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IgG4 related disease is a term used to describe a fibroinflammatory condition characterized by storiform fibrosis, inflammation with a dense lymphoplasmocytic infiltrate rich in plasma cells expressing IgG4, and often, if not always, raised serum levels of IgG4. We report a case of a patient with a past history of retroperitoneal fibrosis presenting with a swollen left testicle, who underwent an orchidectomy due to suspicion of malignancy. The surgical specimen revealed a paratesticular pseudotumor with histopathological and immunohistochemical characteristics of IgG4 related disease. To the best of our knowledge, just nine such cases have previously been reported, of which only three were manifestations of systemic disease, as in the present case. Whilst it is important to recognize the clinical and radiological features of this entity, histopathology is often essential in order to confirm the diagnosis.

Retroperitoneal fibrosis (RPF) is a rare disease associated with the formation of hard inflammatory and fibrous tissue in the retroperitoneum. Taking into consideration the fact that RPF is a rare disease with different subtypes, we compared the basal clinical and biochemical characteristics of the vascular and urorenal subtypes.From January 2005 until December 2021, 27 patients were identified as vascular subtype (18 males) and 11 as urorenal subtype (9 males).Patients with a primary urorenal origin had significantly worse kidney function as reflected by serum creatinine and eGFR (bothVascular subtype is more prevalent in our study with more cardiovascular risk factor present. Due to the diversity of symptoms, diagnosis of RPF becomes a challenge for specialists as well as therapy.

Gastric signet-ring-cell adenocarcinoma (SRCC) is a rare disease entity, often characterized by early age of the onset and sometimes attributable to heritable genetic mutations. Overall prognosis is usually poor due to diagnosis at late stages. There are a handful of case reports that describe patient presentation with retroperitoneal fibrosis secondary to malignancy from a concurrent gastric SRCC found on the workup. No information exists on timing from primary tumor development to retroperitoneal disease. Further, there is speculation that gastric SRCC may have an indolent phase prior to symptomatic disease, but its natural history is essentially entirely unknown. In this case report, we describe a 39-year-old male with an incidentally discovered gastric SRCC who then underwent multimodality treatment with curative intent. No evidence of recurrence was documented on interval surveillance scans for 4.5 years, at which point, he rapidly developed a large retroperitoneal mass that was biopsied for metastatic disease. He succumbed to his pathology within 6 months. This presentation suggests that gastric SRCC could have both a relatively long indolent phase and an unpredictable propensity for explosive metastatic progression. Tumor biology factors that affect this balance are not understood.

To evaluate the clinical and pathological features of IgG4-related and non-IgG4-related idiopathic retroperitoneal fibrosis (IRF) according to the latest classification criteria for IgG4-related disease in 2019.Patients with IRF confirmed by histological examination from our hospital between 2000 and 2020 were selected in this study. Medical records of all patients were reviewed by independent researchers. Retroperitoneal specimens were obtained for hematoxylineosin staining, elastic-collagenous fiber staining, and immunohistochemical analysis. The clinical and pathological features between IgG4-related and non-IgG4-related IRF were analyzed.A total of 105 patients were included with 77 in the IgG4-related group and 28 in non-IgG4-related group. The ratio of male to female patients and the incidence of acute renal failure were significantly higher in the IgG4-related group than in the non-IgG4-related group. Elevated erythrocyte sedimentation rate and C-reactive protein were more common and the recurrence rate was significantly higher in the IgG4-related group than in the non-IgG4-related group. Radiographically, the ureter was more easily involved by retroperitoneal soft tissue in the IgG4-related group. Histologically, there were no significant differences in the incidence of dense lymphoplasmacytic infiltration, storiform fibrosis, and obliterative phlebitis between the two groups except for the IgG4 staining.Idiopathic retroperitoneal fibrosis can be classified into IgG4-related and non-IgG4-related subtypes. There were no significant pathological differences between the two subtypes of IRF, except for the IgG4 staining. Patients with the IgG4 subtype tended to be more likely to be male, have a higher inflammatory index, and be more likely to have recurrence.

Background: Retroperitoneal fibrosis (RPF) is a rare disease characterized by the presence of inflammatory and fibrous retroperitoneal tissue that often encircles abdominal organs including the aorta and ureters. Data on the incidence of this disease are limited. Summary: The disease may be idiopathic or secondary to infections, malignancies, drugs, or radiotherapy. The idiopathic form is an immune-mediated entity and a part of the broader spectrum of idiopathic diseases termed chronic periaortitis, characterized by a morphologically similar fibroinflammatory changes in the aorta and surrounding tissues. Taking into account the dominant symptoms and clinical characteristics of patients with periaortitis, 2 subtypes of disease could be distinguished. The vascular subtype includes patients with nondilated aorta or with inflammatory abdominal aortic aneurysm, both with and without involvement of adjacent structures and with numerous risk factors for atherosclerosis. In the renoureteral subtype, obstructive uropathy manifesting with hydronephrosis and acute kidney injury is the predominant finding. Due to the variety of symptoms, diagnosis of RPF remains challenging, difficult, and often delayed. A series of diagnostic tests should be performed, in order to confirm the diagnosis idiopathic RPF. Laboratory workup includes evaluation of inflammatory indices and immunological studies. A biopsy and histopathological evaluation may be necessary to confirm diagnosis and differentiate the disease. Computed tomography, magnetic resonance imaging, and positron emission tomography are the modalities of choice for the diagnosis and follow-up of this disease. Management of ureteral obstruction, hydronephrosis, and aortic aneurysms often requires surgical evaluation and treatment. The pharmacological treatment of RPF has been evaluated in a few randomized trials and is mainly based on observational studies. Steroid therapy remains the gold standard of treatment. Key Messages: Nowadays, multidisciplinary team approach with clinical and diagnostic experience in both primary and secondary RPF as well as 2 major subtypes should be offered. Centers specialized in rare diseases with collaboration with other units and referral system yield the best possible outcomes.

Забрюшинный (ретроперитонеальный) фиброз (РПФ) — редкое заболевание, характеризующееся разрастанием воспалительной и фиброзной ткани в забрюшинном пространстве. Обычно эти разрастания локализуются вокруг инфраренальной части брюшной аорты и подвздошных артерий, часто покрывая мочеточники или другие органы брюшной полости. Идиопатические заболевания составляют 70 % случаев. РПФ может быть ассоциирован с иммуноглобулином G4 (IgG4), что является причиной двух третей случаев идиопатического РПФ. Вторичный РПФ может развиться вследствие инфекций, злокачественных новообразований, приема лекарственных препаратов, забрюшинного кровотечения или других заболеваний. Описан клинический случай идиопатического РПФ, вероятно, ассоциированного с IgG4, у пациента, прошедшего симптоматическое оперативное лечение в урологической клинике и продолжившего лечение у ревматолога патогенетической терапией (метилпреднизолон и мофетила микофенолат) со значительным улучшением. Для ревматологов данная патология представляет безусловный клинический интерес, поскольку, с одной стороны, в основе данных изменений лежат процессы, аналогичные таковым при системных заболеваниях соединительной ткани, а с другой стороны — это редкая патология, встречаемость РПФ составляет от 0,1 до 1,3 случая на 100 000 человек в год. Однако чаще такие пациенты сразу попадают на прием к врачам смежных специальностей (урологам, нефрологам, хирургам, сосудистым хирургам), что подчеркивает важность разбора такого клинического случая.

