OBJECTIVE: To explore the diagnostic value of contrast-enhanced ultrasound (CEUS) in the disease activity of idiopathic retroperitoneal fibrosis (IRPF).
METHODS: This retrospective study included 148 CEUS examinations from 63 patients with IRPF treated in our hospital from April 2016 to September 2021. They were divided into two groups: IRPF active group (69 examinations) and inactive group (79 examinations). Uni- and multivariable analyses were used to identify independent risk factors for IRPF activity. Receiver operating characteristic (ROC) curves were drawn to establish different diagnostic models to evaluate the diagnostic value of IRPF activity. The z test was used to compare the differences of the area under the curves (AUCs). The value of CEUS in evaluating the variation of disease activity over time was also investigated between repeat patient studies.
RESULTS: Univariate and multivariate logistic regression analyses revealed the thickness [odds ratio (OR) = 14.125, 95% confidence interval (CI) = 3.017-66.123] was the most significant independent risk factor for IRPF activity (P < 0.01). The best diagnostic model was model 3, which was established by CEUS score combined with thickness. The AUC was 0.944 (95%CI = 0.912-0.977), and the sensitivity and specificity were 89.86% and 86.08%, respectively. The diagnostic performance was not significantly improved after combining clinical symptom (back pain) and laboratory indicators [erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)]. Compared with before treatment, the CEUS score and thickness were significantly decreased after treatment (x2 = 14.580, P < 0.001 and z = 4.708, P < 0.001, respectively).
CONCLUSIONS: CEUS has good diagnostic value for IRPF disease activity. Key points • Thickness and contrast-enhanced ultrasound score were significantly higher in the active group than those in inactive group (P < 0.001). • With thickness of 4 mm and contrast-enhanced ultrasound 2 score as optimal cut-off values, the sensitivity and specificity were 89.86%, 81.01% and 52.17%, 100.00%, respectively. • During follow-up, when the disease progressed, the change of CEUS score was earlier than the change of thickness.

To investigate the effectiveness and safety of TCZ (tocilizumab) monotherapy for chronic periaortitis (CP) patients at acute active stage.
Twelve patients with definite or possible diagnosis of CP were enrolled and received intravenous infusions of TCZ (8 mg/kg) every 4 weeks for at least 3 months. Clinical features, laboratory and imaging findings were recorded at baseline and during the follow-up. The primary endpoint was the rate of partial and complete remission after 3 months TCZ monotherapy and the secondary endpoint was the frequency of treatment related adverse events.
Three patients (27.3%) achieved partial remission and seven patients (63.6%) obtained complete remission after 3 months TCZ treatment. The total remission rate achieved 90.9%. All patients reported improvement in clinical symptoms. Inflammatory markers such as erythrocyte sedimentation rate and C reactive protein decreased to normal levels after TCZ treatment. Nine patients (81.8%) showed remarkable shrinkage of perivascular mass greater than or equal to 50% on CT.
Our study showed that TCZ monotherapy contributed to remarkable clinical and laboratory improvement in CP patients and could be an alternative treatment option for CP.

OBJECTIVE: To evaluate the epidemiology, presentation and outcomes of patients with chronic periaortitis from 1998 through 2018.
METHODS: An inception cohort of patients with incident chronic periaortitis from January 1, 1998 through December 31, 2018, in Olmsted County, Minnesota was identified based on comprehensive individual medical record review utilising the Rochester Epidemiology Project medical record linkage system. Inclusion required radiographic and/or histologic confirmation of periarterial soft tissue thickening around at least part of the infra-renal abdominal aorta or the common iliac arteries. Data were collected on demographic characteristics, clinical presentation, renal and radiographic outcomes, and mortality. Incidence rates were age and sex adjusted to the 2010 United States white population.
RESULTS: Eleven incident cases of chronic periaortitis were identified during the study period. Average age at diagnosis was 61.8±13.4 years. The cohort included 9 men (82%) and 2 women (18%). Age- and sex-adjusted incidence rates per 100,000 population were 0.26 for females, 1.56 for males and 0.87 overall. Overall prevalence on January 1, 2015 was 8.98 per 100,000 population. Median (IQR) length of follow-up was 10.1 (2.5, 13.8) years. Overall mortality was similar to the expected age, sex, and calendar estimates of the Minnesota population with standardised mortality ratio (95% CI) for the entire cohort 2.07 (0.67, 4.84).
CONCLUSIONS: This study reports the first epidemiologic data on chronic periaortitis in the United States. In this cohort of patients with chronic periaortitis, men were 4 times more commonly affected than women. Mortality was not increased compared to the general population.

