Badshah Maaz Bin, Khan Qaisar Ali, Kazi Naima, Ansari Rabia Aslam, Verma Ravina
Shifa International Hospital, Islamabad, Pakistan.
Khyber Teaching Hospital, MTI KTH, Peshawar, Pakistan.
Ann Med Surg (Lond). 2025 Jan 9;87(1):36-42. doi: 10.1097/MS9.0000000000002749. eCollection 2025 Jan.
IgG4-related disease (IgG4-RD) is a chronic, immune-mediated disorder characterized by widespread inflammation and fibrosis, leading to potential organ dysfunction if untreated. Often underdiagnosed due to subtle and varied symptoms, the disease can affect multiple organ systems. This case series highlights two patients who were diagnosed as cases of IgG4-related retroperitoneal fibrosis.
A total of two patients were included in this prospective case series who presented to the gastroenterology department of a tertiary care hospital with the same signs and symptoms and were diagnosed with IgG4-related retroperitoneal fibrosis (IgG4-RPF).
Two patients were included in our case series, aged 25 and 26 years. The chief complaints included dull, radiating epigastric pain, other symptoms include diffuse abdominal pain, intensified, accompanied by nausea, vomiting, and episodic diarrhea, and a history of B-cell lymphoproliferative disorder. Endoscopic ultrasound (EUS)-)-guided biopsies were performed, showing findings consistent with (IgG4-RPF). Both patients were started on a regimen of prednisolone, pantoprazole, and vitamin D, which they tolerated well without adverse effects. They were advised to follow up with a CT scan after one month.
IgG4-related disease (IgG4-RD) is a rare, chronic condition often presenting as retroperitoneal fibrosis (RPF) and affecting multiple organs. Serum IgG4 levels can be normal in certain cases, histopathological and radiological investigations are necessary for the correct diagnosis. Early initiation of immunosuppressive drugs are necessary for the disease control.
IgG4相关疾病（IgG4-RD）是一种慢性免疫介导性疾病，其特征为广泛的炎症和纤维化，如果不进行治疗，可能导致潜在的器官功能障碍。由于症状细微且多样，该疾病常常诊断不足，可累及多个器官系统。本病例系列重点介绍了两名被诊断为IgG4相关腹膜后纤维化的患者。
本前瞻性病例系列共纳入两名患者，他们因相同的体征和症状就诊于一家三级护理医院的胃肠病科，并被诊断为IgG4相关腹膜后纤维化（IgG4-RPF）。
我们的病例系列纳入了两名患者，年龄分别为25岁和26岁。主要症状包括上腹部钝痛、放射痛，其他症状包括弥漫性腹痛、加重，伴有恶心、呕吐和间歇性腹泻，以及B细胞淋巴增殖性疾病史。进行了内镜超声（EUS）引导下活检，结果与（IgG4-RPF）一致。两名患者均开始使用泼尼松龙、泮托拉唑和维生素D治疗方案，他们耐受良好，未出现不良反应。建议他们在1个月后进行CT扫描随访。
IgG4相关疾病（IgG4-RD）是一种罕见的慢性疾病，常表现为腹膜后纤维化（RPF）并累及多个器官。在某些情况下，血清IgG4水平可能正常，正确诊断需要组织病理学和影像学检查。早期启动免疫抑制药物对于疾病控制是必要的。
