UNASSIGNED: Retroperitoneal fibrosis (RPF) is a fibroinflammatory disease in which patients may suffer obstructive uropathy (OU). The optimum treatment strategy for RPF with secondary OU is currently unclear, and the aim of this literature review is to assess the methods used to treat this patient cohort.
UNASSIGNED: Medline, Embase, Cinahl, the Cochrane Library and PubMed were systematically searched to find studies assessing treatment outcomes in this patient cohort. After reviewing the studies\' titles, abstracts and full texts, 12 were found that matched our search aims. Data from these publications were analysed and reported.
UNASSIGNED: The demographic and symptomatic features of patients across the 12 studies were representative of the general RPF population. No randomised control trials (RCTs) were found, and just one study formally compared outcomes between patients who underwent different treatment strategies. Many of the studies concluded that using medical and surgical methods in combination led to positive outcomes; whereas, others found positive outcomes following a variety of regimens. Many studies also highlighted, however, that significant minorities required further treatment after initial therapy. Conclusions regarding optimum treatment methods were limited as most publications did not formally compare outcomes following different strategies and had an observational study design.
UNASSIGNED: Although positive outcomes were commonly seen following medical, surgical and a combination of treatments, the literature currently lacks research formally comparing outcomes after assigning specific treatment protocols to groups of RPF patients. More research is therefore required to determine how to best manage RPF leading to secondary OU.

IgG4-related retroperitoneal fibrosis is a rare cause of renal dysfunction that usually manifests as obstructive nephropathy (sometimes with extrarenal manifestations). Due to the non-specific symptoms at the onset of the disease and often latent course, special laboratory and instrumental examination methods are usually needed to establish a diagnosis. The article describes a clinical case of a relapse of IgG4-related retroperitoneal fibrosis in a 53-year-old patient, who developed bilateral ureterohydronephrosis with postrenal acute kidney injury after the withdrawal of glucocorticoid therapy. The patient underwent bilateral percutaneous nephrostomy and resumed glucocorticoids at a dose of 30 mg/day. Postobstructive diuresis was 22 L. Treatment resulted in a complete normalization of the creatinine level and transient hypokalemia, which was eliminated with potassium medications. At the final stage of the treatment, bilateral stenting of both ureters was performed with a tapering of glucocorticoids to 5 mg per day with CT control of the retroperitoneal space after 5 months. A clinical case demonstrates that an interruption of glucocorticoid treatment in patients with IgG4-related retroperitoneal fibrosis can lead to ureterohydronephrosis with the development of acute kidney injury. In such cases, stenting of the ureters could be considered an optimal therapeutic option.

A potential complication of pharmacotherapy for a given patient is the possibility of various side effects of drugs, which are manifested in many ways and constitute iatrogenic causes of diseases. Among the systemic side effects of drugs, there are also those involving the urinary tract, although these are less reported in the literature. The use of numerous drugs-especially of anticholinergics or drugs with anticholinergic potential, opioid analgesics, non-steroidal anti-inflammatory drugs, antidepressants, first-generation antipsychotics (classic neuroleptics) and selected cardiovascular drugs (beta-blockers, thiazides potassium-sparing diuretics, statins), as well as others-may increase the risk of developing urological disorders, such as urinary retention or incontinence, urinary tract infections, urolithiasis, erectile dysfunction in men and retroperitoneal fibrosis. The purpose of this paper is to characterise the abovementioned drug-induced disorders of the lower urinary tract on the basis of a non-systematic literature review.

Retroperitoneal fibrosis (RPF) can occur due to many etiologies and is categorized into idiopathic and secondary. Etiologies of secondary RPF include medications, autoimmune disease, malignancy, and IgG4-related disease (IgG4-RD). Although IgG4-RD usually involves multiple systems synchronically including the pancreas, aorta, and kidneys, it can present with isolated RPF without involvement of other organ systems. Caution must be exercised in these instances as the diagnosis should be confirmed based on specific clinical, radiographic, and histopathologic criteria. Such confirmation can affect the work-up and therapeutic approach as treatment with corticosteroids can lead to remission, both clinically and radiographically.

Immunoglobulin G4-related disease (IgG4-RD) is a systemic, immune-mediated disease that can affect multiple organs, including the orbits, salivary glands, thyroid gland, lungs, aorta, pancreas, bile ducts, lymph nodes, and retroperitoneum. While timely diagnosis is particularly important given the efficacy of glucocorticoid treatment for IgG4-RD, accurate recognition can prove a challenge given the overlap between the imaging features of this disease and other entities.
After a review of the epidemiology, pathophysiology, and clinical considerations (including treatment) associated with IgG4-RD, this pictorial review will showcase the variable imaging manifestations of this disease in the abdomen and pelvis. Post-treatment imaging appearance of these entities will be reviewed and mimickers of this disease in the abdomen and pelvis will be presented.
The presence of mass-like soft tissue with radiographic characteristics of fibrosis affecting multiple organs should raise suspicion for IgG4-RD, although definite diagnosis can only be made with appropriate clinical, serological, and pathologic data.

