Retroperitoneal Fibrosis: A Puzzle of Elusive Causal Link.

Retroperitoneal fibrosis (RPF) is a rare condition characterized by the presence of fibro-inflammatory tissue surrounding the abdominal aorta and iliac arteries, often leading to the involvement of adjacent organs such as the ureters and inferior vena cava. We present a case report of a 56-year-old Caucasian woman with a complex medical history, including Hodgkin's lymphoma treated with chemotherapy and radiotherapy (31 years before), a significant smoking history, and a current presentation of acute kidney injury with oliguria, edema, and hypertension. Initial diagnostic considerations included rapidly progressive glomerulonephritis, supported by clinical and imaging findings. However, the absence of specific autoantibodies and the presence of bilateral calyx-pelvic dilation raised questions regarding alternative diagnoses. Imaging studies, including CT, contrast-enhanced CT, and subsequent MRI, revealed periaortic and paracaval adipose tissue thickening suggestive of periaortitis, leading to a diagnosis of retroperitoneal fibrosis. The multifactorial etiology, including previous radiation therapy, lymphoma history, and significant smoking, posed challenges in establishing a definitive causal link. Despite extensive investigations, including laboratory tests and imaging modalities, no single etiological factor could be conclusively identified. This case underscores the diagnostic complexity of RPF, especially in the presence of multiple potential risk factors, and highlights the importance of considering this condition in the differential diagnosis of patients presenting with renal dysfunction and obstructive uropathy.

Bessel's disease-the first report of an IgG4-related disorder.

Many aspects of IgG4-related diseases were initially described during the late 19th and early 20th century. A variety of clinical presentations caused by this common pathology have been named after the researchers who first described the disorders, such as Mikulicz, Küttner, Riedel or Ormond. However, the initial description of retroperitoneal fibrosis dates back to even 50 years earlier, when in 1846, the Prussian private practitioner Raphael Jakob Kosch described a hitherto unknown constellation of symptoms and pathological findings in a famous patient. This celebrity was the mathematician and astronomer Friedrich Wilhelm Bessel, a close friend of Alexander von Humboldt and Carl Friedrich Gauss.

Rotura de aorta abdominal en fibrosis retroperitoneal idiopática / Abdominal aortic rupture in idiopathic retroperitoneal fibrosis

Introducción: la fibrosis retroperitoneal (FRP) es una enfermedad poco frecuente que se caracteriza por la formación de tejido inflamatorio y fibroso en el espacio retroperitoneal, alrededor de la aorta abdominal y de las arterias ilÍacas, incluyendo con frecuencia los uréteres. Caso clínico: describimos el caso de un paciente de 90 años con diagnóstico de FRP tratado previamente con metilprednisona que acudió a urgencias por la rotura de un aneurisma inflamatorio de la aorta abdominal. Discusión: en las formas de FRP asociadas con aneurisma aórtico abdominal se recomienda tratamiento quirúrgico o endovascular cuando el diámetro aórtico es superior a 5,5 cm o tiene un crecimiento rápido (> 1 mm al mes), ya que el peligro de ruptura parecería no ser mayor que para los aneurismas ateroscleróticos. Sin embargo, no existen pautas ni algoritmos a seguir en el manejo vascular de pacientes con FRP y una aorta “no aneurismática” ante el fracaso del tratamiento con esteroides. Por otro lado, está descrito el riesgo de remodelación arterial y dilatación aórtica progresiva durante el tratamiento con esteroides. Aunque tales cambios suelen ser subclínicos, se han reportado casos que requirieron tratamiento quirúrgico por ruptura, como en nuestro paciente, en el que la complicación ocurrió a pesar de tener un diámetro aórtico máximo inferior a 4 cm.(AU)

Introduction: retroperitoneal fibrosis (RPF) is a rare disease characterized by the formation of inflammatory and fibrous tissue in the retroperitoneal space, around the abdominal aorta and iliac arteries, often including the ureters. Case report: we present a 90-year-old male patient with RPF treated with meprednisone, who attended to the emergency room due to a ruptured inflammatory abdominal aortic aneurysm.Discussion: in RPFs associated with abdominal aortic aneurism, endovascular or surgical treatment is recommended when the aortic diameter is larger than 5.5 cm or when it is rapidly growing (> 1 mm/month) because the danger of rupture would appear to be the same as atherosclerotic aneurysms. However, there are no guidelines or algorithms to follow the vascular management of patients with PRF and a "non-aneurysmal" aorta in the event of failure steroid treatment. On the other hand, the risk of arterial remodeling and progressive aortic dilatation during steroids treatment has been described. Although the changes are subclinical, there were reported cases in which the patients received surgical treatment due to the rupture, such as in our patient where a complication occurred despite having a maximum aortic diameter of less than 4 cm. Recently, a series of 6 patients with RPF non-aneurysmal abdominal aorta, that had not responded properly to medical treatment, received infrarenal abdominal aorta endovascular exclusion.(AU)

