嗜酸粒细胞性胃肠炎临床特点分析

目的探讨嗜酸粒细胞性胃肠炎(eosinophilic gastroenterirtis,EG)患者的临床特点,以提高临床医师对此病的认识,减少误诊和漏诊率。方法回顾性分析确诊的20例EG患者的临床表现、实验室检查、影像学检查、胃肠镜检查、病理学结果及治疗方法和效果等。结果本研究中40.0%的患者有过敏史,25.0%伴支气管哮喘。临床症状主要表现为腹痛、腹泻、腹胀、恶心、呕吐等。85.0%患者伴外周血嗜酸粒细胞增多,100%患者胃肠道黏膜组织的病理学检查见嗜酸性粒细胞浸润。在十二指肠、食管、回盲瓣等处取材,嗜酸性粒细胞的检出率50.0%。对症治疗对于轻症患者有效,激素治疗对于重症患者有效。结论 EG是过敏反应相关性疾病,其临床表现和内镜表现均无特异性,外周血和胃肠道黏膜的病理学检查中检测到嗜酸性粒细胞的增多是明确诊断的关键。内镜下在十二指肠、食管、回盲瓣取活检组织可提高诊断率。根据患者病情轻重选择不同治疗方法。     

嗜酸性粒细胞性胃肠炎的临床诊治分析

[目的]探讨嗜酸性粒细胞性胃肠炎患者的临床表现及诊治。[方法]回顾性分析21例嗜酸性粒细胞性胃肠炎患者的分型、临床表现、实验室检查、特殊检查和临床疗效等指标。[结果]21例嗜酸性粒细胞性胃肠炎患者均有不同程度的嗜酸性粒细胞计数和百分比增高。临床分型:黏膜型17例、浆膜型4例、肌型0例。胃镜活检部位为十二指肠降部3例,十二指肠球部11例和胃窦部4例。肠镜活检部位为降结肠2例,横结肠1例。病理学检查提示所有病理标本均有嗜酸性粒细胞浸润。对于激素(强的松)治疗反应良好。[结论]嗜酸性粒细胞性胃肠炎患者主要的临床表现为血液和消化道组织中嗜酸性粒细胞性增多,缺乏特异性内镜表现,强的松治疗反应良好。     

嗜酸性粒细胞性胃肠炎14例临床分析

目的分析嗜酸性粒细胞胃肠炎(eosinophilic gastroenteritis,EG)的临床特点、诊断要点、治疗和预后。方法对14例诊断为嗜酸粒细胞性胃肠炎患者的病史、临床表现、实验室检查、内镜检查、病理结果、治疗及随访情况进行回顾性临床分析。结果 14例EG患者中,10例病变范围涉及黏膜层,1例病变范围涉及肌层,3例病变范围涉及浆膜层。所有患者均出现不同程度的胃肠道症状,内镜下黏膜活检或手术活检均证实胃肠道组织有较多量嗜酸性粒细胞浸润。给予糖皮质激素类治疗的9例患者,1～2周后症状得到明显缓解,且嗜酸性粒细胞数逐渐下降至正常水平。14例患者均预后良好。结论嗜酸性粒细胞性胃肠炎是临床上较为少见的一种疾病,病因和发病机制尚不十分明确。内镜或手术取到活体组织中提示较多嗜酸性粒细胞浸润是确诊的关键。糖皮质激素是主要的治疗药物。该疾病预后良好。     

间接血凝和免疫荧光试验诊断贾第虫病的比较

诊断人体贾第虫病必须从粪便、肠的活检或十二指肠引流液中找到贾第虫的胞囊或滋养体。有人设想用贾第虫胞囊为抗原做免疫荧光试验作为诊断方法,但是至今尚未用过滋养体抗原。随着小鼠动物模型的建立,从感染后的小鼠体内取得大量贾第虫滋养体已有可能。本文的目的是在于对用滋养体抗原作血凝试验和胞囊抗原作免疫荧光试验诊断贾第虫感染的评价。     