Intraperitoneal adhesions complicate over half of abdominal-pelvic surgeries with immediate, short, and long-term sequelae of major healthcare concern. The pathogenesis of adhesion development is similar to the pathogenesis of wound healing in all tissues, which if unchecked result in production of fibrotic conditions. Given the similarities, we explore the published literature to highlight the similarities in the pathogenesis of intra-abdominal adhesion development (IPAD) and other fibrotic diseases such as keloids, endometriosis, uterine fibroids, bronchopulmonary dysplasia, and pulmonary, intraperitoneal, and retroperitoneal fibrosis. Following a literature search using PubMed database for all relevant English language articles up to November 2020, we reviewed relevant articles addressing the genetic and epidemiological similarities and differences in the pathogenesis and pathobiology of fibrotic diseases. We found genetic and epidemiological similarities and differences between the pathobiology of postoperative IPAD and other diseases that involve altered fibroblast-derived cells. We also found several genes and single nucleotide polymorphisms that are up- or downregulated and whose products directly or indirectly increase the propensity for postoperative adhesion development and other fibrotic diseases. An understanding of the similarities in pathophysiology of adhesion development and other fibrotic diseases contributes to a greater understanding of IPAD and these disease processes. At a very fundamental level, blocking changes in the expression or function of genes necessary for the transformation of normal to altered fibroblasts may curtail adhesion formation and other fibrotic disease since this is a prerequisite for their development. Similarly, applying measures to induce apoptosis of altered fibroblast may do the same; however, apoptosis should be at a desired level to simultaneously ameliorate development of fibrotic diseases while allowing for normal healing. Scientists may use such information to develop pharmacologic interventions for those most at risk for developing these fibrotic conditions.

Background and objectives Although arterial involvement for advanced tumors is rare, vascular resection may be indicated to achieve complete tumor resection. Given the potential morbidity of this approach, we sought to evaluate perioperative outcomes, vascular graft patency, and survival among patients undergoing tumor excision with en bloc arterial resection and reconstruction. Methods From 2010 to 2020, we identified nine patients with tumors encasing or extensively abutting major arterial structures for whom en bloc arterial resection and reconstruction was performed. Results Mean age was 53 ± 20 years, and 89% were females. Diagnoses were primary sarcomas (5), recurrent gynecologic carcinomas (3), and benign retroperitoneal fibrosis (1). Tumors involved the infrarenal aorta (2), iliac arteries (6), and superficial femoral artery (1). Three patients (33%) had severe perioperative morbidity (Grade III + ) with no mortality. At a median follow-up of 23 months, eight patients (89%) had primary graft patency, and five patients (56%) had no evidence of disease. Conclusions Arterial resection and reconstruction as part of the multimodality treatment of regionally advanced tumors is associated with acceptable short- and long-term outcomes, including excellent graft patency. In appropriately selected patients, involvement of major arterial structures should not be viewed as a contraindication to attempted curative surgery.

Summary Objectif Evaluate kidney autotransplantation (KAT) and ileal ureter substitution (IUS) practice and outcome as alternatives to indwelling ureteral stents for the management of long ureteral stenosis (US). Material We included all patients treated for US with KAT or IUS in 5 French university urology centers between 2010 and 2018. We excluded US due to urothelial carcinoma. Primary endpoint was the preservation of ipsilateral kidney and renal function without any urinary diversion. Results 22 patients were treated with KAT (n = 8, 36.4%) and IUS (n = 14, 63.6%). Mean US length was 4.6 cm and 6 cm (P = 0.52) in KAT and IUS groups respectively. US etiologies were lithiasis, iatrogenic, retroperitoneal fibrosis or extrinsic compression. US level was varied. The surgery was described as difficult because of peritoneal adhesions or major peri-ureteral fibrosis. Mean operating time and hospital stay were 336 and 346 minutes (P = 0.87) and 8 and 15 days respectively (P = 0.001). Postoperative complications were mostly Clavien ≤ 2 (n = 17, 77.3%). Revision surgery was required in the KAT group in 3 cases (37.5%), for textiles, renal vein thrombosis and anastomotic leak, none in the IUS group. The mean follow-up was 15.7 months. All but one (in the KAT group) ipsilateral kidneys were preserved, without renal function impairment (Δcreat +2.1 vs. +2.4 μmol/l respectively, P = 0.67), nor urinary diversion. Conclusion KAT and IUS are safe alternatives whose indication depends on surgeons expertise. Our study pointed out the scarcity of this practice suggesting the need to refer patients to expert centers. Level of evidence 3.

IgG4-related disease (IgG4-RD) is a systemic, immune-mediated fibro-inflammatory disease that can affect virtually every organ system. It is usually insidious in onset and often mimics malignant or other inflammatory disorders. Diagnosis frequently requires a combination of clinical, serological, radiographic, and histopathological features, including increased serum-IgG4 levels and tissue infiltration of IgG4-positive plasma cells with associated fibrosis. Unlike more frequently affected sites, including the hepatobiliary system, salivary glands and retroperitoneum, pericardial involvement of IgG4-RD has only rarely been described. We report the case of a 76-year-old woman presenting with refractory pericarditis and imminent cardiac tamponade, successfully treated with therapeutic pericardiectomy. A diagnosis of IgG4-RD was made based on elevated serum-IgG4 levels and the presence of typical pericardial histopathological findings, meeting all 3 of the 2011 comprehensive diagnostic criteria for IgG4-RD. Following pericardiectomy, the patient remained in remission without a need for glucocorticoids or additional immunosuppressive therapy. Adding to this case, we reviewed the literature for previously described cases of IgG4-RD presenting with pericarditis and described their characteristics and the available treatment options. Our case-based literature review provides a clear overview of the diagnostic process for IgG4-RD and the need to apply classification criteria with the necessary caution, particularly in the case of rare disease manifestations, including pericarditis. ispartof: RHEUMATOLOGY INTERNATIONAL vol:42 issue:7 pages:1287-1295 ispartof: location:Germany status: published

Pulmonary hyalinizing granuloma (PHG) is an extremely rare benign mass-forming fibrosclerotic lesion of the lung with unknown etiology. Although PHG mimics malignancies in clinical and radiological examinations, concurrence with primary lung carcinoma has never been reported. A 67-year-old asymptomatic man was presented with a pulmonary nodule incidentally detected during follow-up for retroperitoneal fibrosis. Chest CT confirmed a 1.5-cm solitary subpleural nodule in the upper left lung without lymphadenopathy. Fluorodeoxyglucose positron emission tomography (FDG-PET) indicated low uptake of FDG (maximum standardized uptake value, 3.0) in the same site, and the patient underwent wedge resection with suspected primary lung cancer. Intraoperative frozen section biopsy of the lesion revealed histopathological features of PHG without malignancy, and no additional surgical treatments were considered necessary; the operation was completed at this point. However, postoperative thorough examination of permanent sections from the resected specimen revealed a concurrent adenocarcinoma component, sized 0.6 cm, which was forming a composite tumor with PHG; the patient subsequently underwent a second surgery including a completion left upper lobectomy and regional lymph node dissection. The rarity of disease and the unknown etiological connection between PHG and co-existing carcinomas may cause a diagnostic pitfall and obscure clinical treatment strategies for patients. Diagnosis of PHG in small biopsy samples should be made with caution, especially when diagnosed by intraoperative consultation (frozen section) aimed to exclude malignancies.

A 71-year-old Japanese man with progressive kidney failure was referred to our hospital. Laboratory tests showed elevated IgG4 levels. Contrast-enhanced computed tomography (CT) revealed soft tissue surrounding the left kidney and right atrophic kidney. A histopathological examination revealed inflammation and fibrosis with rich IgG4-positive cells in the thickened kidney capsule, but not in the kidney parenchyma. Poor enhancement in the left kidney on contrast-enhanced CT and wrinkling of glomerular capillaries in pathological tissues were also observed. These findings indicated IgG4-related perirenal lesions leading to low renal perfusion and kidney failure. The perirenal lesions and kidney failure were improved by corticosteroid therapy.

