Objective To analyze the value of abdominal CT images combined with serological indicators in predicting the ureteral involvement in idiopathic retroperitoneal fibrosis(IRF). Methods The CT images of 79 IRF patients were analyzed retrospectively,including the involved sites and enhancement characteristics of the lesions.According to the inclusion and exclusion criteria,43 patients with complete serological data were selected and assigned into a ureteral involvement group(n=29)and a non-ureteral involvement group(n=14) according to whether ureters were involved in IRF.Logistic regression analysis was performed to select independent risk factors for ureteral involvement in IRF.The receiver operating characteristic(ROC)curve was plotted to evaluate the predictive value of the CT arterial phase enhancement magnitude and serum cystatin C(CysC)for ureteral involvement in IRF. Results The CT images of IRF usually showed a soft tissue density lesion encompassing the abdominal aorta,iliac arteries,ureters,and retroperitoneal tissue,with a wide range of distribution.The ureteral involvement group and the non-ureteral involvement group showed differences in gender(P=0.031),CT arterial phase enhancement amplitude(P=0.014),CT venous phase enhancement amplitude(P=0.032),and serum CysC(P=0.036).Logistic regression analysis showed that gender(P=0.034),CT arterial phase enhancement amplitude(P=0.046),and serum CysC(P=0.041)were independent risk factors for ureteral involvement in IRF.The area under the curve for CT arterial phase enhancement combined with serum CysC to predict ureteral involvement in IRF was 0.776.Ten patients had lower levels of erythrocyte sedimentation rate(P<0.001),C-reactive protein(P=0.021),and IgG4(P<0.001)in the follow-up period than before treatment. Conclusion The combination of abdominal CT images with serological indicators demonstrates high accuracy in predicting the ureteral involvement in IRF,providing reference for early clinical diagnosis.
Humans ; Male ; Female ; Retroperitoneal Fibrosis/pathology* ; Middle Aged ; Retrospective Studies ; Tomography, X-Ray Computed ; Aged ; Adult ; Ureter/diagnostic imaging* ; Predictive Value of Tests ; Cystatin C/blood*

A 51-year-old male patient was presented to our hospital for inspection of right renal space-occupying lesions. CT revealed soft tissue density in the right renal pelvis and renal pelvis ureter transitional crumb, for which a clinical diagnosis of right renal cancer was made. After laparoscopic radical resection of the right kidney, pathological examination supported the diagnosis of idiopathic retroperitoneal fibrosis. With an unclear pathogenesis, idiopathic retroperitoneal fibrosis presents with atypical clinical manifestations but shows specific features in imaging examination. Its treatment is individualized according to the specific condition for which conservative medical or surgical treatment can be considered. Retroperitoneal fibrosis has a low incidence and a high misdiagnosis rate, and imaging examinations remain currently the primary modality for diagnosis with specific findings.
Diagnostic Errors ; Humans ; Kidney Neoplasms ; Kidney Pelvis ; pathology ; Male ; Middle Aged ; Retroperitoneal Fibrosis ; diagnosis ; Ureter ; pathology

The cardiovascular system may be one of the target organs of both immunoglobulin G4 related and non-related systemic multifocal fibrosclerosis. We present a case of IgG4 non-related systemic multifocal fibrosclerosis mimicking mitral stenosis on echocardiography. For a more detailed differential diagnosis, we used multimodal imaging techniques. After surgical biopsy around the abdominal aortic area in the retroperitoneum, histological examination revealed IgG4 non-related systemic multifocal fibrosclerosis. We describe the multimodal imaging used to diagnose IgG4 non-related systemic multifocal fibrosclerosis and a positive response to steroid treatment. There have been no previous case reports of IgG4 non-related systemic multifocal fibrosclerosis with intracardiac involvement. Here, we report a case of IgG4 non-related systemic multifocal fibrosclerosis mimicking mitral stenosis.
Aged ; Aorta, Abdominal/pathology ; Diagnosis, Differential ; Echocardiography ; Female ; Humans ; Immunoglobulin G/*blood/immunology ; Magnetic Resonance Imaging ; Mitral Valve Stenosis/diagnosis ; Myocardium/*pathology ; Peritoneum/surgery ; Positron-Emission Tomography ; Retroperitoneal Fibrosis/*congenital/diagnosis/drug therapy/ultrasonography ; Steroids/therapeutic use ; Tomography, X-Ray Computed

