Clinical characteristics of IgG4-related retroperitoneal fibrosis versus idiopathic retroperitoneal fibrosis.

Retroperitoneal fibrosis (RPF) is an uncommon condition characterized by inflammation and fibrosis in the retroperitoneal space. More than two-thirds of RPF are idiopathic, with the remaining stemed from a variety of secondary causes. It was suggested that IgG4-related RPF is a secondary form of RPF. We undertook this study to compare detailed demographic, clinical and laboratory characteristics of IgG4-related RPF and IRPF in a large Chinese cohort. We retrospectively reviewed the medical records of 132 RPF patients diagnosed at Peking University People's Hospital between March 2010 and March 2018. Among the 132 patients, the mean age at disease onset was 54.8 years. IgG4-related RPF group showed greater male predominance compared to IRPF group. IgG4-related RPF patients showed a longer interval between symptom onset and diagnosis, and allergic diseases were more common in this group. Sixty-four patients (48.4%) had lower back pain, which was more common in IRPF group than that in IgG4-related RPF patients. In terms of organ involvement, although 42 of 47 patients (89.3%) with IgG4-related RPF had other organ involvement, there were no patients in the IRPF group with other organ involvement. In addition, the serum IgG4 level, elevated eosinophils counts and IgE level were significantly higher in IgG4-related RPF patients. We described the demographic, clinical and laboratory differences between IgG4-related RPF and IRPF patients, indicating their potential differences in pathogenesis, which was of great importance to diagnose and manage the two phenotypes.

IgG4-related disease in patients with newly diagnosed idiopathic retroperitoneal fibrosis: a population-based Danish study.

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.

[Retroperitoneal fibrosis in adults: Diagnostic approach based on a retrospective multicenter study on 32 cases]. / Fibrose rétropéritonéale de l'adulte : approche diagnostique à partir d'une série rétrospective multicentrique de 32 cas.

INTRODUCTION: 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. PATIENTS AND 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. CONCLUSION: 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. LEVEL OF EVIDENCE: 4.

A disclosed diagnosis for 24 year's unknown illness.

IgG4-related disease (IgG4-RD) is a newly described illness over the last several years. A 57-year-old man, who had been followed for chronic kidney disease (CKD), chronic pancreatitis and history of operated cholangitis, was admitted to our hospital for abdominal pain and worsening renal function. Serum levels of IgG and IgG4 were elevated. CT scan showed the characteristic findings of IgG4-related retroperitoneal fibrosis, pancreas and kidney disease. An endoscopic biopsy revealed the finding compatible with IgG4-RD. Steroid therapy led to the remission of his abdominal pain. Patients with CKD of unknown aetiology may have IgG4-RD.

Overlap of Post-obstructive Diuresis and Unmasked Diabetes Insipidus in a Case of IgG4-related Retroperitoneal Fibrosis and Tuberoinfundibular Hypophysitis: A Case Report and Review of the Literature.

The clinical picture of IgG4-related disease (IgG4-RD) is diverse because various organs can be affected. We describe the case of a 56-year-old man with acute renal failure and tuberoinfundibular hypophysitis due to IgG4-RD. Steroid therapy lowered the serum IgG4 level and ameliorated renal dysfunction, bilateral hydronephrosis and retroperitoneal fibrosis. However, polyuria from post-obstructive diuresis and unmasked central diabetes insipidus ensued. The patient's polyuria continued despite the administration of a therapeutic dose of glucocorticoid; the patient's pituitary swelling and anterior pituitary dysfunction were partially ameliorated. The pituitary swelling recurred seven months later. In patients with IgG4-RD, the manifestation of polyuria after steroid therapy should prompt suspicion of post-obstructive diuresis and the unmasking of central diabetes insipidus.

[Retroperitoneal fibrosis: about 12 cases]. / La fibrose rétropéritonéale: à propos de 12 cas.