The first case of eosinophilic granulomatosis with polyangiitis (EGPA) simultaneously demonstrating various clinical manifestations, including retroperitoneal fibrosis (RPF) causing hydronephrosis and membranous nephropathy (MN) leading to nephrotic syndrome, is presented. There have been no previous case reports demonstrating the simultaneous onset of these three disease categories with significant complex pathologies. This case was successfully managed by providing adequate combination therapies according to each disease category, leading to complete remission (CR) of all three diseases. In conclusion, we believe this case is extremely rare and clinically suggestive, and that these findings can be applied to a future phenotype-tailored treatment strategy for EGPA.

Idiopathic retroperitoneal fibrosis is a rare fibro-inflammatory disease of varied etiology which usually originates around aorta and spreads caudally along Iliac vessels into adjacent retroperitoneum causing ureteral obstruction as the most frequent complication. A 53-year-old male patient presented with complaint of mild pain in both the legs off and on. On investigating further, we found that he had been struggling with intermittent relapses every 3-4 years for last 20 years since he was first diagnosed with Idiopathic Retroperitoneal Fibrosis. He was 33-year-old when he first developed the symptoms of anuria for 48 hours and was diagnosed with Idiopathic retroperitoneal fibrosis. This was followed by atrophy of left kidney and hypertension 6 years later, then hypothyroidism after another 3years and finally involvement of Inferior Vena Cava and acute Deep Vein Thrombosis of lower limbs after another 3-4 years. His deep vein thrombosis was well managed in time. He was put on glucocorticoids everytime he had a relapse and a complication. We did a review of literature to understand recent advances about its pathogenesis, diagnosis, investigations and management. We searched in PubMed using terms like retroperitoneal fibrosis alone and in combination with related terms such as Inferior Vena Cava thrombosis, Deep Vein Thrombosis, Tamoxifen, Methotrexate. This case is unique as it is very rare to find acute Deep Vein Thrombosis in Idiopathic retroperitoneal fibrosis without development of any collaterals when Inferior Vena Cava lumen is compromised to almost complete obstruction. After a follow up of 20 years patient is doing well in terms of physical activity and psychological wellbeing with anti-hypertensives, thyroxine and anti-coagulants. Is the disease-free interval actually free of the disease or it just subsided with immunosuppressants to become active after some time?

OBJECTIVE To present long-term outcome of Yang-Monti ileal ureter, with a focus on patients with mild/moderate loss of kidney function and solitary kidney. PATIENTS AND METHODS Between March 2001 and December 2019, Yang-Monti ileal ureter was performed on 36 patients with ureteric defects and median age 46.5 years. Of these, 4, 14, 15 and 3 patients had stage 1, stage 2, stage 3a and stage 4a chronic kidney disease, respectively; 6 had solitary kidney. Patients were regularly followed for complications, morphological, and functional outcome. RESULTS Ureteric stricture etiology was iatrogenic (16), Bilharzial (7), tuberculous (4), retroperitoneal fibrosis (5), malignancy (3), and gunshot injury (1). The median (range) ureteric defect length was 11 (8-16) cm. Four grade 1/2 postoperative Clavien-Dindo complications were noted. Median follow-up was 68 months (range 12-215). Intestinal obstruction developed in 1 patient and urinary tract infection in 10. At last follow-up, serum creatinine, split renographic clearance, and estimated glomerular filtration rate showed significant improvement compared to preoperative values, in the whole series, in cases with chronic kidney disease (stages 2, 3a and 3b) and solitary kidney. Four cases with chronic kidney disease (stage 3) showed deterioration of the kidney function parameters. Magnetic resonance urography showed improvement of hydronephrosis in most patients. No metabolic complications were noted. CONCLUSION Yang-Monti Ileal ureter is durable and effective in improving kidney function with few complications. It can be safely used in cases of mild/moderate kidney function loss and solitary kidney. A threshold eGFR

Objectives The aim of this report was to describe the clinical signs, diagnostic imaging findings, surgical management, histopathological findings, outcome and possible risk factors for cats that developed retroperitoneal fibrosis (RPF) following renal transplantation. Methods Medical records of cats that underwent renal transplantation and developed clinically significant RPF between 1995 and 2019 were reviewed. Results Eighty-one cats underwent 83 renal transplantations. Of these 81 cats, six developed clinically significant RPF. For all six cats, renal transplantation was performed using cold organ preservation solution and ureteral papilla implantation. Immunosuppression protocol included ciclosporin and prednisolone. All cats had at least one subtherapeutic trough ciclosporin level (Conclusions and relevance The incidence of RPF in this population of cats was relatively low (7%), but still represents a significant cause of morbidity and mortality. The cause of RPF remains unknown, although investigation into suboptimal immunosuppression as a potential cause for local rejection reaction is warranted.

This collection of cases describes some unusual urological tumors and complications related to urological tumors and their treatment. Case 1: A case of uretero-arterial fistula in a patient with long-term ureteral stenting for ureteral oncological stricture and a second case associated to retroperitoneal fibrosis were described. Abdominal CT, pyelography, cystoscopy were useful to show the origin of the bleeding. Angiography is useful for confirming the diagnosis and for subsequent positioning of an endovascular prosthesis which represents a safe approach with reduced post-procedural complications. Case 2: A case of patient who suffered from interstitial pneumonitis during a cycle of intravesical BCG instillations for urothelial cancer. The patient was hospitalized for more than two weeks in a COVID ward for a suspected of COVID-19 pneumonia, but he did not show any evidence of SARS-CoV-2 infection during his hospital stay. Case 3: A case of a young man with a functional urinary bladder paraganglioma who was successfully managed with complete removal of the tumor, leaving the urinary bladder intact. Case 4: A case of a 61 year old male suffering from muscle invasive bladder cancer who was admitted for a radical cystectomy and on the eighth postoperative day developed microangiopathic hemolytic anemia and thrombocytopenia, which clinically defines thrombotic microangiopathy.

IgG4-related disease (IgG4-RD) is still an underestimated disorder which affects multiple organs, and its recognition as a distinct clinical disease has been only proved in the recent decades. The renal involvement has been documented in approximately 15% of patients with IgG4-RD, and the typical manifestation is a tubulo-interstitial nephritis. The main histological findings in IgG4-RD are typically a dense tissue infiltration of IgG4-positive plasma cells, storiform fibrosis, obliterative phlebitis, and frequently elevated IgG4 serum levels. Herein we report our atypical and peculiar clinical presentation of an IgG4-related nephropathy (IgG4-RN) and the remarkable response to rituximab (RTX) treatment at the renal imaging with computerized tomography assessment. The current nephrological evidences support the renal function recovery after steroids or steroids plus RTX therapy, even if the renal imaging data are not always shown. In a complex and enigmatic clinical scenario such as the IgG4-RN, both the renal biopsy and the renal imaging before and after the immunosuppressive therapy become mandatory tools to thoroughly define the diagnosis, the management and the response to the immunological therapy.

Idiopathic retroperitoneal fibrosis (IRF) is a rare condition characterized by the development of a peri-aortic and peri-iliac tissue showing chronic inflammatory infiltrates and pronounced fibrosis. Ureteral entrapment with consequent obstructive uropathy is one of the most common complications which can lead to acute renal failure and, in the long term, to varying degrees of chronic kidney disease. Common symptoms at onset include lower back, abdominal or flank pain. Pain is frequently referred to the hip, to the groin and to the lateral regions of the leg, often with nocturnal exacerbations and not responding to position changes. The disease is commonly associated with signs of systemic inflammatory response (malaise, fever, and anorexia and weight loss). Glucocorticoids are considered the cornerstone of the therapy. The use of other immunosuppressive agents, including cyclophosphamide, azathioprine, methotrexate, mycophenolate mofetil and biological agents such as rituximab, tocilizumab and infliximab have been reported as a valuable option mostly in case reports, cases series and small studies. These agents allowed to reduce cumulative dose of glucocorticoids and their adverse effects. Combined therapy is preferable for all patients who suffer from significant glucocorticoid- related toxicity or in cases where glucocorticoids alone are insufficient to treat the condition.

