Abstract：
UNASSIGNED: A 50-year-old woman presented a dry syndrome, joint pain, inflammatory syndrome, polyclonal hypergammaglobulinemia, and tubulointerstitial nephritis. Imaging studies (including FDG PET/CT) revealed infrarenal retroperitoneal fibrosis with periaortitis and hypermetabolic osteosclerotic lesions. Bone scintigraphy demonstrated intense uptake in the femoral, tibial, and radial regions, suggestive of non-Langerhans histiocytosis, specifically Erdheim-Chester disease. A bone biopsy confirmed the presence of IgG4-positive plasma cells but no histiocytes. The patient received corticosteroid therapy followed by rituximab, resulting in a complete response. This case suggests an atypical manifestation of bone lesions in IgG4-related disease, emphasizing the diagnostic challenge between IgG4-related disease and Erdheim-Chester disease.
摘要：
■一名50岁的妇女出现干燥综合征,关节痛,炎症综合征,多克隆高丙种球蛋白血症，和肾小管间质性肾炎。影像学检查（包括FDGPET/CT）显示肾下腹膜后纤维化伴主动脉周围炎和高代谢性骨硬化病变。骨闪烁显像显示股骨强烈摄取，胫骨,和径向区域，提示非朗格汉斯组织细胞增生症，特别是Erdheim-Chester病.骨活检证实存在IgG4阳性浆细胞但不存在组织细胞。患者接受皮质类固醇治疗，随后接受利妥昔单抗，导致一个完整的响应。此病例提示IgG4相关疾病的骨病变的不典型表现,强调IgG4相关疾病和Erdheim-Chester病之间的诊断挑战。