A Review on The Role of Environmental Exposures in IgG4-Related Diseases.

PURPOSE OF REVIEW: 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). RECENT FINDINGS: 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.

Retroperitoneal Fibrosis: Still a Diagnostic Challenge.

Retroperitoneal fibrosis (RPF) is a rare fibroinflammatory disorder usually involving the abdominal aorta and surrounding structures. It is divided into primary (idiopathic) and secondary RPF. Primary RPF can be immunoglobulin (Ig) G4-related disease or non-IgG4-related disease. Recently, there has been a rise in case reports regarding the topic, but awareness about the disease is still far from ideal. Hence, we present the case of a 49-year-old female who had repeated admissions for chronic abdominal pain attributed to chronic alcoholic pancreatitis. She had a medical history significant for psoriasis and surgical history significant for cholecystectomy. Her computed tomography (CT) scans on each admission for the last year showed some signs of RPF, but it was never considered the primary etiology of her chronic symptoms. We also obtained magnetic resonance imaging (MRI) which did not show any underlying malignancy but showed the progression of her RPF. She was started on a steroid regimen, which significantly improved her symptoms. She was diagnosed with idiopathic RPF due to unclear etiology, although her underlying risk factors, including psoriasis, past surgeries, and pancreatitis-associated inflammation, were considered predisposing factors. Idiopathic RPF accounts for more than two-thirds of total cases of RPF. Patients with autoimmune diseases can overlap with other autoimmune disorders. For non-malignant RPF, medical management with 1mg/kg/day steroids is deemed effective. Still, there is a lack of prospective trials and consensus for guidelines on treating RPF. The follow-up involves laboratory tests, including erythrocyte sedimentation rate, C-reactive protein, and CT or MRI in an outpatient setting to identify treatment response and relapse. There is a need for more streamlined guidelines to diagnose and manage this disease.

Minimally invasive ureterolysis and intraperitonealization of ureter for idiopathic retroperitoneal fibrosis; single center analysis of an erratic disease.

INTRODUCTION: Idiopathic retroperitoneal fibrosis is a known cause of obstructive uropathy. Ureterolysis is done when medical management fails or the presentation is at an advanced stage. Conventionally ureterolysis without omental wrap has been considered incomplete. Our Institute has experience of laparoscopic or robotic ureterolysis with intraperitonealization of the ureter alone and no other adjunctive procedure. This study retrospectively assesses the result of the procedure with patients presenting with varying severity of disease. METHODS: From 2008, all patients who underwent laparoscopic or robotic ureterolysis were analyzed retrospectively for pre-operative management, operative findings, and post operative outcomes. RESULTS: We operated and released nine renal units in seven patients. Two of the nine cases were performed robotically completely and the rest was performed by laparoscopic approach. Median follow up was 60 months. All patients documented resolution of symptoms. The mean post-operative creatinine at 1 year was significantly decreased to 1.47 ± 0.49 mg/dl in comparison to preoperative creatinine (p < 0.05). The postoperative mean ESR decreased significantly from a preoperative value of 58.2 ± 19.41 mm to 15.8 ± 17.23. The nuclear scan revealed unobstructed drainage and radiological imaging revealed resolution of hydronephrosis and fibrosis in all. The mean GFR on the nuclear scan after 3 and 12 months of surgery was 36.3 ± 4.33 and 40 ± 3.77, respectively. Thus, there was significant increase noted in GFR at 3 and 12 months in comparison to preoperative GFR (p < 0.05). CONCLUSION: Laparoscopic/robotic ureterolysis with intraperitonealization alone is secure and durable procedure for idiopathic retroperitoneal fibrosis needing surgical release.

Idiopathic Retroperitoneal Fibrosis: What Is the Optimal Clinical Approach for Long-Term Preservation of Renal Function?