IgG4-related disease (IgG4-RD) is a fascinating clinical entity first reported in this century in Japan, and includes a wide variety of diseases, such as formerly named Mikulicz's disease (MD), autoimmune pancreatitis (AIP), interstitial nephritis, prostatitis and retroperitoneal fibrosis. The Japanese IgG4 team organized by the Ministry of Health, Labor and Welfare (MHLW) of Japan has published the first criteria, comprehensive diagnostic (CD) criteria for IgG-RD 2011. Thereafter, IgG4-RD has been accepted widely and many cases have been reported from all over the world. Several problems have arisen in clinical practice, however, including the difficulty obtaining biopsy samples, and the sensitivity and specificity in cut off level of serum IgG4 and impaired immunostaining of IgG4. Given these situations, the Japanese IgG4 team has updated the 2011 comprehensive diagnostic criteria for IgG4-RD and propose the 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-RD, which consists of 3 domains; 1) Clinical and radiological features, 2) Serological diagnosis and 3) Pathological diagnosis. In addition, the new pathological diagnosis is composed by three sub-items including storiform fibrosis and obliterative phlebitis.

Objective To report our experience with ureterolysis for the management of retroperitoneal fibrosis. Methods The data of 25 patients who underwent ureterolysis due to primary retroperitoneal fibrosis between 2002 and 2017 were reviewed retrospectively. Initial symptoms, laterality, renal function status (initial/final), operation complications and serum creatinine levels (diagnosis/preoperative/6 months, 12 months postoperatively) were recorded. After surgery, patients were followed up by ultrasonography and serum creatinine levels. Patients with impaired results underwent furosemide renogram and/or late phase of computed tomography. Factors affecting final serum creatinine levels were evaluated. The χ2 -test was used for nominal data among groups. The level of statistical significance was set as P Results A total of 19 patients (76%) were operated bilaterally. The mean follow-up period was 46.2 ± 9.2 months. Among 44 operated renal units, non-functioning kidney developed in seven (15.9%). A total of 34 renal units (77.3%) did not require any additional surgical intervention, and two underwent balloon dilatation (4.5%), one (2.25%) followed with double J stent changes. Two patients developed end-stage renal disease secondary to bilateral unresolved obstruction. High final serum creatinine levels developed in eight (32%) patients without dialysis. Eight patients (32%) were treated with immunosuppressive therapy for systemic recurrence. There was a significant relationship between preoperative serum creatinine levels with final serum creatinine levels (P = 0.005). There was no statistically significant relationship between diagnosis serum creatinine levels with final serum creatinine levels and postoperative dialysis requirement (P = 0.79 and P = 0.817, respectively). Conclusions Ureterolysis provides acceptable success with low complication rates in patients with retroperitoneal fibrosis. Preoperative high-serum creatinine levels can be considered as a risk factor for long-term renal impairment and these patients should be followed closely.

Aims: Retroperitoneal fibrosis (RPF) is a rare chronic fibro-inflammatory disorder that may be secondary to certain drugs, including β-blocking agents (BBAs). However, their causative role is unclear. We aimed to investigate this association. Methods: Disproportionality analysis was carried out on cases from 1985 to 4 October 2020 in VigiBase, the World Health Organization pharmacovigilance database. The Bayesian-based IC 025 metric and reporting odds ratio were used in order to assess the adverse event signal. We also analysed all published case reports from the literature regarding BBA-associated RPF to assess the value of suggested supportive clinical evidence. Results: In total, 1599 individual case safety reports of RPF were reported to VigiBase, of which 132 (32%) concerned 16 different single BBA. For 12 of these agents (75%), reporting of RPF was disproportionate, indicating a potential safety signal. Line listing analysis of individual case safety reports showed no consistent time interval from start of BBA to RPF diagnosis (range 0.7–264 mo). Dechallenge was negative or unknown in the majority of cases (74%). In 18 published cases from the literature, time from start of BBA to RPF diagnosis varied widely (range 3–156 mo). BBA were discontinued 6 months before (n = 1) or at the time of RPF diagnosis (n = 17). Most patients (84%) also received RPF specific treatment. Follow-up duration was short (median 5 mo [range 1–24 mo]) and in most cases (83%) relevant follow-up data were lacking. Conclusion: Although disproportionality analysis indicated a potential safety signal for RPF associated with BBAs, clinical evidence did not support a cause-and-effect relationship.

Objective. Hydronephrosis, a common complication of idiopathic retroperitoneal fibrosis (iRPF), may lead to poor renal outcomes unless resolved in a timely manner. IgG4-related diseases characterized by elevated serum IgG4 levels are responsible for a few iRPF cases. However, the underlying immunologic features of most iRPF cases have not been clearly defined, and these cases exhibit varied responses to medical treatment. Thus, we investigated the predictive factors for hydronephrosis-associated outcomes among iRPF patients. Methods. We retrospectively included 18 iRPF patients with hydronephrosis in a tertiary referral hospital from 2012 to 2019. Hydronephrosis improvement was assessed on images taken 6 months after diagnosis. Categorical variables were compared using chi-square or Fisher’s exact test. Continuous variables were compared using Mann-Whitney U-test. Results. On follow-up images, 8 patients (44.4%) showed an improvement in hydronephrosis. Patients with improvement more frequently had reverse serum IgG4/IgG3 ratio (87.5% vs. 30%, p=0.025), abdominal aorta involvement (87.5% vs. 30%, p=0.025) and glucocorticoid treatment administration (87.5% vs. 30%, p=0.025) than those without improvement. The proportion of elevated serum IgG4 level did not differ between the two groups. Even in the 14 cases with normal serum IgG4 levels, reverse serum IgG4/IgG3 ratio was more frequently observed in patients with improvement than in those without improvement (83.3% vs. 12.5%, p=0.026). Conclusion. The reverse serum IgG4/IgG3 ratio was associated with hydronephrosis improvement in iRPF patients, suggesting it to be a suitable serologic marker for predicting favourable responses to glucocorticoid treatment. (J Rheum Dis 2021;28:38-44)

Introduction and importance Retroperitoneal fibrosis (RPF) is a rare disease characterized by the replacement of normal tissue with fibrosis and inflammation. We present the case of a 40-years-old gentleman with RPF whose presenting complaints were bilateral flank pain and weight loss and was found to have IgG4 related disease. To the best of our knowledge, IgG4-related disease with isolated retroperitoneal involvement is a very rare occurrence. Case presentation The diagnosis of IgG4-Related retroperitoneal fibrosis was made based on clinical, radiological and histopathological criteria. Imaging revealed isolated retroperitoneal involvement and the patient was started on oral steroids with a good clinical response after ten days. Repeated imaging months later showed significant regression in the fibrosis. Clinical discussion RPF can occur due to many etiologies and is categorized to idiopathic and secondary. Factors associated with secondary RPF include medications, autoimmune disease, malignancy and IgG4 related disease. Almost all the reported cases of IgG4 related disease had evidence of multifocal involvement such as the pancreas, aorta and kidneys. Our patient was diagnosed with isolated RPF due to IgG4 disease. He met all the suggested diagnostic criteria, was started on oral steroids and had an excellent clinical outcome. Conclusion IgG4 related disease can present with isolated retroperitoneal fibrosis without involvement of other organ systems. The diagnosis should be based on specific criteria. Treatment with corticosteroids can lead to remission both clinically and radiographically., Highlights • Retroperitoneal fibrosis can present in a variety of clinical pictures. • IgG4 related disease can present with isolated retroperitoneal fibrosis. • It is diagnosed based on clinical, radiological and pathological criteria. • Treatment with corticosteroids can lead to clinical and radiological remission.

