以十二指肠溃疡为突出表现的IgG4相关性疾病1例

A case of duodenal ulcer as prominent manifestation of IgG4-related disease

报告1例以十二指肠溃疡为主要表现的IgG4相关性疾病,以提高对IgG4相关性疾病的认识。患者为70岁男性,因间断皮肤瘙痒、腹痛4年,伴黑便2月余入院。患者4年前因全身瘙痒、腹痛就诊外院,检查血IgG43.09 g/L(参考值0~1.35 g/L)、谷丙转氨酶554 U/L(参考值9~40 U/L)、谷草转氨酶288 U/L(参考值5~40 U/L)、总胆红素54.16μmol/L(参考值2~21μmol/L)、直接胆红素29.64μmol(参考值1.7~8.1μmol/L),均明显升高。腹部CT和磁共振胰胆管造影检查显示胰头及胰尾肿胀,胆总管狭窄,肝内外胆管扩张。诊断IgG4相关性疾病,予醋酸泼尼松40 mg,每日1次。治疗后患者皮肤瘙痒及黄疸消退,后患者停药。2个月前患者出现黑便,血常规提示重度贫血,诊断消化道出血。在外院治疗未见好转来北京医院急诊,胃镜显示十二指肠球部1.5 cm溃疡,给予奥美拉唑治疗后复查仍便潜血阳性。检查正电子发射计算机断层扫描(positron emission tomography-CT,PET-CT)提示十二指肠壁的代谢活性未见明确异常,未发现肿瘤病变,考虑IgG4相关性疾病,为进一步诊治收入北京医院风湿免疫科。患者既往有右侧颌下腺肿物切除术及糖尿病。入院后检查血IgG45.44 g/L(参考值0.03~2.01 g/L),腹部增强CT可见胰腺轻度肿胀及异常强化,自身免疫性胰腺炎可能;肝内外胆管略扩张,肠系膜上血管周围软组织影,考虑腹膜后纤维化可能。十二指肠球部溃疡活检组织病理可见纤维组织增生及多量淋巴细胞浸润,每高倍镜视野IgG4阳性浆细胞约20~30个,IgG4阳性浆细胞占IgG阳性浆细胞的比例超过40%。诊断为IgG4相关性疾病,给予甲泼尼龙琥珀酸钠40 mg静脉滴注,每日1次,持续2周,后改为口服醋酸泼尼松50 mg,每日1次,逐渐减量至醋酸泼尼松5 mg维持,同时联合环磷酰胺0.4 g,静脉滴注,每2周1次,复查患者十二指肠溃疡愈合。IgG4相关性疾病是一种较罕见的免疫介导的慢性炎症伴纤维化疾病,可累及全身多个器官和系统,临床表现复杂多样。IgG4相关性疾病的消化系统表现多为急性胰腺炎和胆管炎,罕见表现为消化道溃疡。该病例证实IgG4相关性疾病可以表现为十二指肠溃疡,是十二指肠溃疡的罕见原因之一。

SUMMARY A case of IgG4-related disease presented with a duodenal ulcer to improve the understan-ding of IgG4-related diseases was reported.A 70-year-old male presented with cutaneous pruritus and abdominal pain for four years and blackened stools for two months.Four years ago,the patient went to hospital for cutaneous pruritus and abdominal pain.Serum IgG4 was 3.09 g/L(reference value 0-1.35 g/L),alanine aminotransferase 554 U/L(reference value 9-40 U/L),aspartate aminotransferase 288 U/L(reference value 5-40 U/L),total bilirubin 54.16μmol/L(reference value 2-21μmol/L),and direct bilirubin 29.64μmol/L(reference value 1.7-8.1μmol/L)were all elevated.The abdominal CT scan and magnetic resonance cholangiopancreatography indicated pancreatic swelling,common bile duct stenosis,and secondary obstructive dilation of the biliary system.The patient was diagnosed with IgG4-related disease and treated with prednisone at 40 mg daily.As jaundice and abdominal pain improved,prednisone was gradually reduced to medication discontinuation.Two months ago,the patient developed melena,whose blood routine test showed severe anemia,and gastrointestinal bleeding was diagnosed.The patient came to the emergency department of Beijing Hospital with no improvement after treatment in other hospitals.Gastroscopy revealed a 1.5 cm firm duodenal bulb ulcer.After treatment with omeprazole,the fecal occult blood was still positive.The PET-CT examination was performed,and it revealed no abnormality in the metabolic activity of the duodenal wall,and no neoplastic lesions were found.IgG4-related disease was considered,and the patient was admitted to the Department of Rheumatology and Immunology of Beijing Hospital for further diagnosis and treatment.The patient had a right submandibular gland mass resection history and diabetes mellitus.After the patient was admitted to the hospital,the blood test was reevaluated.The serum IgG4 was elevated at 5.44 g/L(reference value 0.03-2.01 g/L).Enhanced CT of the abdomen showed that the pancreas was mild swelling and was abnormally strengthened,with intrahepatic and extrahepatic bile duct dilation and soft tissue around the superior mesenteric vessels.We pathologically reevaluated and stained biopsy specimens of duodenal bulbs for IgG and IgG4.Immunohistochemical staining revealed remarkable infiltration of IgG4-positive plasma cells into duodenal tissue,the number of IgG4-positive cells was 20-30 cells per high-powered field,and the ratio of IgG4/IgG-positive plasma cells was more than 40%.The patient was treated with intravenous methylprednisolone at 40 mg daily dosage and cyclophosphamide,and then the duodenal ulcer was healed.IgG4 related disease is an immune-medicated rare disease characterized by chronic inflammation and fibrosis.It is a systemic disease that affects nearly every anatomic site of the body,usually involving multiple organs and diverse clinical manifestations.The digestive system manifestations of IgG4-related disease are mostly acute pancreatitis and cholangitis and rarely manifest as gastrointestinal ulcers.This case confirms that IgG4-related disease can present as a duodenal ulcer and is one of the rare causes of duodenal ulcers.