IgG4-related disease (IgG4-RD) is recently recognized as a fibro-inflammatory condition featured by tumefactive lesions in multiple organs, and the retroperitoneum is one of the common involved sites. We undertook this study to compare detailed demographic, clinical and laboratory characteristics of IgG4-RD patients with retroperitoneum lesion (IgG4-RD RPF+) and retroperitoneum free IgG4-RD (IgG4-RD RPF-) in a large cohort.
We carried out a retrospective review of the medical records of 407 cases of IgG4-RD diagnosed at Peking University People\'s Hospital between March 2009 and May 2019.
Among 407 patients, 58 had retroperitoneum affected. As compared with IgG4-RD RPF- patients, IgG4-RD RPF+ patients showed older age at disease onset and diagnosis. IgG4-RD RPF+ group involved more male patients. In terms of organ involvement, IgG4-RD RPF+ group was more frequently presented with kidney involvement, while salivary gland, lacrimal gland and pancreas were more prominent in the IgG4-RD RPF- group. In addition, the CRP, ESR level and creatinine level were significantly higher in IgG4-RD RPF+ patients, and hypocomplementemia were more common in this group.
We have revealed demographic, clinical and laboratory differences between IgG4-RD RPF+ and RPF- patients, which indicated potential differences in pathogenesis and important implications for the diagnosis and management of these two phenotypes.

OBJECTIVE: The potential role of drugs in the onset of retroperitoneal fibrosis (RPF) is poorly understood. The aim of this study was to identify drugs that may cause RPF.
METHODS: The authors used case/non-case method in the French PharmacoVigilance Database (FPVD).
RESULTS: Among the 722992 reports recorded, 73 cases of RPF were identified. 67% were men and the median age was 60 years (range 26-87). In these 73 cases, 176 drugs were \'suspect.\' Derivatives of ergot alkaloids (DEA) presented the most significant association with RPF. To a lesser extent, significant associations were found with many drugs used in cardiology, e.g. beta-blockers, platelet antiaggregant, statins, and antihypertensive drugs, drugs used in neuropsychiatry, e.g. hypnotics, antiepileptic drugs, anxiolytics, antipsychotics, and antidepressants, and with other pharmacological classes, e.g. TNF-alpha antagonists.
CONCLUSIONS: This study confirmed an association between RPF and derivatives of ergot alkaloids. These data represent a pharmacovigilance signal despite the limits of non/non-case method (underreporting, confounding factors, etc.). Indeed, a significant signal was found with drugs less known (TNF-α antagonists) or not known (some hypnotics, antiepileptic drugs, antipsychotics, anxiolytics, and antidepressants) to induce such an adverse drug reaction (ADR). Finally, these data could contribute to realize prospective studies to confirm these signals.

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to study the presentation, clinical course, laboratoryresults, imaging findings, medical and urological treatments ofidiopathic retroperitoneal fibrosis at our institution.
Between January 2006 and December 2017, medical recordsand operatives\' notes of 116 patients with idiopathic retroperitonealfibrosis (IPRF) were reviewed retrospectively. Diagnosis was doneby clinical and radiological imaging that fulfilled a strict criterion.All patients were initiated on Prednisolone 60 mg for two months,and reduced until reaching 10 mg daily, with a total duration of24 months in the responding patients. Renal drainage was done incases of obstructed kidneys. To assess response, both laboratoryresults and imaging studies at initiation and after 4 months werereviewed and compared.
Of 116 patients diagnosed with IRPF, eighty five (73.3%)were male and thirty one (26.7%) female, with mean ± SD age atpresentation was (50.5 ± 10.6) years. 79% of the patients complaintof abdominal and low back pain, 27% uremic symptoms, 10.3% hada new onset of hypertension, 3.4% presented with anejaculation,and 13.8% were totally asymptomatic. Uretric obstruction hadbeen resolved in 132 ureters after a mean of 152 days of treatment.Almost 30 % reduction in the fibrotic mass size was achieved in82 % of patients.
Glucocorticoids is the the mainstay of treatment. Therenal function tests, of the vast majority of patients, normalizedwith treatment. Relapse may occur, so a follow-up over a longperiod of time is required. A high index of suspicion is neededfor diagnosis in asymptomatic patients.