Objective To determine the potential predictors of antegrade ureteral stenting (AUS) failure in patients with malignant and benign ureteral obstruction. Method We retrospectively evaluated 116 AUS procedures performed in 80 patients for ureteral obstruction due to malignant and benign causes. Variables such as etiology for obstruction, ureter shape, previous treatment regimen, history of ileal loop diversion, and presence of percutaneous nephrostomy were recorded. Univariate and multivariate logistic regression methods were used between these variables and stent failure. Results Antegrade ureteral stenting was performed as single stage in 24 procedures (n: 24/116, 21%) and performed as a two-step approach after percutaneous nephrostomy in 92 procedures (n: 92/116, 79%). Ureteral stent was successfully deployed in 112 AUS procedures (n: 112/116, 96.5%). In 35 of these successful procedures, the patients were referred to our department due to prior failed retrograde ureteral stenting (RUS). Subsequent stent failure occurred in 40 procedures after a median interval of 39 days. Pre-stenting percutaneous nephrostomy (PN) was a statistically significant risk factor for stent failure (p: 0.041), and age showed an inverse relationship with stent failure (p: 0.008). Complications in early (within the first 30 days after procedure) and late stage occurred in a total of 17 procedures. Early complications included urinary tract infection (n: 11), stent migration (n: 3), and malposition (n: 1). Late complications (after 30 days) were urinary tract infection (n: 1) and stent migration (n: 1). Conclusion This study suggests that AUS can be performed effectively in both benign and malignant ureteral obstructions including cases with prior failed RUS. Two-step AUS after percutaneous nephrostomy was found to be a significant risk factor for subsequent stent failure in our study cohort.

This paper considers the new 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease, discusses essential differences with IgG4-RD comprehensive diagnostic criteria (Umehara H., 2011) and comments their potential use in clinical practice.

Idiopathic retroperitoneal fibrosis, or Ormond’s disease, is a rare syndrome which is in essence the deposition of pathological fibrous tissue in the retroperitoneal space. In most cases, it is initially asymptomatic, but as the disease progresses, clinical symptoms develop. The main complaint are abdominal pain and symptoms resulting from the involvement of ureters, arterial and venous vessels. Diagnosis is based on computed tomography or magnetic resonance imaging, demonstrating a fibrotic tissue mass accumulated in the retroperitoneal space. Due to the typically late presentation of clinical symptoms, a diagnosis is difficult and is often made after complications occur. In our article, we present the case of a 71-year-old patient in whom coexisting ankylosing spondylitis and retroperitoneal fibrosis were diagnosed 15 years after non-specific abdominal pain occurred.

Presentamos el caso de una mujer de 55 años sin antecedentes, que acude por dolor abdominal, se realiza unatomografía abdominopélvica evidenciando un tumor retroperitoneal de aspecto quístico en la periferia de laaorta abdominal, que afecta el riñón izquierdo.Se le realizó una laparotomía exploratoria, nefrectomía izquierda, tumorectomía retroperitoneal y unaresección de un fragmento de la aorta abdominal con reporte histopatológico, corroborado por una pruebainmunohistoquímica de fibrosis retroperitoneal y aortitis por IgG4.El espectro de enfermedades relacionadas con IgG4 es de origen inmunológico con la capacidad de involucrarcasi cualquier órgano. La epidemiología no es precisa, aunque se observa mayor predilección por la raza asiática,siendo más común durante la séptima década de vida. El diagnóstico radica en la coexistencia de varios parámetrosclínicos, laboratoriales e histopatológicos, sin ser ninguno de estos patognomónicos.El objetivo del presente artículo es exponer el reporte de un caso con manifestación inusual de fibrosisretroperitoneal y dar a conocer que la importancia del diagnóstico radica en la decisión terapéutica, ya que cursacon buena respuesta al tratamiento inmunosupresor

Retroperitoneal fibrosis is a rare disease with an incidence of 0-1/100 000 inhabitants per year and is associated with chronic inflammatory fibrosis of the retroperitoneum and the abdominal aorta. This article sheds light on the role of radiological imaging in retroperitoneal fibrosis, names various differential diagnoses and provides an overview of drug and surgical treatment options. A literature search for the keywords "retroperitoneal fibrosis" and "Ormond's disease" was carried out in the PubMed database between January 1, 1995 and December 31, 2019 (n = 1806). Mainly original papers were selected, but also reviews, in English and German language, with a focus on publications in the last 10 years, without excluding older publications that the authors believe are relevant to the topic discussed in the review (n = 40). Ormond's disease is a rare but important differential diagnosis for nonspecific back and flank pain. Imaging diagnostics using CT or MRI show a retroperitoneal mass, which must be differentiated from lymphoma, sarcoma, multiple myeloma and Erdheim-Chester disease. Patients have an excellent prognosis under adequate therapy. FDG-PET/CT or FDG-PET/MRT should be considered as potential modalities, as hybrid imaging can evaluate both the morphological changes and the inflammation. · Ormond's disease is a differential diagnosis for nonspecific back and flank pain.. · Radiological imaging is essential and the gold standard in the diagnosis and follow-up of RPF.. · Patients have an excellent prognosis under adequate therapy..· Peisen F, Thaiss WM, Ekert K et al. Retroperitoneal Fibrosis and its Differential Diagnoses: The Role of Radiological Imaging. Fortschr Röntgenstr 2020; 192: 929 - 936. Die retroperitoneale Fibrose ist mit einer Inzidenz von 0–1/100 000 Einwohner/Jahr eine seltene Erkrankung, welche mit einer chronisch-entzündlichen Fibrosierung des Retroperitoneums und der abdominalen Aorta einhergeht. Dieser Artikel beleuchtet die Rolle der radiologischen Bildgebung bei retroperitonealer Fibrose, benennt verschiedene Differenzialdiagnosen und gibt einen Überblick der medikamentösen und operativen Therapieoptionen. Es wurde eine Literaturrecherche zu den Stichwörtern „retroperitoneal fibrosis“ und „Ormond’s disease“ zwischen dem 01.01.1995 und dem 31.12.2019 in der Datenbank PubMed durchgeführt (n = 1806). Selektiert wurden hauptsächlich Originalarbeiten, aber auch Übersichtsarbeiten, in englischer und deutscher Sprache mit Fokus auf Publikationen in den letzten 10 Jahren, ohne jedoch ältere Publikation auszuschließen, die nach Ansicht der Autoren relevant für das im Review diskutierte Thema sind (n = 40). Der Morbus Ormond ist eine zwar seltene, aber wichtige Differenzialdiagnose bei unspezifischen Rücken- und Flankenschmerzen. Die bildgebende Diagnostik mittels CT oder MRT zeigt eine retroperitoneale Raumforderung, welche differenzialdiagnostisch insbesondere von Lymphomen, Sarkomen, dem multiplen Myelom sowie der Erdheim-Chester-Erkrankung abgegrenzt werden muss. Patienten haben unter adäquater Therapie eine ausgezeichnete Prognose. Ggf. kann eine FDG-PET/CT oder FDG-PET/MRT erwogen werden, da die Hybridbildgebung sowohl die morphologischen Veränderungen als auch die Inflammation durch den FDG-Tracer-Uptake bewerten kann. · Der Morbus Ormond ist eine Differenzialdiagnose bei unspezifischen Rücken- und Flankenschmerzen.. · Die radiologische Bildgebung stellt eine zentrale Rolle und den Goldstandard in der Diagnostik und Verlaufskontrolle der RPF dar.. · Patienten haben unter adäquater Therapie eine ausgezeichnete Prognose..· Peisen F, Thaiss WM, Ekert K et al. Retroperitoneal Fibrosis and its Differential Diagnoses: The Role of Radiological Imaging. Fortschr Röntgenstr 2020; 192: 929 – 936.