Immunoglobulin G4-related diseases (IgG4-RDs) are immune-mediated fibroinflammatory multisystemic conditions identified by the presence of tumefactive lesions with a rich infiltrate of IgG4-positive plasma cells, and often by a high IgG4 serum concentration. IgG-RDs have a prevalence of at least 1 case every 100,000 persons, and they are mostly diagnosed after age 50, with a male to female ratio of about 3:1. IgG4-RD pathophysiology is still uncertain: it has been proposed that both genetic predisposition and chronic environmental exposures may play a role by triggering abnormal immune activation that perpetuates the disease. The purpose of this review is to summarize the evidences supporting the hypothesis that certain environmental/occupational exposures can trigger IgG4-RDs, focusing on the possible role of asbestos in an emerging IgG4-RD called idiopathic retroperitoneal fibrosis (IRF).
Although some studies suggested a relationship between tobacco smoking and IgG4-RD risk, occupational exposures seem to have the most interesting effects. Positive history of blue-collar work increases the risk of developing an IgG4-RD, and mineral dusts and asbestos were the most strongly associated industrial compounds. Asbestos has been found to be a risk factor for IRF years before its classification as IgG4-RD, and later in two large case-control studies. In the most recent one, conducted on 90 patients and 270 controls, asbestos exposure conferred an increased IRF risk, quantified by odds ratios from 2.46 to 7.07. Further structured studies including serum IgG4 evaluation should be conducted to clarify the effect of asbestos on patients with confirmed diagnosis of IgG4-related IRF. Environmental exposures, especially of occupational origin, appear to play a role in the development of different types of IgG-RDs. In particular, although first suggested very recently, the relationship between asbestos and IRF deserves to be explored in more structured studies, especially because of the biological plausibility of the role of asbestos in IRF pathogenesis.

UNASSIGNED: Multifocal fibrosclerosis is a rare disorder causing progressive fibrosis of multiple organ systems. Existing data on the disease show that there are multiple manifestations of the disease, with different outcomes. However, quantitative data are scarce, prompting the need for our investigation.
UNASSIGNED: A comprehensive systematic review was performed from inception to November 16, 2022, with the restriction of English language, not including review articles. Article screening and extraction was performed independently, and shortlisted articles were assessed for bias. Analysis was performed using SPSS Version 25 (IBM® SPSS® Statistics; Chicago, IL, USA). Data were presented as frequencies and percentages, with a confidence interval of 95%.
UNASSIGNED: This review included 134 patients, with 78 (58.2%) males. Mean age was 53.6 years and included two pediatric patients. The most common comorbidity was diabetes (9.7%). Prevalent presenting symptoms included pain (47.8%) and swelling (35.1%). A mean of 2.51 organs or anatomical sites was affected, retroperitoneum (64.2%) being most affected. The pancreas (30.0%) and digestive system (47.0%) were the organs/organ systems most affected. Patients had favorable outcomes in 79.1% of cases, 87.3% had no relapse, and 91.8% of patients survived the condition.
UNASSIGNED: The findings in this study provide a quantitative measurement of the demographics, presentations, organ manifestations, and outcomes of multifocal fibrosclerosis. We found the disease to be prevalent in males in Japan or the United States, with the abdomen and its organs being commonly involved.

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.
METHODS: 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.
CONCLUSIONS: 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 disease is an immune-mediated deposition of fibro-inflammatory tissue with IgG4-positive plasma cells and dense fibrosis in a single or multiple organs. It often raises concern for malignancy requiring biopsy for diagnosis. Presentation may vary according to organ involved. IgG4-related retroperitoneal fibrosis accounts for two-thirds of the previously considered idiopathic retroperitoneal fibrosis cases. In IgG4-related retroperitoneal fibrosis, computed tomography scan or magnetic resonance imaging shows periaortic soft tissue that extends from below the kidneys to iliac arteries and entrap ureters causing hydronephrosis and renal failure. We present a rare case of IgG4-related retroperitoneal fibrosis presenting with leg swelling and pain due to inferior vena cava compression and thrombosis, and we review current concepts on disease pathogenesis, diagnosis and treatment.

BACKGROUND: Retroperitoneal fibrosis (RPF) is a rare disease characterized by fibro-inflammatory tissue proliferation in the retroperitoneum. It results in a chronic inflammatory and fibrosis condition, possibly leading to compression of the retroperitoneal structures, especially to encasement of the ureters and the inferior vena cava. It may have an idiopathic or a secondary origin. Spondyloarthritis (SpA) is one of the rare conditions described among the secondary forms.
METHODS: Herein, we report a new case of RPF in a patient with AS. Who presented with acute abdominal pain radiating to the lumbar region and the left testicle. On clinical examination, we found a mild stiffness of the lumbar spine and a decrease in chest expansion. Sacroiliac joint pain was also found. The rest of the physical examination was normal. Laboratory tests showed inflammation with increased C-reactive protein (130 mg/l) and creatinine (112 micromol/l) levels. The computed tomography scan revealed a soft tissue density mass located around the sub-renal aorta. Diagnosis of idiopathic RPF associated with AS was retained. The patient was treated by a daily dosage of 1 mg/kg of oral glucocorticoid with good outcome.
CONCLUSIONS: RPF is a rare condition that can be either idiopathic or secondary. Its association with spondyloarthritis, mainly in its ankylosing spondylitis form, seems to be more than anectodal. Treatment may involve medical therapy and/or surgical management.
CONCLUSIONS: In the presence of back pain, fatigue, weight loss, and low grade fever in spondyloarthritis patients, physicians should screen for retroperitoneal fibrosis as it could be a possible cause.