BACKGROUND: The aim of this study was to investigate the long-term effects of ureteral stenting and the exact timing of stent removal in favor of surgery in patients with idiopathic retroperitoneal fibrosis (IRF). SUMMARY: Medline research terms of "idiopathic retroperitoneal fibrosis" AND " medical therapy" OR "ureteral stenting" OR "surgical treatment" were done. Systematic reviews and observational and clinical studies were analyzed to obtain indication regarding the objective of the study for a narrative review. Ninety-two papers were analyzed. The treatment of IRF includes the monitoring of retroperitoneal fibrotic process spread and the prevention of abdominal organs entrapment. Treatment of ureteral obstruction includes medical therapy and ureteral stenting (US) or percutaneous nephrostomy (PNS) to overcome the worsening of renal function. Up to now, the timing of US or PNS removal is not yet clear, both for the complexity of evaluating the efficacy of the medical therapy and demonstrating the resolution of obstructive nephropathy. Moreover, it is not yet clear if the long-term ureteral stent placement or PNS is able to maintain an efficient renal function. Ureterolysis with a laparoscopic robot-assisted approach is now considered as an ultimate treatment for ureteral obstruction, limiting the progression of kidney impairment and improving the quality of life of patients, although nephrologists are generally abdicant regarding the potential switch toward the surgical approach. KEY MESSAGES: Prospective studies regarding the long-term effects of US on the renal function impairment in patients with IRF should be structured to obtain adequate information on the exact timing for the surgical approach.

Effective therapy of pirfenidone in a patient with idiopathic retroperitoneal fibrosis: report of a case.

Idiopathic retroperitoneal fibrosis (iRPF) is a chronic autoimmune disease characterized by fibroinflammatory tissue surrounding the abdominal aorta and iliac arteries and extending into the retroperitoneum to envelop neighboring structures. Hydronephrosis due to obstruction of ureters is the most common complication of iRPF. Glucocorticoid with or without immunosuppressants or tamoxifen, the mainstay of iRPF treatment, usually brings good response. Nevertheless, in some conditions, the obstruction of ureters remains unresolved with the treatment of all these medications. One of the reasons lies in the innate feature of the fibroinflammatory tissue. The proliferation of fibrosis tissue in addition to inflammation in the mass was associated with insufficient response to immunosuppressive therapies. Pirfenidone, an anti-fibrosis agent, has been successful in treating pulmonary fibrosis and renal fibrosis. Therefore, it is rationale to assume the effectiveness of pirfenidone in the treatment of iRPF. In the current article, we report a 61-year-old Chinese man with iRPF who responded well to pirfenidone.

Decreased absolute number of peripheral regulatory T cells in patients with idiopathic retroperitoneal fibrosis.

Objective: In order to determine whether the immune balance of T helper 17(Th17)/regulatory T(Treg) is related to the pathogenesis of idiopathic retroperitoneal fibrosis (IRPF), we analyzed the differences in peripheral blood lymphocytes, CD4+T cell subsets and cytokines between patients with IRPF and healthy people to clarify the CD4+T cell subsets, especially Treg cell subsets, and the role of cytokines in the pathogenesis of IRPF. Methods: This study included 22 patients with IRPF, 36 patients with IgG4-related diseases (IgG4-RD) without retroperitoneal fibrosis (RPF), and 28 healthy controls. The absolute numbers and percentage of peripheral blood lymphocyte subsets and CD4+T cell subsets in each group were detected by flow cytometry, and the serum cytokine level was detected by flow cytometric bead array (CBA). Results: Compared with the healthy group, the absolute value of B cells in peripheral blood of IRPF patients was significantly decreased, and T, natural killer (NK), CD4+ and CD8+ were not significantly abnormal. The absolute numbers of Th2 cells were lower than healthy group(p=0.043). In particular, the absolute numbers of Treg cells were significantly lower than healthy group(p<0.001), while the absolute numbers of Th17 cells increased(p=0.682). Th17/Treg was significantly higher than healthy group (p< 0.001). Cytokine analysis showed that the level of interleukin (IL)-4 in IRPF patients was higher than healthy group(p=0.011), IL-6, IL-10, IL-17, TNF-α and IFN-γ were significantly higher than healthy group (all p<0.001). Receiver operating characteristic (ROC) curves showed that IL-10 and TNF-α could distinguish bilateral ureteral dilatation in IRPF patients, with areas under the ROC curve (AUCs) of 0.813 (95% CI:0.607-1.000, p=0.026) and 0.950 (95% CI:0.856-1.000, p=0.001), respectively. IL-6 could distinguish bilateral ureteral obstruction, with an AUC of 0.861 (95% CI: 0.682-1.000, p=0.015). Conclusions: Our study showed that IRPF patients had reduced Treg cells and indeed had Th17/Treg imbalance, which may be related to the pathogenesis of the disease. The levels of IL-6, IL-10 and TNF-α appear to be associated with the progression of IRPF.