BACKGROUND: Retroperitoneal fibrosis is an exceptionally rare disease characterized by proliferation of fibrous tissue and inflammation in the retroperitoneum. It features many symptoms in the kidneys and in other organs and usually leads to ureteral obstruction. CASE SUMMARY: Here we present 9 consecutive cases of idiopathic retroperitoneal fibrosis (IRPF) in patients who presented to the Department of Nephrology or Department of Rheumatology, Xuanwu Hospital, Capital Medical University, Beijing, China, between January 2012 and June 2017 with ureteral obstruction due to external compression of the ureter that led to hydronephrosis and kidney dysfunction. Computed tomography imaging was used to identify hydronephrosis and ureteral obstruction and to evaluate kidney function. Each patient was diagnosed with IRPF based on clinical observation and computed tomography examination results. To restore kidney function, a retrograde metallic stent was placed in the ureter under X-ray guidance 2 d after each patient’s admission. No perioperative complications occurred in any patient, but postoperative complications occurred in two patients as follows: Patient 2 had stent migration and repeated metallic stent infections that resolved with treatment; and patient 4 had postoperative hematuria because he resumed normal activities too soon after stent placement (contrary to instruction). Placement of the metallic ureteral stents provided relief from ureteral obstruction and restored kidney function in all patients. CONCLUSION: Our 9-case series underscores the utility and efficacy of applying the Resonance(®) metallic ureteral stent to treat ureteral obstruction in patients with IRPF. For all retroperitoneal fibrosis cases in our series, ureteral stents provided effective relief and were shown to reduce the incidence rate of perioperative and postoperative complications.

Background. IgG4-related disease is a new disease entity, but little attention was drawn to urinary system involvement besides nephritis or nephropathy. Here, we described clinical, radiological, and pathological manifestations of IgG4-related urinary disease (IgG4-RUD) and assess its treatment responses. Methods. We conducted a retrospective study enrolling 65 IgG4-RUD patients from an IgG4-related disease (IgG4-RD) cohort of the Peking Union Medical College Hospital. Clinical, laboratory, radiological, pathological data were collected, and treatment response to immunosuppressants were analysed. Results. IgG4-related interstitial nephritis (TIN, 32.3%), glomerular nephritis (GN, 7.7%), renal pelvis and ureter involvement (21.5%), abnormal radiology with quiescent clinical presentation (13.8%), and renal parenchymal lesion plus retroperitoneal fibrosis (RPF, 18.5%) were major lesion types of IgG4-RUD. All patients had elevated serum IgG4, 76.9% had hyperglobulinemia, and 92.3% had elevated serum IgE at diagnosis. IgG4-TIN patients presented with renal dysfunction, and 94.3% had low serum complement C3 and IgG4-GN presented with nephrotic syndrome, while renal pelvis and ureter involvement had normal renal function and urinalysis. IgG4-RPF with renal parenchymal involvement presented with acute renal dysfunction and required emergency medical intervention. Renal cortex low-density areas, parenchyma or pelvis nodular mass, bilateral enlargement of the kidney, and renal pelvis and ureter mass/wall thickening were specific image patterns of IgG4-RUD. Infiltration of plasma lymphocytes and storiform fibrosis were histopathological features of IgG4-RUD. Patients showed satisfactory responses to immunosuppressive treatment, but complete recovery of renal function was difficult to achieve in IgG4-TIN. Four patients (6.2%) experienced clinical relapses during the maintenance period. Conclusion. IgG4-RUD had diverse lesion types and distinctive manifestations. Radiological examinations were helpful for diagnosis and treatment evaluation. Patients showed good initial response to immunosuppressive treatment but relapses could occur at the maintenance period.

Introduction Four clinical phenotypes of IgG4-related disease (IgG4-RD) have been recently identified by latent class analysis (LCA): pancreato-biliary (group 1); retroperitoneum/aortitis (group 2); head and neck limited (group 3); and Mikulicz/systemic (group 4). The reproducibility of this classification in clinical practice and its relevance for patient management, however, remain unknown. Methods The study included 179 patients. Four IgG4-RD experts were asked to classify a validation cohort of 40 patients according to published LCA-derived phenotypes based on clinical judgement. Agreement between LCA and clinical clustering was calculated. To assess differences among disease phenotypes, the following variables were recorded on an additional 139 patients: serum IgG4 and IgE; inflammatory markers; eosinophils; plasmablasts; IgG4-RD responder index (RI); history of atopy, diabetes, osteoporosis, relapses and malignancy; cumulative dose of glucocorticoids; and use of rituximab. Results Clinical judgement replicated LCA classification with strong agreement among IgG4-RD experts (κ = 0.841, P Conclusion Clinical phenotypes of IgG4-RD reflect differences in epidemiological features and prognostic outcomes.

Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis with a putative neoplastic and inflammatory nature. The disease is driven by mutations in proto-oncogenes such as BRAF and MEK, while immune-mediated mechanisms contribute to disease development and progression. The clinical presentation of ECD is highly heterogeneous, ranging from smouldering unifocal forms to multiorgan life-threatening disease. Almost any organ can be involved, but the most common lesions include long-bone involvement, retroperitoneal fibrosis, interstitial lung disease, pericardial and myocardial infiltration, CNS, retro-orbital, and large-vessel involvement. These manifestations may mimic those of neoplastic and systemic immune-mediated diseases. Overlap with these conditions represents an emerging challenge for the clinician. A variety of treatments are efficacious for ECD, targeting both the MAPK-pathway and the immune-mediated pathomechanisms. The traditional approach is based on immunomodulatory agents (interferon-α), but recent alternatives-including anti-cytokine therapies (IL1- and TNFα-blockers) and immunosuppressants (mTOR-inhibitors)-showed promising results. However, since the detection of MAPK pathway activation in most patients and the dramatic efficacy of BRAF and MEK inhibitors, these targeted treatments represent the first-line approach in patients with severe disease forms. High rates of radiologic responses do not often mean clinical remission, especially for CNS involvement, which often results in chronic disability. This review will outline the main clinical features of ECD, with emphasis on the emerging challenges in pathogenesis and management, and on the role of recent targeted approaches.

Sclerosing lipogranuloma (SLG) in children is a rare, benign disease of unknown etiology suspected to be due to abnormal fatty tissue reaction. A 13-year-old girl presented with progressively worsening back pain. Cross-sectional imaging identified a retroperitoneal mass compressing the left ureter as well as infrarenal inferior vena cava atresia with extensive venous collaterals and chronic partially occlusive thromboses of the iliac veins. Surgical biopsy was consistent with SLG and it resolved spontaneously. SLG is typically a disease of adulthood but may be seen in children. The association between inferior vena cava atresia with venous thrombosis and development of SLG has not been reported previously.

Mikulicz’s disease, an immunoglobulin G4-related disease (IgG4-RD) occurs frequently in the head and neck region but rarely in the larynx. We report a case of IgG4-RD with pseudotumor formation in the larynx. A 50-year-old man presented at our facility for a complete physical examination and diagnostic testing of a left arytenoid tumor. On examination, a large supraglottic mass was noted with signs of dyspnea. Movement disorder of the vocal fold was absent. The patient underwent surgery with general anesthesia to improve respiratory symptoms and a resected specimen was submitted for diagnosis. The pathology findings revealed lymph follicle formation, interstitial follicular fibrosis, angiogenesis, and inflammatory cell infiltration with plasmacytosis. Immunohistologic staining at high magnification revealed one hundred or more IgG4-positive plasma cells and fifty percent IgG4/IgG. In addition, obliterative phlebitis was observed. Medical history was positive for retroperitoneal fibrosis with serum IgG4 levels below the diagnostic criteria of IgG4-RD, but histological characteristics of IgG4-RD were met. The diagnosis of IgG4-RD was established, oral treatment with prednisolone (0.6 mg/kg/day) was initiated, and the tumor reduced in size. IgG4-RD may cause irreversible functional impairment. Early diagnosis and treatment are important in reducing the risk of permanent impairment of vocal fold mobility.