Retroperitoneal fibrosis (RPF) is rare. It is characterized by the gradual transformation of retroperitoneal adipose tissue into a fibrous mass surrounding the aorta, the inferior vena cava and the urinary tract responsible for progressive alteration in the renal function. It usually manifests as lumbar pain associated with renal failure and biologic inflammatory syndrome. We report 12 cases of retroperitoneal fibrosis in order to determine its clinical, radiological and therapeutic features. We conducted a retrospective study of 12 patients with retroperitoneal fibrosis whose medical data were collected in the Departement of Urology at the University Hospital Hassan II, Fes over a period of 9 years (2005-2013). The study involved ten men and two women. Clinical symptomatology was very variable, dominated by lumbar pain in all patients and hydrocele in 1 patient. Laboratory tests showed renal failure in all patients and inflammatory syndrome in 10 patients. The diagnosis of retroperitoneal fibrosis was suspected in all patients on ultrasound showing obstruction of the upper urinary tract without visible obstacle. The diagnosis was confirmed by abdominal CT scan without contrast that objectified a retroperitoneal tissue lesion enveloping the vessels and the urinary tract. Nine patients had idiopathic retroperitoneal fibrosis. Two patients had perianeurysmal fibrosis and one patient had post-radiation fibrosis. All patients underwent urinary drainage by double J. ureteral catheter. Seven patients underwent corticosteroid therapy. Six patients had an improvement of clinical and laboratory test outcome with disappearance of pain and improvement of the general condition. This study confirms the rarity of retroperitoneal fibrosis, the difficulty of diagnosis, pain associated with inflammatory syndrome and renal failure as common manifestations. Abdominal CT scan without contrast is the gold standard for definitive diagnosis. Urinary drainage is essential in most cases as well as regular monitoring of patients.

Monitoring Disease Activity in Patients with Aortitis and Chronic Periaortitis Undergoing Immunosuppressive Therapy by Perfusion CT.

RATIONALE AND OBJECTIVES: To evaluate the role of perfusion CT for monitoring inflammatory activity in patients with aortitis and chronic periaortitis undergoing immunosuppressive therapy. MATERIALS AND METHODS: Seventeen symptomatic patients (median age 68.5 years) who underwent perfusion-based computed tomography (CT) monitoring after diagnostic contrast-enhanced CT were retrospectively included in this study. Blood flow (BF), blood volume (BV), volume transfer constant (k-trans), time to peak, and mean transit time were determined by setting circular regions of interest in prominently thickened parts of the vessel wall or perfused surrounding tissue at sites where the perfusion CT color maps showed a maximum BF value. Differences in CT perfusion and, morphological parameters, C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR) were tested for significance during therapy. RESULTS: In all patients BF and BV dropped at second perfusion CT (P < 0.05). In aortitis patients, CRP dropped from 3.86 ± 5.31 mg/dL to 0.9 ± 1.37 mg/dL and in periaortitis patients from 1.78 ± 2.25 mg/dL to 0.79 ± 1.55 mg/dL, whereas ESR dropped from 45.71 ± 37.59 seconds to 8.57 ± 3.1 seconds and 36.78 ± 34.67 seconds to 17.22 ± 21.82 seconds in aortitis and in periaortitis, respectively. CONCLUSIONS: The course of perfusion CT parameters in aortitis and chronic periaortitis undergoing immunosuppressive therapy dropped at different extent after therapy.

Combination of Steroids and Azathioprine in the Treatment of Ormond's Disease--A Single Centre Retrospective Analysis.

