OBJECTIVE: To investigate the clinical features and outcome of hydronephrosis induced by retroperitoneal fibrosis (RPF), and to evaluate the effect of corticosteroid based therapy combined with surgical intervention of ureteral obstruction. METHODS: A total of 17 RPF patients with hydronephrosis hospitalized in Peking University International Hospital from May 2016 to December 2019 were analyzed retrospectively. RESULTS: The median age was 56 (53, 65) years, the male to female ratio was 2.4 : 1, and the disease duration was 4.00 (0.83, 8.00) months. The initial symptoms included back pain (9 cases), abdominal pain (6 cases), oliguria (2 cases) and lower limb edema (3 cases). Eight patients presented left hydronephrosis, 1 right hydronephrosis and 8 bilateral hydronephrosis. C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) were both elevated in 13 patients (76.5%, n=17). Immunoglobin (Ig) G4 increased in 5 cases (29.4%, n=17). IgG, IgE and IgA increased in 4 cases (30.8%, n=13), 4 cases (30.8%, n=13) and 1 case (7.7%, n=13), respectively. Among 12 patients who underwent biopsy, 3 patients were diagnosed with IgG4-relate disease. The level of IgG4 in the tissues varied, 6 cases expressed less than 10 per high power field (HPF) or no expression (50.0%). Only 2 cases expressed 10-30/HPF (16.7%), and 4 cases revealed more than 30/HPF (33.3%). Among the 17 patients with ureteral obstruction, no urinary drainage procedure was needed in 4 patients who had mild ureteral obstruction, whereas, ureteral stenting was carried out in the other 13 cases before drug treatment. Time was too short to evaluate the effect of urinary drainage procedures in 4 patients. For the rest, ureterolysis had to be performed in 3 cases after failed ureteral stent insertion. Successful drain removal was accomplished in all of these 9 patients and the mean time to drain removal was (6.7±3.0) months. In addition, 10 patients had complete medical records after an average follow-up time of 5 (3-13) months. Levels of ESR, CRP, IgG4, IgG, IgE, IgA were 54.0 (36.3, 98.5) mm/h, 26.8 (8.7, 53.0) mg/L, 1.34 (0.55, 3.36) g/L, 16.3 (13.0, 21.1) g/L, 40.5 (31.4, 203.0) IU/mL, 2.51 (1.82, 3.25) g/L at baseline, which all decreased predominantly after treatment. ESR, CRP, IgG4, IgG, IgE and IgA dropped by 38.5 (23.5, 54.3) mm/h (P < 0.01), 23.0 (5.5, 52.0) mg/L (P < 0.05), 0.92 (0.40, 2.85) g/L (P < 0.01), 6.5 (1.7, 9.1) g/L (P < 0.05), 23.7 (4.8, 162.0) IU/mL (P < 0.05) and 0.77 (0.32, 1.26) g/L (P < 0.05), respectively. Size of mass measured by CT/MRI imaging became smaller significantly and hydronephrosis relieved. CONCLUSION Onset of RPF is insidious and lack of specific initial symptoms. Corticosteroid based therapy combined with surgical intervention of relieving obstruction is effective.
Aged ; Female ; Humans ; Hydronephrosis/etiology* ; Male ; Retroperitoneal Fibrosis/complications* ; Retrospective Studies ; Ureter ; Ureteral Obstruction

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

Retroperitoneal fibrosis is associated with fibroblast proliferation due to inflammatory changes in adipose/fibrous tissue. Given that aortic dilation in abdominal aortic aneurysm can cause compression of the ureter, abdominal aortic aneurysm complicated by retroperitoneal fibrosis is likely to result in urinary tract obstruction. Accordingly, close attention to changes in perioperative urine volume is warranted when operating on patients with abdominal aortic aneurysm complicated by retroperitoneal fibrosis. We have recently performed laparotomies on two cases of abdominal aortic aneurysm complicated by retroperitoneal fibrosis. In the first case, surgery was performed without the placement of a ureteral stent. The patient developed postrenal acute renal failure caused by postoperative urinary retention. In the second case, ureteral stent placement in advance enabled perioperative management without complications. The clinical course of these cases suggests that, in laparotomy with concomitant retroperitoneal fibrosis, preoperative ureteral stent placement can prevent postoperative complications in the renal and urinary systems.
Acute Kidney Injury ; Aortic Aneurysm ; Aortic Aneurysm, Abdominal ; Fibroblasts ; Humans ; Laparotomy ; Postoperative Complications ; Retroperitoneal Fibrosis ; Stents ; Ureter ; Urinary Retention ; Urinary Tract

No abstract available.
Anti-Inflammatory Agents/therapeutic use ; Autoimmune Diseases/complications/*diagnosis/drug therapy ; Humans ; Immunoglobulin G/blood ; Male ; Middle Aged ; Pancreatitis/complications/*diagnosis/immunology ; Prednisolone/therapeutic use ; Retroperitoneal Fibrosis/complications/*diagnosis/immunology ; Tomography, X-Ray Computed

Retroperitoneal fibrosis is a rare disease characterized by the formation of dense plaque of fibrous tissue covering the retroperitoneal structures. This disease is commonly presented as ureteral obstruction, but the involvement of duodenum is rare. We report a case of retroperitoneal fibrosis which was complicated with duodenal stenosis and was successfully treated with corticosteroids. A 58-yr-old man, who had history of aorto-iliac bypass graft due to arteriosclerosis obliterans with infrarenal aortic occlusion was admitted to the hospital with abdominal pain and a mass. Abdominal CT scan revealed the periaortic soft tissue mass encircling grafted aorta and stenosis of duodenal third portion. Retroperitoneal fibrosis with duodenal stenosis was diagnosed and prednisolone therapy was initiated. Follow-up CT scan showed that the patient responded to prednisolone therapy with eased pain, shrinking periaortic mass, and reduced duodenal stenosis.
Anti-Inflammatory Agents, Steroidal/therapeutic use ; Case Report ; Duodenal Obstruction/*complications/drug therapy/physiopathology/radiography ; Glucocorticoids, Synthetic/therapeutic use ; Human ; Male ; Middle Age ; Prednisolone/therapeutic use ; Retroperitoneal Fibrosis/*complications/drug therapy/physiopathology/radiography ; Tomography, X-Ray Computed/methods ; Treatment Outcome

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

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