Retroperitoneal fibrosis is characterized by fibrotic lesions around the abdominal aorta and common ileac artery causing ureteral obstruction. Secondary retroperitoneal fibrosis is associated with malignant disease, drugs, exposure to radiation and surgery. In contrast, the majority of retroperitoneal fibrosis is classified into idiopathic retroperitoneal fibrosis, for which immunological etiology has been suggested. Recently, idiopathic retroperitoneal fibrosis has been considered to be a spectrum of immunoglobulin G4-related disease, a systemic inflammatory disease, the concept of which has been developed during the past decade. In the management of retroperitoneal fibrosis, assessment of systemic lesions associated with immunoglobulin G4-related disease and the exclusion of secondary retroperitoneal fibrosis is mandatory. Histological examination of retroperitoneal lesions is desired for accurate diagnosis and management. Laparoscopic or open biopsy is often beneficial, although it is more invasive than needle biopsy. Treatment for idiopathic retroperitoneal fibrosis consists of meticulous glucocorticoid therapy based on that for immunoglobulin G4-related disease, which is expected to be highly effective. Ureteral obstruction is usually managed with conservative procedures, such as ureteral stenting or percutaneous nephrostomy. The goal of treatment for retroperitoneal fibrosis should be freedom from the stent/nephrostomy with withdrawal of the glucocorticoid in addition to salvage of renal function; however, conservative management does not always provide favorable outcomes. In contrast, aggressive surgical treatment, such as ureterolysis, can achieve the goal; however, the procedure is associated with high morbidity. Establishment of a consensus about treatment for idiopathic retroperitoneal fibrosis, including the optimal indications for the invasive surgical procedure and conservative management, is desired.

Retroperitoneal fibrosis (RPF) causing large vessel stenosis and thrombosis is rare but well-described. We describe a 50-year-old man with rapid progression of central venous thrombosis in the presence of RPF and exogenous testosterone use. Therapeutic anticoagulation was initiated and catheter directed thrombolysis was performed after placement of an inferior vena cava (IVC) filter. Repeat venogram revealed severe focal retrohepatic IVC stenosis, which was treated with serial venoplasty and stenting. Clinical improvement was significant 48 hours after intervention. This case represents a rare presentation of IVC occlusion in the setting of RPF and exogenous testosterone administration successfully treated with endovascular interventions. Keywords: Renal vein thrombosis, Inferior vena cava occlusion, Retroperitoneal fibrosis, Testosterone

Background Retroperitoneal fibrosis is a rare disease characterized by chronic nonspecific inflammation, which leads to clinical compression manifestations of retroperitoneal organs especially ureter. Approximately 70 percent of retroperitoneal fibrosis cases are idiopathic which has no clear etiology. This study reported a rare case of a 48-year-old woman presented with idiopathic retroperitoneal fibrosis and endometrial cancer. Case presentation A 48-year-old woman presented with irregular vaginal bleeding without abdominal pain, bloating or discomfort. The patient was diagnosed iRPF after splenectomy 13 years ago. Then she took prednisone for 2 years and took tamoxifen for about 11 years. She stopped taking the medication from October 2019 to May 2020 and then started taking tamoxifen again until November 2020. Two weeks after she stopped taking tamoxifen, she presented with irregular vaginal bleeding. Gynecological ultrasound revealed a thick endometrium with uneven echo enhancement and blood flow signals. Then diagnostic curettage was performed with pathological examination showed endometroid carcinoma. Later, the patient was admitted to Peking University Third Hospital for surgery. Preoperative imaging examinations, including CT, MRI, and PET/CT, all showed pelvic enlarged lymph nodes and they were highly suspected to have lymph node metastasis. The patient underwent laparoscopic surgical staging and enlarged lymph nodes in the pelvic and aortic regions were removed. Finally, the pathology confirmed that endometrioid adenocarcinoma and fibrosis, but there was no tumor infiltration in these enlarged lymph nodes. The patient is now in good condition. Conclusion This case report stressed the difficulty to distinguish between lymph node metastasis and inflammatory hyperplasia by common imaging methods. Due to increased surgical difficulty among retroperitoneal patients, lymphadenectomy should be carefully evaluated to avoid additional surgical complications and over-treatment.

IgG4-related retroperitoneal fibrosis is a type of IgG4-related diseases. The etiology is still unclear. It is often classified as idiopathic retroperitoneal fibrosis, but the pathological process and clinical results between them are different. Therefore, the differential diagnosis of them is very important. An 18-year-old patient was admitted to the Second Xiangya Hospital of Central South University due to "left lower extremity edema for 1 and a half month" on December 19, 2019. In examination of PET/CT, the starting point of the left aorta-intra-thoracic artery showed a soft tissue density lump with increased sugar metabolism, which indicated a suspected malignant tumor, and dilatation of the left renal pelvis and ureter. On January 8, 2020, the patient was performed pelvic edema excision, ureterectomy and anastomosis. The postoperative examination results were considered as IgG4-related disease. As a rare systemic disease, early correct diagnosis and precise and individualized treatment can alleviate the suffering of patients and ensure that patients are minimally traumatized.IgG4相关性腹膜后纤维化作为IgG4相关性疾病中的一类疾病，病因尚不明确，既往常将其归为原发性腹膜后纤维化疾病，但二者的病理过程和临床结局都不相同，因此，对二者的鉴别诊断非常重要。2019年12月19日中南大学湘雅二医院收治1例因“左下肢水肿1个半月”的18岁患者，患者的全身PET/CT提示：左侧髂总动脉-髂内外动脉起始处糖代谢增高的软组织密度肿块，疑为恶性肿瘤；左侧输尿管及左侧肾盂扩张并积水。患者于2020年1月8日行盆腔肿物切除+输尿管切除吻合术，术后病理检查结果考虑为IgG4相关性疾病。作为一种罕见的系统性疾病，早期正确诊断与精准化和个体化治疗能减轻患者的痛苦，并使患者创伤降至最小。.

Background Granulomatosis with polyangiitis is a systemic inflammatory disease characterized by necrotizing vasculitis that affects small- and medium-sized blood vessels. Granulomatous inflammation affects the lungs, ears, nose, and throat, and commonly affects the kidneys, although the retroperitoneal tissue is rarely affected. Several studies have reported an increased risk of venous thromboembolism. Early diagnosis and treatment are of vital importance due to the rapid progression of the disease. Case presentation We present the case of a 66-year-old Moroccan man followed for bilateral jugular thrombosis. Cavitary pulmonary nodules and retroperitoneal fibrosis with thrombosis involving several vascular territories were detected on thoracoabdominopelvic computerized tomography scan. Laboratory analyses revealed that the patient was positive for cytoplasmic antineutrophilic antibodies. The diagnosis of granulomatosis with polyangiitis was retained. Treatment with glucocorticoids and immunosuppressive agents resulted in significant clinical and radiological improvement over the following months. Conclusions We describe the diagnostic steps and the difficulty of managing this patient. Rare manifestations, such as retroperitoneal fibrosis, have been reported in the literature in association with granulomatosis with polyangiitis, and should not delay the diagnosis and treatment of granulomatosis with polyangiitis owing to its severity.