A Rare Co-association of Autoimmune Thyroiditis and Idiopathic Retroperitoneal Fibrosis.

Retroperitoneal fibrosis is a rare fibroinflammatory disease that is reported to be associated with other autoimmune conditions. Here, we report the case of a 51-year-old Caucasian female with a history of autoimmune thyroiditis and Hashimoto's hypothyroidism who presented with symptoms of fever, chills, and hot flashes for three weeks associated with nausea, vomiting, frequent thirst, and frequent urination. On examination, the patient had elevated blood pressure and an excoriated rash on the forearms. Laboratory evaluation showed elevated blood urea nitrogen and creatinine with a hypertensive emergency. Renal ultrasound showed bilateral hydronephrosis suggestive of obstructive uropathy. Computerized tomography of the abdomen and pelvis was suggestive of extensive retroperitoneal fibrosis. The patient was diagnosed with idiopathic retroperitoneal fibrosis without an identifiable secondary cause. Treatment was focused on relieving the ureteral obstruction, managing renal functions, and optimizing blood pressure, following which immunomodulatory agents were used.

Idiopathic Retroperitoneal Fibrosis Presented As Urinary Tract Obstruction.

Retroperitoneal fibrosis (RPF) or Ormond's disease is a very uncommon fibro-inflammatory disease, under the umbrella of systemic autoimmune diseases. The majority of cases are idiopathic, known as idiopathic RPF (IRPF); however, diseases secondary to other causes are also seen in clinical practice. The commonest presenting features are seen due to the effects of fibrous tissue around iliac vessels, aorta and ureters, where compression of ureters is the major and most common complication. Computed tomography (CT) scans and magnetic resonance imaging (MRI) are the modalities of choice for the diagnosis. The primary management involves medical therapy with corticosteroids and reserving surgical options for ureteric obstruction and related complications. We present a case of a 65-year-old man who presented with bilateral pedal oedema, facial puffiness, decreased appetite, decreased urine output, and breathlessness with dry cough, tachypnoea, hypoxia and crepitation in both lung fields on examination. The blood investigations were suggestive of acute kidney injury (AKI); whereas radio imaging diagnosed him as a case of bilateral hydroureteronephrosis with RPF. The patient was treated for AKI in the case of IRPF. Once the patient stabilized, a low-dose systemic steroid was started for IRPF, and subsequently, the patient underwent stent placement surgery for ureteric obstruction. RPF, being a rare disease, is difficult to diagnose. However, CT and MRI scanning can easily reveal fibrous tissue surrounding the aorta and ureters. Medical management with glucocorticoids is the backbone drug for the disease, keeping surgery as a reserved option for ureteric obstruction and its complications.

[Non-infectious diseases of the aorta and large arteries].

This article describes the various forms of inflammatory lesions of the aorta and large arteries, including chronic periaortitis, as well as the diagnostic methods are considered. Large vessel vasculitis represent the most common entities, however, there is also an association with other rheumatological or inflammatory diseases, drug-induced or paraneoplastic entities. Instrumental imaging modalities play an important role in the diagnosis.

Serum IgG4 Concentration Is a Potential Predictive Biomarker in Glucocorticoid Treatment for Idiopathic Retroperitoneal Fibrosis.