We present a retrospective analysis of patients treated in our Department of Clinical Biochemistry, Haematology and Immunology, Na Homolce Hospital, during 1997-2013 for Ormond's disease. We analyse the clinical history, diagnostic approaches, surgical, and immunosuppressive therapies and their subsequent effect on our patients. 28 patients treated for Ormond's disease were included. Patients with established disease activity (26 patients) were given immunosuppressive treatment, using corticosteroids in combination with azathioprine. Treatment response was evaluated using clinical symptomatology, inflammatory parameters and imaging methods. In the cohort as a whole, immunosuppressive therapy was applied in 26 patients; in two patients it was not used as no inflammatory activity was found with the disease. In all 26 patients, computed tomography showed that immunosuppressive treatment resulted in partial or complete regression of inflammatory infiltrate. Out of the total number of 26 patients, two patients experienced disease exacerbation 7 and 16 months after the immunosuppressive treatment was discontinued. The longest follow-up period was 16 years; the shortest one was 21 months. Idiopathic retroperitoneal fibrosis--Ormond's disease--is a disease with serious complications. Standard treatment involves a combination of surgery and immunosuppressive treatment. The combination of corticosteroids and azathioprine represents a potentially safe and useful method of treatment.

Computed tomography parameters can be used as predictive markers for the improvement of renal function in patients with retroperitoneal fibrosis.

OBJECTIVES: To discover the relationship between computed tomography (CT) parameters of retroperitoneal soft tissue of untreated retroperitoneal fibrosis patients and the variation of renal function after treatment. METHODS: Data were collected retrospectively from 42 patients with established diagnosis of retroperitoneal fibrosis, at the Department of Rheumatology in Peking University First Hospital from May 2009 to April 2015. The demographic information, clinical characteristics, laboratory data, treatment and general therapeutic response were collected. The CT parameters such as radial lines, Hounsfield unit values, radiographic classification at baseline before the initiation of therapy were measured. Then the correlations between baseline CT parameters and clinical data were analysed. RESULTS: The Hounsfield unit values of venous phase and delayed phase at baseline were negatively correlated with the change of estimated glomerular filtration rate (eGFR) at the 12th month after the initiation of medications. The baseline transverse diameters of the retroperitoneal soft tissue were larger in the group of eGFR <60 ml/min/1.73 m² compared with the group of eGFR ≥ 60 ml/min/1.73 m² at the 12th month, while the vertical diameters and maximum cross-section thickness were not. The baseline transverse diameters were also significantly correlated with the duration of intubation. CONCLUSIONS: The baseline Hounsfield unit values and baseline transverse diameters of soft tissue of untreated retroperitoneal fibrosis patients may be used to predict the renal function after 12 months of therapy.

IgG4-related periaortitis vs idiopathic periaortitis: is there a role for atherosclerotic plaque in the pathogenesis of IgG4-related periaortitis?

OBJECTIVE: The diagnosis of (isolated) IgG4-related periaortitis is often based on elevated serum IgG4 levels since in tissues such as the aorta, biopsies cannot be easily performed. However, the role for serum IgG4 as a biomarker for IgG4-related periaortitis is indistinct. The main purpose of our study was to identify clinical differences between periaortitis with elevated vs normal serum IgG4 levels. METHODS: A retrospective study was performed on 68 non-infectious periaortitis patients. We compared demographic, clinical, biochemical and radiological data in patients with elevated serum IgG4 levels with data from patients with normal serum IgG4 levels. The calcium content of the aortic wall was calculated to determine the amount of atherosclerosis. RESULTS: After applying exclusion criteria, our study population consisted of nine IgG4-related periaortitis patients and eight idiopathic periaortitis patients. Striking significant differences were male predominance (P = 0.001) and multifocal organ involvement (P = 0.004) in IgG4-related periaortitis patients compared with the idiopathic periaortitis group. The calcium content of the total aortic wall was significantly higher in IgG4-related periaortitis patients (P = 0.005). No other significant differences were found. CONCLUSION: Elevated serum IgG4 levels, male gender, a higher calcium content of the aortic wall and multifocal organ involvement are features that might provide a higher probability for IgG4-related periaortitis compared with idiopathic periaortitis. Our study results might be compatible with the hypothesis of Mitchinson and Parums that atherosclerotic plaque plays a role in the pathogenesis of chronic periaortitis.

Early onset of periaortic inflammation after EVAR with inferior vena cava involvement: a case report.

