Aung Thanda, Celestin Mia, Bau Sherona, Saab Sammy
Rheumatology, University of California Los Angeles David Geffen School of Medicine, Los Angeles, USA.
Hepatology, University of California Los Angeles David Geffen School of Medicine, Los Angeles, USA.
Cureus. 2024 Dec 19;16(12):e76018. doi: 10.7759/cureus.76018. eCollection 2024 Dec.
Chylous ascites occur when the lymphatic flow is blocked or disrupted, causing a leakage of fluid into the peritoneal space. It can be caused by a number of etiologies and identifying the exact cause can be challenging. We present the case of a 77-year-old man who presented with chylous ascites. An abdominal computed tomography scan revealed an abnormal spiculated mass and lymph nodes in the central mesentery encasing the pancreas, raising suspicion of pancreatic malignancy. A subsequent mesenteric biopsy indicated sclerosing mesenteritis characterized by dense fibrous tissue and positive Immunoglobulin G4 (IgG4) staining, leading to a final diagnosis of IgG4-related disease (IgG4-RD). The patient responded positively to a treatment regimen that included systemic steroids and rituximab. This case, with its atypical presentation of IgG4-RD, contributes to our understanding of this rare disease, providing valuable insights for future diagnosis and treatment.
当淋巴液流动受阻或中断，导致液体漏入腹腔时，就会发生乳糜性腹水。它可能由多种病因引起，确定确切病因可能具有挑战性。我们报告一例77岁男性乳糜性腹水病例。腹部计算机断层扫描显示中央肠系膜有一个异常的毛刺状肿块和淋巴结，包绕胰腺，怀疑为胰腺恶性肿瘤。随后的肠系膜活检显示为硬化性肠系膜炎症，其特征为致密纤维组织和免疫球蛋白G4（IgG4）染色阳性，最终诊断为IgG4相关疾病（IgG4-RD）。患者对包括全身用类固醇和利妥昔单抗在内的治疗方案反应良好。该病例IgG4-RD表现不典型，有助于我们对这种罕见疾病的理解，为未来的诊断和治疗提供了有价值的见解。