An optimal treatment for cap polyposis has not been established. Several treatment approaches, including anti-inflammatory agents, antibiotics, immunomodulators, and endoscopic therapy have been described. Surgical resection of the affected colon and rectum may be indicated for patients with persistent disease. Repeat surgery is indicated in cases of recurrence after surgery. However, symptomatic polyposis may still recur, and spontaneous resolution of cap polyposis is possible. We report a case of recurrent cap polyposis complicated with retroperitoneal fibrosis after inadequate low anterior resection with a positive resection margin. Surgical approaches for the treatment of cap polyposis should be carefully considered before treatment.
Anti-Inflammatory Agents/therapeutic use ; Colonic Polyps/surgery ; Colonoscopy ; Female ; Humans ; Intestinal Polyposis/complications/*diagnosis/pathology ; Middle Aged ; Prednisolone/therapeutic use ; Recurrence ; Retroperitoneal Fibrosis/complications/*diagnosis/drug therapy ; Tomography, X-Ray Computed

Sclerosing mesenteritis is a rare inflammatory disease of the bowel mesentery. It produces tumor-like masses of the mesentery composed of varying degrees of fibrosis, chronic inflammation, and fat necrosis. It has been described variously as fibrosing mesenteritis, retractile mesenteritis, mesenteric Weber Christian disease, and systemic nodular panniculitis. The etiology and pathogenesis of the disease are as yet unknown, but autoimmune disorder, previous abdominal surgery, trauma, and ischemia could play a role. The clinical features include abdominal pain, vomiting, diarrhea, and constipation. Occasionally, patients with this condition may present with bowel obstruction. Rarely, It can be associated with other idiopathic inflammatory disorders such as retroperitoneal fibrosis, sclerosing cholangitis, and orbital pseudotumors. We report a case of idiopathic sclerosing mesenteritis with retroperitoneal fibrosis in a 58-year-old man.
Anti-Inflammatory Agents/therapeutic use ; Antineoplastic Agents, Hormonal/therapeutic use ; Diagnosis, Differential ; Humans ; Laparoscopy ; Male ; Middle Aged ; Panniculitis, Peritoneal/complications/*diagnosis/drug therapy ; Prednisolone/therapeutic use ; Retroperitoneal Fibrosis/complications/*diagnosis/pathology ; Tamoxifen/therapeutic use ; Tomography, X-Ray Computed

Retroperitoneal fibrosis (RPF) is a rare disease with unclear etiology, which is characterized by chronic non-specific inflammation of the retroperitoneum. This study was performed to investigate the clinical characteristics, laboratory findings, radiologic findings, treatment and outcome in Korean patients with RPF. We retrospectively reviewed medical records of 27 RPF patients who were admitted to Yonsei University Medical Center between 1998 and 2009. Twenty-two patients (81%) were male. The mean age at diagnosis was 56 yr. Nine patients had identifiable risk factors of RPF and three patients had combined autoimmune diseases. Acute phase reactants were elevated in most patients. Rheumatoid factor was positive in 3 of 16 patients (19%) and antinuclear antibody in 4 of 17 (24%). Five of 6 patients who were taken positron-emission tomography showed positive uptake. Glucocorticoids were used in 16 patients (59%) and four of them received combination therapy with azathioprine. After immunosuppressive treatment, the levels of acute phase reactants dropped, and the size of mass also decreased in most patients. In conclusion, the clinical characteristics of RPF in Korean patients are similar with other series except for higher proportion of male. Some patients with RPF have autoimmune features. The effect of immunosuppressive treatment on RPF is good.
Acute Disease ; Adult ; Aged ; Antibodies, Antinuclear/immunology/metabolism ; Azathioprine/therapeutic use ; Drug Therapy, Combination ; Female ; Glucocorticoids/therapeutic use ; Humans ; Immunosuppressive Agents/therapeutic use ; Male ; Middle Aged ; Positron-Emission Tomography ; Republic of Korea ; Retroperitoneal Fibrosis/*diagnosis/drug therapy/pathology ; Retrospective Studies ; Sex Factors ; Tomography, X-Ray Computed