20例嗜酸性粒细胞性胃肠炎临床及内镜特点分析

目的 探讨嗜酸性粒细胞性胃肠炎(EG)患者的临床表现及内镜检查对该病的诊治价值.方法 对20例EG患者的临床特点、试验室检查、内镜表现和治疗随诊情况进行分析.结果 20例患者中黏膜型12例、浆膜型2例、混合型6例;EG临床表现以腹痛为主,可伴有腹胀、腹泻、腹水、恶心、呕吐;外周血和骨髓中嗜酸性粒细胞计数明显增多(13.5%～50.6%和7.8%～38.5%).腹水中可见大量嗜酸性粒细胞;内镜表现为黏膜充血水肿、糜烂,病理检查可见大量嗜酸性粒细胞浸润;糖皮质激素治疗1～2周内可迅速缓解症状,减量维持,然后逐渐减量至停药;病情可反复,但预后良好.结论 EG患者临床和内镜表现无特异性,外周血和腹水中嗜酸性粒细胞计数明显增多,胃肠黏膜组织中嗜酸性粒细胞浸润是诊断的关键,糖皮质激素治疗效果良好.     

十二指肠粪类圆线虫病的临床、病理及内镜特征

目的探讨十二指肠粪类圆线虫病的临床、病理组织学及内镜特点。方法回顾性分析珠海市人民医院诊治的1例十二指肠粪类圆线虫病的临床资料、实验室检查、内镜检查、病理组织学检查及治疗、随访情况并复习相关文献。结果患者男,40岁,右上腹阵发性疼痛、腹胀1年余,伴恶心、乏力及体质量减轻。慢性乙型病毒性肝炎病史30余年。血常规示:嗜酸性粒细胞计数及百分比升高。上消化道内镜检查示:十二指肠黏膜弥漫性充血水肿,皱襞肿胀,可见白色绒毛。组织病理学检查示:十二指肠黏膜慢性炎,伴嗜酸性粒细胞浸润,隐窝内及上皮内可见粪类圆线虫虫体及虫卵。最终诊断为十二指肠粪类圆线虫病。经阿苯达唑治疗后症状消失,随访10个月,无明显不适。结论粪类圆线虫病的早期正确诊断对治疗有重要指导意义。除粪便检查外,上消化道内镜检查并活检行组织学检查是一种准确的诊断方法。     

埃索美拉唑和替普瑞酮对功能性消化不良影响的对比研究

目的探讨埃索美拉唑和替普瑞酮对H.pylori阴性功能性消化不良(functional dyspepsia,FD)患者症状评分及胃肠道嗜酸粒细胞的影响。方法基线时对FD患者进行腹部症状问卷调查、胃镜检查、病理学检查、H.pylori检测。对H.pylori阴性者行埃索美拉唑或替普瑞酮治疗。第2周和第6周分别行腹部症状问卷调查,部分患者第6周末复查胃镜检查及病理学检查。结果第2周末时埃索美拉唑组和替普瑞酮组的症状均较基线时明显改善。第6周末时埃索美拉唑组症状评分较基线时也明显改善,但替普瑞酮组未见显著改变。第6周末时埃索美拉唑组较替普瑞酮组的症状显著减轻。治疗前后埃索美拉唑组与替普瑞酮组在胃十二指肠嗜酸性粒细胞计数及集簇率比较差异均无统计学意义(P0.05)。结论埃索美拉唑对H.pylori阴性FD患者的治疗效果优于替普瑞酮。埃索美拉唑和替普瑞酮均不影响H.pylori阴性FD患者的胃十二指肠嗜酸性粒细胞计数及集簇率。     

嗜酸粒细胞性胃肠炎临床分析24例

目的:分析嗜酸粒细胞性胃肠炎的临床特点、诊断要点、治疗及预后.方法:对本院确诊的24例嗜酸粒细胞性胃肠炎患者的病史、临床表现、实验室检查、内镜检查、病理检查、治疗及随访情况进行系统分析.结果:本组资料显示,有过敏史者占37.5%,过敏诱因者占25%.腹痛(87.5%)为最常见的临床表现.83.3%患者外周血嗜酸粒细胞计数升高,为(4.59±3.69)×109/L.内镜下主要表现为黏膜充血水肿、糜烂,以十二指肠最为明显,活检可证实大量嗜酸粒细胞浸润.腹水为渗出液,可见嗜酸粒细胞.激素治疗可在1 wk内迅速缓解症状,并使嗜酸粒细胞恢复正常.病情可有反复,但预后良好.结论:外周血嗜酸粒细胞计数、过敏史对诊断嗜酸粒细胞性胃肠炎有提示作用,内镜下黏膜活检和腹水嗜酸粒细胞增多是诊断的关键.糖皮质激素治疗优于饮食治疗,预后良好.     

