Barbosa Orivaldo Alves, Andrade Talita Guimarães, Simões Sergio Saldanha Freire, Macedo Andre Luis Coutinho de Araújo, Barroso Dower Frota, Fontenele João Paulo Uchoa, Correia José Walter
Department of Internal Medicine, General Hospital Dr. César Cals, Avenida do Imperador, 545, Centro, Fortaleza, Ceará, Brazil.
Internal Medicine Residency Program, General Hospital Dr. César Cals, Avenida do Imperador, 545, Centro, Fortaleza, Ceará, Brazil.
Case Reports Immunol. 2025 Aug 28;2025:6543528. doi: 10.1155/crii/6543528. eCollection 2025.
IgG4-related disease (IgG4-RD) is a rare, progressive, and immune-mediated fibroinflammatory disorder that primarily affects middle-aged men and is more prevalent in Asian populations. Although extensively studied, its pathophysiology remains incompletely understood. This case report describes a 44-year-old male presenting with multiple abscesses and progressive inflammatory symptoms, ultimately diagnosed with IgG4-RD with musculoskeletal involvement. Imaging and histopathological evaluation confirmed osteolytic lesions and significant IgG4-positive plasma cell infiltration. Soft tissue tumors in IgG4-RD are exceptionally rare, further emphasizing the uniqueness of this case. The patient showed clinical improvement with corticosteroid therapy. This case highlights the importance of considering IgG4-RD in the differential diagnosis of soft tissue and bone lesions and underscores the need for a multidisciplinary diagnostic approach.
IgG4相关性疾病（IgG4-RD）是一种罕见的、进行性的、免疫介导的纤维炎症性疾病，主要影响中年男性，在亚洲人群中更为常见。尽管已进行了广泛研究，但其病理生理学仍未完全明确。本病例报告描述了一名44岁男性，表现为多处脓肿和进行性炎症症状，最终被诊断为累及肌肉骨骼的IgG4-RD。影像学和组织病理学评估证实存在溶骨性病变以及显著的IgG4阳性浆细胞浸润。IgG4-RD中的软组织肿瘤极为罕见，进一步凸显了该病例的独特性。患者接受皮质类固醇治疗后临床症状改善。本病例强调了在软组织和骨病变的鉴别诊断中考虑IgG4-RD的重要性，并突出了多学科诊断方法的必要性。
