两例IgG4相关疾病的个案报道

Two Case Reports of IgG4-Related Disease

摘要: 目的：探讨IgG4相关疾病的临床表现及诊治要点。方法：回顾分析我院收治的2例IgG4相关疾病的临床资料。结果：例1因咳嗽、胸腔积液入住外院，查CT示腹主动脉周围及肠系膜根部软组织密度占位，双侧输尿管中下段包绕双侧输尿管及肾盂肾盏扩张积液，PET-CT示全身多组淋巴结肿大伴FDG代谢增高，考虑血液系统疾病，恶性淋巴瘤可能。后入住我院，辅助检查提示全血细胞减少、血肌酐升高、血清IgG4及IgE水平升高。淋巴结病理：符合IgG4相关性淋巴结病。治疗上予醋酸泼尼松片30 mg qd口服。2月后随诊，患者血常规及血清肌酐水平在正常范围。例2体检查肌酐165 μmol/L，因疲倦乏力入外院，查血肌酐198 μmol/L，血清IgG4升高，上腹部核磁提示：胆管支架术后观，胰腺萎缩，胰管轻度扩张。入院前查血肌酐440.0 μmol/L，后入住我院，查血清IgG4升高、双肾实质损害，诊断考虑IgG4相关性肾病。治疗上予甲泼尼龙片24 mg qd口服，治疗2周后复查肌酐下降，随访17月，肌酐波动300~400 μmol/L，IgG4持续转阴。结论：IgG4相关疾病临床上可累及多个系统，临床上容易误诊，组织病理及血清IgG4检测有助于病情诊断。

Abstract: Objective: To investigate the clinical manifestations and key points of diagnosis and treatment of IgG4-related disease. Methods: A retrospective analysis of clinical data from two patients with IgG4-related disease admitted to our hospital. Results: Case 1 was admitted to another hospital due to cough and pleural effusion. CT scans revealed soft tissue masses of increased density around the abdominal aorta and at the root of the mesentery, encasement of the middle and lower segments of both ureters, and dilation and hydronephrosis of both ureters and renal pelvis. PET-CT showed generalized lymphadenopathy with increased FDG uptake, suggesting a hematologic disorder, possibly malignant lymphoma. The patient was subsequently admitted to our hospital. Auxiliary tests revealed pancytopenia, elevated serum creatinine, and elevated serum IgG4 and IgE levels. Lymph node pathology was consistent with IgG4-related lymphadenopathy. Treatment consisted of oral prednisone acetate 30 mg once daily. At a follow-up visit two months later, the patient’s complete blood count and serum creatinine levels were within normal ranges. Case 2: During a physical examination, serum creatinine was 165 μmol/L. The patient was admitted to another hospital due to fatigue and weakness, where serum creatinine was 198 μmol/L and serum IgG4 was elevated. An upper abdominal MRI revealed post-biliary stent placement findings, pancreatic atrophy, and mild pancreatic duct dilation. Pre-admission serum creatinine was 440.0 μmol/L. The patient was subsequently admitted to our hospital, where elevated serum IgG4 and bilateral renal parenchymal damage were noted, leading to a diagnosis of IgG4-related nephropathy. Treatment consisted of oral methylprednisolone 24 mg once daily. After 2 weeks of treatment, serum creatinine levels decreased. During a 17-month follow-up, serum creatinine fluctuated between 300 and 400 μmol/L, and serum IgG4 remained negative. Conclusion: IgG4-related disease can clinically affect multiple systems and is prone to misdiagnosis. Histopathological examination and serum IgG4 testing are helpful for diagnosis.