嗜酸性粒细胞性胃肠炎临床特点分析

目的 探讨嗜酸性粒细胞性胃肠炎（EG）的临床特征.方法 对21例EG患者的临床资料进行回顾性分析.结果 21例EG患者中,最常见的临床症状为腹痛（76.2％）,以及恶心呕吐、腹胀、腹泻、纳差等.20例外周血嗜酸性粒细胞增高（95.2％）,内镜检查见炎症最常累及胃窦及十二指肠球部,组织活检可见嗜酸性粒细胞浸润.黏膜型13例,混合型（黏膜型合并浆膜型）8例.腹腔积液阳性者（7例）,混合型所占比例、合并电解质紊乱例数及CRP水平均高于腹腔积液阴性者（14例）.21例确诊后均予饮食调节和去除过敏原治疗,同时给予抑酸、保护胃黏膜、调节胃肠动力和肠道菌群等药物,其中15例予泼尼松治疗.治疗后患者嗜酸性粒细胞计数低于治疗前,症状得到改善.结论 EG临床表现无特异性,超敏反应病史、外周血嗜酸性粒细胞计数增高、胃肠道组织嗜酸性粒细胞浸润有助于诊断,确诊依靠组织活检.合并腹腔积液者较无腹腔积液者病情重.治疗主要是去除过敏原及对症治疗,对于症状较重者可加用糖皮质激素.     

血清嗜酸性粒细胞阳离子蛋白在冠心病患者中的意义

目的 通过检测血清嗜酸性粒细胞阳离子蛋白在冠心病患者中的水平,探讨血清嗜酸性粒细胞阳离子蛋白水平与冠心病及其冠状动脉病变严重程度的关系.方法 选择冠心病患者70例,非冠心病患者(对照组)30例,进行选择性冠状动脉造影,并根据其冠状动脉病变血管支数将冠心病患者分为单支病变组、双支病变组和三支病变组,分别比较3组之间血清嗜酸性粒细胞阳离子蛋白的水平.结果 冠心病组患者血清嗜酸性粒细胞阳离子蛋白水平明显高于对照组(P＜0.01);冠心病中冠状动脉单支病变组、双支病变组、三支病变组血清中嗜酸性粒细胞阳离子蛋白水平比较具有统计学意义(P＜0.01).多元线性回归分析显示血清中嗜酸性粒细胞阳离子蛋白与冠状动脉严重程度有密切关系.多元逐步Logistic回归分析显示,血清嗜酸性粒细胞阳离子蛋白水平是冠心病发病的危险因素.结论 血清中嗜酸性粒细胞阳离子蛋白水平与冠状动脉病变的进展可能有直接关系.嗜酸性粒细胞阳离子蛋白水平越高,提示冠状动脉病变越严重.     

Nodular duodenitis

This prospective study evaluated the radiographic, endoscopic, histologic, and clinical characteristics of nodular duodenitis found in 17 of 50 (34%) patients with end-stage renal disease. By comparison, nodular duodenitis was noted in only 23 of 557 (4%) consecutive endoscopies in a general medical population. Endoscopic nodular duodenitis consisted of two or more nodules, 2.5-7.0 mm in diameter, with apical erythema, with or without tip erosions. Eight patients had nodules in the bulb only, eight had diffuse duodenal nodules, and a single patient had nodules only in the second portion of the duodenum. Single-contrast barium x-rays were sensitive in detecting the nodules only when they were 5 mm or greater in diameter. Some degree of inflammatory infiltrate was found in 14 of 17 (82%) of the patients with nodular duodenitis; 10 of 17 had a moderate to severe histologic grade compared to 3 of 18 (P = 0.015) patients with a normal endoscopic appearance to the duodenum. Several patients with endoscopic nodular duodenitis, in whom biopsies were taken both of the nodule and surrounding mucosa, were found to have a focal histologic lesion which consisted of villous blunting and thickening due to fibrosis and a chronic inflammatory infiltrate or lymphoid aggregate in the stroma. A higher incidence of peptic ulcers occurred in the nodular duodenitis group (3 of 17) compared to the remainder of the group (0 of 33) during a mean follow-up of 38 months (P = 0.03). Resolution of the nodules occurred in six patients following successful renal transplant (four patients) and following vagotomy and pyloroplasty (two patients).(ABSTRACT TRUNCATED AT 250 WORDS)     