OBJECTIVE: The outcome of CT-guided biopsy in patients with suspected retroperitoneal fibrosis (RF), regarded as technically challenging, remains unclear. This study aimed to evaluate the results of CT-guided biopsy in patients with lesions considered in the differential diagnosis of RF and compare them with results from patients with other retroperitoneal lesions.
METHODS: Patients who underwent CT-guided biopsy of retroperitoneal lesions between January 2010 and September 2018 were retrospectively reviewed. The study cohort with retroperitoneal lesions surrounding the infra-abdominal aorta, iliac vessels, and/or ureters was divided into two groups: Group F included patients with lesions for which RF was considered in the differential diagnosis, and Group C comprised patients with a retroperitoneal mass or lymphadenopathy. Lesion size and depth, and biopsy details including technical success, position, procedure time, diagnostic yield, and complications between the two groups were compared.
RESULTS: Group F included 27 patients (mean age 68.5 years ± 11.6, 17 male) and Group C 30 patients (mean age 65.0 years ± 11.8, 16 male). The short axis was significantly smaller in Group F than in Group C (19 mm vs 26 mm, P = 0.041), and procedure time was significantly longer in Group F than in Group C (31.5 min ± 13.2 vs 20.3 min ± 8.4, P = 0.001). Technical success rate (93% vs 100%), accuracy (93% vs 93%), and complications (3.7% vs 10%) between Groups F and C were not significantly different.
CONCLUSIONS: CT-guided biopsy of patients with suspected RF is considered safe and effective.

Objectives: IgG4-related disease (IgG4-RD) may present as \'idiopathic\' retroperitoneal fibrosis (IRPF). We aimed to determine the occurrence of IgG4-retroperitoneal fibrosis (IgG4-RPF) in a nationwide study on patients with newly diagnosed IRPF, and to compare histopathological, imaging, and clinical features in the IgG4-RPF and non-IgG4-RPF subsets. Method: The National Danish Pathology Register was searched for biopsy codes relating to retroperitoneal tissue from 1 January 2004 to 31 December 2013. Secondary causes of RPF were excluded. Among 724 candidate cases, 68 were identified with IRPF. Clinical, laboratory, and imaging recordings were reviewed, and tissue blocks were scrutinized for IgG4-RPF features according to international consensus. Results: Forty-two patients (28 males), median age 56 (25-74) years were included. Nineteen (45%) met the criteria for IgG4-RPF, seven with definite and 12 with possible IgG4-RPF, while 23 had non-IgG4-RPF. Local manifestations and laboratory measures did not differ between RPF subsets. Arterial hypertension (p = 0.037) and periaortic fibrosis (p = 0.024) were more common in IgG4-RPF vs non-IgG4-RPF. Plasma cell IgG4/total IgG ratios ≥ 40% were associated more with core histopathological features of IgG4-RD compared to ratios < 40% (p < 0.001). There was a positive correlation between tissue IgG4-positive plasma cells and eosinophil cell count in patients with IgG4-RPF (rho = 0.50, p = 0.043). Conclusion: Forty-five per cent of this nationwide study population with newly diagnosed IRPF could be reclassified with IgG4-RPF. The association between high numbers of IgG4-bearing plasma cells and histopathological features of IgG4-RPF supports IgG4-bearing plasma cells with a perturbed distribution between IgG4 and total IgG being implicated in the pathogenesis of IgG4-RPF.

BACKGROUND: Retroperitoneal fibrosis is a rare disease, typically with an insidious and various clinical course. The peak incidence is seen in patients 40 to 60 years of age and mostly in man. The characteristic finding in this disease is a periaortic fibrous mass that often surrounds the ureters. The diagnostic approach remains uncodified. We aimed to determine the different clinical, radiological and biological aspects of retroperitoneal fibrosis.
METHODS: Retrospective multicenter study of 32 retroperitoneal fibrosis cases hospitalized between 1999 and 2014 in the Internal Medicine Department and Urology Department in the university hospital center Sahloul Sousse.
RESULTS: There were 24 men and 8 women with a mean age of 58 years. The lumbar pain is the most common clinical signs (53.1%). An inflammatory syndrome and renal failure were the most common biological signs. The diagnosis was suspected on data from the abdominal ultrasound and confirmed by pelvic CT scan that showed a periaortic fibrous mass that often surrounds the ureters. Histological analysis of a surgical biopsy specimen was performed in only eight cases.
CONCLUSIONS: The most common mode of presentation of retroperitoneal fibrosis remains lumbar pain with renal failure and a high sedimentation rate. Although abdominal ultrasound may contribute to the general evaluation of patients with retroperitoneal fibrosis, CT-scanner is the preferred imaging method. The imaging capability of magnetic resonance and the TEP-scan may facilitate assessment of disease extent.
METHODS: 4.