0.44) 84.0(21/25) 总25羟维生素D(x±s, μg/L，n=24) 14.19±6.72(≥20) 83.3(20/24) 总Ⅰ型胶原氨基端延长肽[M(P25, P75), μg/L，n=15] 70.30(21.40, 169.50)(15.1~58.6) 60.0(9/15) 骨钙素[M(P25, P75), μg/L，n=12] 1.56(1.103, 1.820)(1.8~8.4) 75.0(9/12) ECD：Erdheim-Chester病；*由于研究时间跨度较大，碱性磷酸酶正常范围进行了多次调整，本文所列为多个正常范围的最大区间 
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表 2 44例ECD患者骨显像分布特点
骨骼 受累例数
[n(%)]分布特点 双侧对称性病变患者
占比[%(n/N)]颅骨与颌面骨 37(84.1) 颅骨 8(18.2) 颅骨弥漫性或局灶性摄取增高 37.5(3/8) 颌面骨 36(81.8) 副鼻窦区域局灶性或弥漫性摄取增高，两侧对称；伴或不伴下颌骨弥漫性摄取增高 94.4(34/36) 脊柱 13(29.5) 单个或多个椎体灶性摄取增高 38.5(5/13) 胸骨与肋骨 17(38.6) 胸骨 10(22.7) 胸骨柄受累为主，可沿骨骼边缘分布 50.0(5/10) 肋骨 8(18.2) 多个肋骨点条状摄取增高 12.5(1/8) 四肢骨 44(100) 股骨 42(95.5) 摄取增高呈双侧对称，以远端增高为主，骨干分布可欠均匀 100(42/42) 胫骨 43(97.7) 摄取增高呈双侧对称，近端和远端均可受累，以近端摄取增高为主 100(43/43) 肱骨 34(77.3) 绝大数病例摄取增高呈双侧对称，以肱骨头和中上段肱骨干摄取增高为主 85.3(29/34) 尺桡骨 32(72.7) 摄取增高双侧基本对称，近端和远端均可受累，摄取增高程度较一致 93.8(30/32) 足部 17(38.6) 以跟骨受累为主，且左右对称 100(17/17) 手部 6(13.6) 以腕关节旁腕骨受累为主，且左右对称 100(6/6) 肢带骨 29(65.9) 锁骨 19(43.2) 呈局灶性或弥漫性摄取增高，可单侧受累 89.5(17/19) 肩胛骨 3(6.8) 呈局灶性摄取增高 0(0/3) 骨盆 12(27.3) 多发生于骶髂关节和髋关节周围，可呈对称性摄取增高 66.7(8/12) ECD：同表 1
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