影像呈结节状占位样的特发性肥厚性硬脑膜炎临床分析

 目的 探讨影像呈结节状占位样的特发性肥厚性硬脑膜炎(IHCP)的临床、影像及病理特点。方法 对4例影像呈结节状的IHCP患者的临床资料进行回顾性分析。结果 4例均为男性,平均发病年龄40.25(33～50)岁,病程长(4个月～12年),病情反复发作。主要表现为慢性头痛、多组脑神经麻痹及癫痫发作。头颅影像示局部硬脑膜增厚,呈团块或结节状,与脑膜瘤等肿瘤相似。2例组织病理学检查示硬脑膜结缔组织增生、散在中性粒细胞及浆细胞浸润。甲泼尼龙或联合免疫抑制剂治疗有效。结论 结节状占位样IHCP多以慢性头痛起病,糖皮质激素或联合免疫抑制剂治疗有效,病情易复发。头颅影像可见硬脑膜肥厚,呈团块或结节状占位,易与脑肿瘤、炎性肉芽肿相混淆。
        

Evaluation of non-ampullary duodenal polyps: Comparison of non-neoplastic and neoplastic lesions

AIM: To evaluate duodenal polyps, divided into nonneoplastic and neoplastic lesions. In addition, the clinical characteristics of duodenal hyperplastic polyps are determined. METHODS: We analyzed medical records of 50 114 consecutive patients submitted to for first diagnostic esophago-gastroduodenoscopy between January 2004 and December 2009. We excluded lesions on the ampulla of Vater and submucosal tumors. We studied 510 cases that were diagnosed endoscopically with duodenal polyps and enrolled a total of 221 cases that had undergone tissue biopsy. We analyzed the differences between non-neoplastic and neoplastic lesions, and determined the clinical features of duodenal hyperplastic polyps. RESULTS: Non-neoplastic lesions were found in 196 patients and neoplastic lesions in 25 patients. On uni-variate analysis, there were significant differences in shape, location, and size. Polyps more than 10 mm in diameter or polyps in the second portion had independent risk factors for being neoplastic lesions, as identified by multivariate analysis. In 23 cases of hyperplastic polyps (79.3%), they were accompanied by gastroduodenal pathology, which was possibly associated with Helicobacter pylori . CONCLUSION: Polyps of more than 10 mm or polyps in the second portion of the duodenum should be evaluated by histological examination.     

Diagnosis and surgical treatment of primary hepatic cholangiocarcinoma

BACKGROUND/AIMS: To summarize the experience in the diagnosis and surgical treatment of primary hepatic cholangiocarcinoma (PHCC). METHODOLOGY: Forty-seven PHCC patients, who had undergone surgical treatment in our hospital from 1970 to 1999, were retrospectively analyzed with their clinicopathological features, surgical treatments and prognoses. RESULTS: 1) PHCC patients lacked typical clinical manifestation, and some had an accompanying history of virus B hepatitis (36.2%, 17/47) and liver cirrhosis (46.8%, 23/47), and elevation of AFP (25.5%, 12/47). 2) The detection rates of BUS, CT, MRI and liver scintigraphy were 95.7% (45/47), 93.6% (44/47), 100% (23/23) and 100% (4/4), respectively. The lesions detected by those methods needed to differentiate with hepatocellular carcinoma. 3) The resection rate was 76.6% (36/47), the postoperative overall 1-, 3- and 5-year survival rates were 54.1% (24/47), 34.0% (16/47), and 29.8% (14/47) in all patients, respectively, including the 1-, 3- and 5-year survival rates were 83.3% (30/36), 50.0% (18/36), and 38.9% (14/36) in hepatectomy cases, respectively. 4) All of the four patients who survived more than 5 years, including small liver cancer (< or = 3 cm) three cases and encapsulated large nodular lesion one case, received curable resection and postoperative adjuvant therapies. CONCLUSIONS: PHCC patients can obtain a better result by early detection and curative resection with adjuvant therapies.     