Idiopathic retroperitoneal fibrosis (IRPF) is a rare disease characterized by a retroperitoneal inflammatory proliferative fibrosing process. Hashimoto's thyroiditis is the most common inflammatory condition of the thyroid gland; and is a frequently-occurring autoimmune disorder manifesting predominantly in middle-aged women. We report a rare association of IRPF with Hashimoto's thyroiditis in a 67-year-old man demonstrating good response to steroid therapy.
Aged ; Anti-Inflammatory Agents/therapeutic use ; Hashimoto Disease/*complications/drug therapy ; Humans ; Male ; Pregnenediones/therapeutic use ; Retroperitoneal Fibrosis/*complications/drug therapy/pathology

OBJECTIVETo investigate the clinical features and outcomes of 18 patients with retroperitoneal fibrosis (RPF).

METHODWe retrospectively analyzed the clinical data of 18 RPF patients who received treatment in Peking Union Medical College Hospital from 1988 to 2004.

RESULTSThe initial symptoms included abdominal pain, notalgia, abdominal distension, and pyrexia. Elevated erythrocyte sedimentation rate (35-120 mm/h), immunoglobumin, and gamma globulin levels were found in 12 patients (66.7%), 4 patients (22.2%), and 5 patients (27.8%), respectively. Mild positive antinuclear antibody (1:160-1:320) was found in 3 patients (16.7%). Urethral obstructions were found by ultrasonography and intravenous pyelography, and the existence of post-peritoneal soft tissue shadow was confirmed by computerized tomography and magnetic resonance imaging. The final diagnosis included 15 primary RPF cases and 3 secondary RPF cases. Eleven patients only received drug therapy and 6 patients underwent surgery. The conditions improved in most patients, except that one patient died of tumor.

CONCLUSIONSThe initial symptoms of RPF are not specific. For patients with positive laboratory and radiological examination results, further imagings, surgeries, or interventional therapy may be considered.

Abdominal Pain ; complications ; Blood Sedimentation ; Fever ; complications ; Humans ; Immunoglobulins ; metabolism ; Magnetic Resonance Imaging ; Retroperitoneal Fibrosis ; diagnosis ; diagnostic imaging ; pathology ; Tomography, X-Ray Computed ; Ultrasonography ; Urethral Obstruction ; diagnostic imaging ; gamma-Globulins ; metabolism

Idiopathic retroperitoneal fibrosis (IRF) is a rare human disease characterized by non-neoplastic fibroblastic proliferation associated with chronic inflammatory cells; its pathogenesis is obscure. We undertook an immunohistochemical study for the expression of HLA-DR antigens and other immune-related markers by retroperitoneal proliferating fibroblasts and inflammatory cells from 2 IRF patients. Patterns of immunoreactivity were compared with those expressed by human nodular fasciitis (NF) and granulation tissue. In IRF, most fibroblasts immunostained strongly for HLA-DR antigens, whereas fibroblasts in NF and granulation tissue did, not immunostain at all. The fibroblasts did not immunostain for interleukin 2 receptor, C3b receptor, CD-4, CD-8, or Leu-M1 in any of the tissue studied. Most macrophages and lymphocytes in IRF and NF immunostained Strangly for HLA-DR antigens. In IRF, the CD-4 and CD-8 immunostained T-lymphocytes appeared equally distributed. The expression of HLA-DR antigens by fibroblasts in IRF indicates that this rare disease may indeed be an immune-associated hypersensitivity disorder.
Adult ; Aged ; MH - ; Biological Markers ; Fasciitis/pathology ; Fibroblasts/*immunology/pathology ; Granulation Tissue/pathology ; HLA-DR Antigens/*analysis ; Humans ; Male ; Middle Aged ; Retroperitoneal Fibrosis/*immunology/pathology