Some patients diagnosed with idiopathic retroperitoneal fibrosis could be reclassified as IgG4-related disease (IgG4-RD). Classification criteria have not been uniform and prevalence of IgG4-related retroperitoneal fibrosis (IgG4-RPF) is unknown in our region. We aimed to describe IgG4-RPF frequency relying on criteria published recently and comparing clinical, histopathologic and radiologic features with non-IgG4-RPF. From January, 2005 to December, 2020, nineteen adults with histopathologic diagnosis of idiopathic retroperitoneal fibrosis were included in a dynamic retrospective cohort at Hospital Italiano de Buenos Aires. Pathology slides were reviewed and immunohistochemistry was performed and assessed for each case. We used classification criteria described in 2019 American College of Rheumatology/European League Against Rheumatism to identify IgG4-RD cases. Ten of 19 patients met criteria for IgG4-RPF. Median age was similar in two subsets (61 versus 55, p = 0.2) and both had male predominance. Three out of 10 patients (p = 0.2) featured other manifestations of IgG4-RD in the IgG4-RPF group, and periaortic fibrosis was the most significant finding in images (p = 0.01). Corticosteroids were mostly used as therapy, followed by azathioprine and rituximab. Most patients did not receive specific treatment. IgG4-RPF patients had dense lymphocytic infiltrate and 8 out of 10 showed storiform fibrosis (p = 0.02). IgG4+ cells/hpf and IgG4/IgG ratio were significantly higher (p = 0.01). Over half of the patients in our cohort met the criteria of IgG4-RPF. New criteria may harmonize the identification of IgG4-RD. As IgG4-RD may be reversible at initial stages, these findings may lead to early recognition, treatment and integral follow-up.Muchos pacientes con diagnóstico de fibrosis retroperitoneal idiopática (FRI) pueden ser reclasificados como enfermedad relacionada con IgG4 (ER- IgG4). Los criterios diagnósticos no han sido uniformes y la frecuencia de fibrosis retroperitoneal relacionada con IgG4 en nuestra región es desconocida. El objetivo fue describir la frecuencia de ER-IgG4 en pacientes clasificados como FRI y comparar características clínicas, histopatológicas y de laboratorio con aquellos que no reunían criterios de la enfermedad. Se incluyeron 19 adultos en un estudio de cohorte retrospectiva dinámica con diagnóstico anatomopatológico de FRI, en el Hospital Italiano de Buenos Aires, desde enero de 2005 hasta diciembre de 2020. Se revisaron las biopsias y se realizó inmun ohistoquímica en cada una. Se consideró caso al paciente que reunía los criterios de la American College of Rheumatology/European League Against Rheumatism 2019. Diez pacientes reunieron criterios de ER-IgG4. La mediana de edad fue similar en ambos grupos (61 vs. 55, p = 0.2) y en ambos hubo predominio masculino. Tres de 10 pacientes (p = 0.2) tuvieron otras manifestaciones de ER-IgG4 y la fibrosis periaórtica fue el hallazgo más significativo en los estudios por imágenes (p = 0.01). Los corticoides fueron las drogas más utilizadas seguidos por azatioprina y rituximab, pero la mayoría no recibió tratamiento específico. Todos los pacientes con fibrosis retroperitoneal relacionada con IgG4 presentaron infiltrado linfocitario denso y 8/10 fibrosis estoriforme (p = 0.01), así como las células IgG4+/hpf y ratio IgG4/IgG fueron significativamente mayores (p = 0.01). Más de la mitad de los pacientes con FRI cumplieron criterios de ER-IgG4. Los nuevos criterios diagnósticos podrían contribuir a homogeneizar la identificación de ER-IgG4. Dado que esta enfermedad puede ser reversible en estadios tempranos, estos resultados promueven aumentar el conocimiento de la entidad para tratamiento precoz y seguimiento integral.

Retroperitoneal fibrosis (RPF) is a disease that develops following inflammation and fibrosis of the retroperitoneum and other abdominal organs, which manifests with various local and systemic symptoms. It has several entities, namely the idiopathic RPF (IRPF), secondary RPF and Immunoglobulin G4-related disease (IgG4-RD). The role of a gynaecologist in the management of retroperitoneal fibrosis is highlighted in this case of a parametrial tumour causing retroperitoneal fibrosis and hence leading to hydronephrosis and hydroureter as a consequence. A 49-year-old woman presenting with a symptom cluster suggestive of acute pyelonephritis was found to have reduced eGFR. Elevated levels of inflammatory markers indicated an inflammatory nature of the disease, in addition to a normochromic anaemia that could be due to chronic inflammation itself. CECT Neck, Chest, Abdomen and Pelvis revealed mild hydronephrosis with a distal narrowing and a retroperitoneal confluent mass. Investigations led to suspect an invasive left ovarian malignancy or an endometriotic nodule with ureteric compression, which was later thought to be unlikely with a normal CA-125 level. To obtain a better understanding, a cysto-ureteroscopy was performed where she was found to have an obstruction. Although stent placement is a reasonable temporary mode of symptom relief, this was proven unsuccessful. Surgical management was opted for considering the severity of her symptoms and the course of time it would take for medical management to take effect. In addition, laparoscopy would aid in the exclusion of other pelvic pathologies under direct vision and treat any if found concurrently. Consequently, a laparoscopic hysterectomy, bilateral salpingo-oophorectomy, left sided ureterolysis with careful resection of the densely adhered tumour surrounding the left cysto-ureteric junction was performed, followed by retrograde ureteric stenting. Histology revealed retro-peritoneal fibrosis, and further immunohistochemistry pointed towards a diagnosis of IgG4 related sclerosing disease. This was also supported by elevated levels of IgG, especially the IgG4 component. Disseminated RPF was subsequently excluded by CECT. Following surgery, she was started on high dose corticosteroids and the stent was left in place for six weeks. She is being kept on a maintenance dose of low dose corticosteroids without disease relapse. Regular follow-up with inflammatory markers, serum IgG4 levels and imaging demonstrate adequate response to corticosteroid therapy.

Idiopathic retroperitoneal fibrosis (iRPF) is a rare chronic fibro-inflammatory disorder of unknown etiology. Activated T-helper cells, which shed soluble interleukin-2 receptor (sIL-2R) into the circulation, may play a pathogenetic role. Hence, measuring sIL-2R may be of value in monitoring disease activity and treatment response in iRPF patients.We performed a prospective inception cohort study of 82 patients with untreated (re)active iRPF stratified by elevated ( 623 U/mL) or normal sIL-2R level at baseline and compared disease characteristics among these groups. Baseline and changes in sIL-2R levels following treatment with tamoxifen (TMX) or prednisone (PDN) were analyzed and related to treatment response.Median sIL-2R level was 668 U/mL (IQR 502.8-827.5); 48 patients (59%) had elevated baseline sIL-2R levels. Patients with elevated sIL-2R presented with higher CRP (P = 0.049) and serum creatinine (sCr) levels (P 0.001) and more often had hydroureteronephrosis (P = 0.01). There was an age and sex adjusted linear association between baseline sIL-2R and both CRP (P = 0.02) and sCr (P 0.001). Baseline and serial levels of sIL-2R were predictive and concordant, respectively, with clinical response in patients treated with PDN. ROC curve analyses of sIL-2R on a continuous scale and PDN treatment success showed an AUC of 0.84. A serum sIL-2R cut-off value for PDN treatment success of ≤ 703 U/mL was found with a sensitivity of 100% and specificity of 72%.Serial measurement of sIL-2R may be of value in monitoring disease activity and PDN treatment response in iRPF patients.

A 52-year-old man was admitted to our hospital with lower abdominal and low back pain, and abdomen CT at an outside hospital presented a retroperitoneal mass with left urinary tract obstruction and hydronephrosis. In 68 Ga-pentixafor PET/CT, the retroperitoneal mass showed intense radioactivity. Subsequently, the patient underwent biopsy of the retroperitoneal mass. The pathological examination showed only fibrous tissue without tumor cells. The patient was finally diagnosed with idiopathic retroperitoneal fibrosis. Our case demonstrated that idiopathic retroperitoneal fibrosis had intense uptake of 68 Ga-pentixafor.