Endovascular treatment of abdominal aortic aneurysm (EVAR) represents a good alternative to open surgery, also in patients who present inflammatory abdominal aortic aneurysm, resulting in reduction of the inflammatory process in many cases. Instead, the onset of periaortic inflammation after EVAR is a rare event with an unclear pathogenesis, time of onset, and clinical presentation. This is a case report of a very early onset of periaortitis after EVAR with inferior vena cava involvement and stretching, resulting in lower limb swelling and back pain, treated by corticosteroid drug with a good remission of the pathology.

[Idiopathic retroperitoneal fibrosis: a multicentric retrospective study of 30 French cases and follow-up of the renal function]. / Fibrose rétropéritonéale idiopathique : évolution à long terme du pronostic rénal dans une série rétrospective multicentrique de 30 cas.

PURPOSE: Idiopathic retroperitoneal fibrosis (IRF) is an inflammatory disorder, affecting the aorta and the surrounding vessels and tissues. The prognosis is mainly driven by the risks of chronic kidney disease and relapse. Our aim was to assess the prevalence of chronic kidney disease at follow-up. METHODS: We retrospectively reviewed the medical records of patients diagnosed for IRF in Seine-Saint-Denis (France) between 1987 and 2011. We collected informations about presentation, radiologic findings and follow-up. Diagnosis of IRF was confirmed when all the following criteria were met: infiltration of the infrarenal aorta or iliac vessels, absence of aneurysmal dilation, lack of clinical suspicion of malignancy. RESULTS: Thirty patients were identified, with a male/female ratio of 4.9. Mean age was 55±13 years old. The mean creatinine clearance was 66 mL/min/1.73 m(2) and the mean CRP was 45±36 mg/L. In 24 (80%) patients, the location of IRF was periaortic and periiliac. Eleven patients (37%) underwent a diagnostic biopsy, and 14 (47%) required an ureteral procedure. A mean follow-up of 63 months was available for 29 patients: 69% relapsed, 7 developed chronic renal disease (24%), and one died of urinary sepsis. Older age (P=0.023), diabetes (P=0.007), and initial renal insufficiency (P=0.05) were associated with a risk of chronic renal insufficiency. CONCLUSION: The high frequency of relapses and chronic renal disease emphasizes the need of close follow-up in patients diagnosed with IRF.

Exercise right heart catheterization for inferior vena cava obstruction: confirming the hemodynamic significance of an anatomic lesion.

Retroperitoneal fibrosis is a rare condition characterized by the presence of inflammatory and fibrous retroperitoneal tissue that surrounds and often encases abdominal structures. In this report we describe an unusual presentation of retroperitoneal fibrosis and inferior vena cava (IVC) obstruction that was unidentified by noninvasive testing and ultimately diagnosed using exercise right heart catheterization and venography. Exercise hemodynamic catheterization revealed a high pressure gradient across the IVC obstruction during exercise that resulted in reduced preload and inappropriately low cardiac output reserve. The patient ultimately underwent angioplasty and stenting of the obstruction resulting in resolution of his symptoms.

[Ormond's disease complicated by infectious endocarditis].

A case of retroperitoneal fibrosis (Ormond's disease) is reported. It is known to be usually diagnosed at the late stages of renal complications Urinary infection ended in infectious endocarditis caused by E. faecium showing multiple resistance to antibiotics. Moderate immunosuppressive and modern antibacterial (daptomycin) therapy ensured remission of both Ormond's diseases and endocarditis.

Retroperitoneal fibrosis.

Retroperitoneal fibrosis (RPF) is a condition characterized by the presence of inflammation and fibrosis in the retroperitoneal space, for which no standard diagnostic criteria exist. Historically, treatment has focused on relieving the obstruction with percutaneous or cystoscopic assisted placement of ureteral stents followed by more definitive resolution of ureteric obstruction with open or laparoscopic ureterolysis. However, over the past several years management has shifted from primarily a surgical approach to an immunosuppressive-based therapy aimed at modulation of the immune system. This review focuses on the recent advances in the classification, epidemiology, pathophysiology, pathology, imaging, and treatment of RPF.