Gastrointestinal stromal tumors (GISTs): clinical aspects]

INTRODUCTION: Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors in the gastrointestinal tract. One of their features is the expression of the c-KIT/CD117 receptor. AIMS AND METHODS: We will focus on describing the symptoms, clinical studies prior to diagnosis, histologic and immunohistochemical characteristics, as well as the progression of disease in a group of patients. RESULTS: Seventeen cases were diagnosed between December 1999 and April 2005. Mean age of patients was 64.5 (+/-11.9); 47% were women. Tumor location was as follows: 52.9% in the jejunum or ileum, 29.4% were gastric, 11.7% were in the duodenum, and 5.8% were located in the mesentery. Tumor size was 6.0 cm on average (+/-5.0); 47% were asymptomatic, and to a lesser degree caused abdominal pain or digestive bleeding; 94.1% of tumors expressed CD117. Most of them were discovered while performing a laparotomy or ultrasound scan; 94.1% of tumors were removed; 35.2% (6 out of 17) of patients suffering from GIST met consensus criteria for aggressive behavior. Over 25.6 months (+/-22.5) metastasis or tumor relapse occurred in 23.5% (4 out of 17) of patients--those with more frequent high-risk criteria, symptomatic and bigger tumors, and tumors not expressing CD117. The three patients with tumor relapse were prescribed imatinib mesylate. Three patients died because of the tumor, and four from other causes unrelated to GIST. CONCLUSIONS: GIST was diagnosed in around 12 cases per million a year. Its diagnosis was usually an incidental finding during a medical evaluation, and tumors were malignant in nearly one fourth of cases. We can predict its outcome depending on different aspects.     

The endoscopic appearance of duodenal folds is predictive of untreated adult celiac disease.

The loss of duodenal folds visible endoscopically has recently been reported as being a marker for celiac disease. We have investigated the sensitivity and specificity of this finding with a prospective study in 75 patients with symptoms or results of investigations compatible with celiac disease. Reported duodenal fold appearance was compared with histological findings, disaccharidase levels, and clinical diagnosis. Fifteen patients were found to have celiac disease and 11 had reduced or absent duodenal folds compared with only 2 of 60 patients who did not have celiac disease (p less than 0.0001). This finding has a sensitivity of 73%, specificity of 97%, and positive predictive value of 85%. Duodenal folds were not reported as being abnormal in seven patients with hypolactasia or two with giardiasis and did not appear to be influenced by age. A reduction in the number or height of duodenal folds as seen endoscopically in the second part of the duodenum is a specific and sensitive sign of celiac disease. Endoscopists should biopsy the duodenum for celiac disease whenever the duodenal folds appear to be reduced or absent.     

Glomerulosclerosis in Type 2 (non0insulin-dependent) diabetes mellitus: relationship to glycaemia in the University Group Diabetes Program (UGDP)

Summary Kidney tissue of acceptable quality was available from autopsies of 55 patients who had been followed prospectively for 3 to 15 years as participants in the University Group Diabetes Program, a study of vascular disease in Type 2 (non-insulin-dependent) diabetic patients. Slides were prepared for light microscopic reading by uniform histologic techniques, and then were randomly intermixed and coded with tissues identically prepared from matched nondiabetic subjects (morphologic controls). After independent review by three morphologists, the results were tabulated and assigned to one of four diagnostic groups: 1) typical diabetic nodular glomerulosclerosis; 2) mesangial changes suggestive of diabetes (diffuse lesion); 3) non-diabetic renal disease; 4) normal for age. Of the diabetic cases 31% (17 of 55) were found to show nodular glomerulosclerosis, and another 47% (26 of 55) showed suggestive changes; none of the morphologic control slides was read as showing nodular glomerulosclerosis, but some were judged to show suggestive mesangial (diffuse) changes. Although only 4 of the 17 diabetic patients with nodules had died of uraemia, many had hypertension, which may have contributed to their deaths from vascular disease. The patients with nodular glomerular changes also showed, on the average, the highest blood glucose levels during life. Type 2 diabetes in later life appears to be associated with a high risk for typical tissue changes of diabetic kidney damage, which may contribute significantly to morbidity and mortality and may be present before azotaemia and qualitative proteinuria have been recognized.     

Prevalence and predictive factors of eosinophilic esophagitis in patients presenting with dysphagia: a prospective study

OBJECTIVES: Eosinophilic esophagitis (EE) is an increasingly recognized cause of dysphagia. We prospectively assessed the prevalence of EE using midesophageal biopsies in patients presenting with no endoscopically evident cause of dysphagia. We also aimed to determine the clinical and endoscopic factors predictive of EE in outpatients undergoing endoscopy for dysphagia. METHODS: Outpatients (18-60 yr of age) undergoing endoscopy for dysphagia at Mayo Clinic, Rochester between June 2005 and June 2006 were enrolled. Patients completed the validated Mayo Dysphagia Questionnaire (MDQ). Biopsies were obtained from the midesophagus if there was no endoscopically evident cause of dysphagia or there were endoscopic findings suggestive of EE. EE was defined as the presence of >20 eosinophils/high-power field. Logistic regression was performed to identify predictors of EE. RESULTS: Of 376 patients enrolled, 238 (63%) completed the MDQ and 222 (59%) had midesophageal biopsies; 33 (15%, 95% CI 6%-12%) had EE by biopsy. Ten of 102 (9.8%) patients who appeared endoscopically normal had EE by biopsy, while 8 of 21 (38%) patients with endoscopic changes suggestive of EE had EE on biopsy. Predictors of EE were younger age, endoscopic features suggestive of EE, absence of use of proton pump inhibitors, and a history of any food impaction for greater than 5 min. CONCLUSIONS: Midesophageal biopsies from normal-appearing mucosa should be obtained in all patients with unexplained solid food dysphagia; this may diagnose EE in about one in 10 cases.     

A Gastrointestinal Endoscopic Study on Secondary Amyloidosis Complicating Rheumatoid Arthritis

Abstract: Biopsy specimens endoscopically obtained from the stomach, duodenum, sigmoid colon and rectum of 58 patients with rheumotoid arthritis (RA) were examined for amyloid deposition by light microscopy. Six cases (10.3%) were diagnosed as being amyloidosis. In cases with more than moderate amyloid deposition, amyloid was most notable in the lamina propria mucosae. In cases with slight amyloid deposition, amyloid was seen in the blood vessel walls of the lamina muscularis mucosae and tela submucosa. On the whole, amyloid deposition was most marked in the duodenum. Endoscopically, whitish, irregular, partially nodular thickening of mucosa was characteristic in the sites with severe amyloid deposition. A fine granular appearance was observed in the sites with moderate amyloid deposition. No particular abnormal findings were noticed in the sites with slight amyloid deposition. Erosions, redness and a tendency for easy mucosal bleeding appeared frequently in the sites with more than moderate amyloid deposition. The endoscopic abnormal findings were most remarkable in the duodenum, and mildest in the recto-sigmoid colon. It was suggested that these abnormal findings were correlated with the degree of amyloid deposition especially in the lamina propria mucosae. All the amyloidosis cases were in advanced anatomical stages of RA. The degree of anemia was more severe, and total protein, albumin and total cholesterol values were lower in the amyloidosis cases than in non-amyloidosis cases. It is clear that when endoscopic examinations of RA patients are done to rule out amyloidosis, a biopsy should be taken from a depth including the submucosa even if the endoscopic finding is normal.     

Idiopathic retroperitoneal fibrosis: clinicopathologic features and outcome analysis

To describe clinical features and outcomes of 26 patients with idiopathic retroperitoneal fibrosis from a single center, we reviewed medical records of consecutive patients with idiopathic retroperitoneal fibrosis evaluated at our facility from January 1, 1998 to December 31, 2013 for clinical features, laboratory and radiographic findings, management, and outcomes. Twenty-six patients met criteria for idiopathic retroperitoneal fibrosis and were included in the study. Median age at diagnosis was 58 years; male–female ratio was 3.3:1.0. Median duration of symptoms was 7 weeks. Abdominal, flank, and/or low back pain were the most common presenting symptoms. Four patients (15 %) had associated autoimmune or fibrosing disorders. Baseline erythrocyte sedimentation rate was elevated in 17 (77 %) of 22 patients tested and C-reactive protein was elevated in 10 (56 %) of 18 patients tested. Hydronephrosis was present in 17 (68 %) patients; 8 (47 %) of 17 had bilateral hydronephrosis. Retroperitoneal mass biopsy was performed in 18 (69 %) patients. Two patients had idiopathic retroperitoneal fibrosis classifiable as IgG4-related disease. Therapy consisted of medications alone in 7 cases, surgical interventions alone in 7 cases, and a combination in 11 cases. One patient achieved remission with no treatment. Most patients treated medically received initial corticosteroids. Methotrexate (1 case), azathioprine (1 case), mycophenolate mofetil (1 case), and tamoxifen (5 cases) were used. No relapses occurred after a median 5-year follow-up. Two (8 %) patients died; five (19 %) developed cancer after diagnosis. In this series, we emphasize the importance of early diagnosis and therapy for overall favorable prognosis of idiopathic retroperitoneal fibrosis.