OBJECTIVES: To evaluate the management and outcome of idiopathic retroperitoneal fibrosis (iRPF) in Japan, and to identify its clinical biomarker. METHODS: We retrospectively analyzed 129 patients with iRPF treated between January 2008 and May 2018 at 12 university and related hospitals. Patients treated with glucocorticoid were analyzed to identify a predictive biomarker. These patients were classified into three groups according to overall effectiveness (no change: NC, complete response: CR, and partial response groups: PR), and each parameter was compared statistically. RESULTS: Male-female ratio was 5:1, and median age at diagnosis was 69 (33-86) years. Smoking history was reported in 59.6% of the patients. As treatment, 95 patients received glucocorticoid therapy with an overall response rate of 84%. As a result, serum concentration of IgG4 was significantly decreased in NC group compared with the other two groups (56.6 mg/dL vs. 255 mg/dL, 206 mg/dL, p = 0.0059 and 0.0078). ROC analysis was performed between the nonresponder (NC) and responder groups (CR + PR) to identify the cut-off value of serum IgG4 as a predictive marker. As a result, AUC of 0.793 was confirmed. CONCLUSIONS: Pre-treatment serum IgG4 concentration may have potential as a predictive biomarker of steroid treatment.

Idiopathic retroperitoneal fibrosis mimicking infiltrative malignancy: a case report.

The treatment strategy for an idiopathic retroperitoneal mass has not yet been established. Additionally, differentiating between benign and malignant is a challenge. Herein, we report a case in which we performed partial resection of a mass in a symptomatic patient with idiopathic retroperitoneal fibrosis that mimicked malignancy. A 44-year-old woman with an unremarkable medical history other than gallstones presented with a 1-month history of abdominal pain and repetitive vomiting. Imaging studies identified a large, retroperitoneal mass compressing the duodenum that had grown acutely over the preceding 2 weeks. The possibility that the mass was malignant could not be excluded. Considering the invasiveness and potential curability, we performed partial resection of the mass, which involved partial colonic resection with reconstruction, to allow for pathological diagnosis and intestinal obstruction treatment. The final pathological findings revealed that the mass consisted of hemorrhagic and fibrotic tissue without a tumorous component. The patient's postoperative course was unremarkable. She is alive 8 years postoperatively with no recurrence. In conclusion, a surgical approach, including biopsies, to idiopathic retroperitoneal fibrosis that mimics malignancy should be actively considered in symptomatic patients. Decisions regarding the required degree of surgical intervention call for sufficient, case-specific discussion.

The contribution of oncovascular surgery in a young patient with idiopathic retroperitoneal fibrosis.

Surgical resection remains the cornerstone for the treatment of oncological disease. When a critical arterial or venous structure is involved in a tumor mass, successful relief of symptoms and long-term oncological control are achieved through careful preoperative planning by an interdisciplinary team that necessarily includes a vascular surgeon. We describe the involvement of a vascular surgeon in the oncology of a 22-year-old woman, who is diagnosed with idiopathic retroperitoneal fibrosis.

Differences of clinicopathological features between IgG4-related and non-IgG4-related idiopathic retroperitoneal fibrosis.

OBJECTIVE: 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. METHODS: 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 hematoxylin & eosin staining, elastic-collagenous fiber staining, and immunohistochemical analysis. The clinical and pathological features between IgG4-related and non-IgG4-related IRF were analyzed. RESULTS: 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. CONCLUSIONS: 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.

Atypical Presentation of Idiopathic Retroperitoneal Fibrosis Effectively Treated With Colchicine After Lymphoma Misdiagnosis.

Idiopathic retroperitoneal fibrosis (RPF) is a rare disease characterized by a fibro-inflammatory mass encasing the abdominal aorta. We report a case of a 43-year-old man with an unusual presentation of RPF who was initially misdiagnosed with lymphoma. Our patient presented with constipation and did not have common findings such as ureteral displacement or renal impairment. Our patient had a complicated disease course complicated by multiple treatment failures and pulmonary embolism. We discuss the patient's first 100 months of treatment, which included the use of prednisone, mycophenolate, tamoxifen, methotrexate, azathioprine, and, now, colchicine. Our case demonstrates that physicians should maintain an index of suspicion for RPF in patients with a homogenously attenuated mass encasing the anterior aorta. It also serves as one example in which RPF appeared to be responsive to colchicine.

Surviving complications of retroperitoneal fibrosis with intermittent relapses over a period of 20 years: case report and literature review / Sobrevivir a las complicaciones de la fibrosis retroperitoneal con recaídas intermitentes durante un período de 20 años: reporte de un caso y revisión de la literatura

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?

La fibrosis retroperitoneal idiopática es una enfermedad fibroinflamatoria rara, de etiología variada que generalmente se origina alrededor de la aorta y se propaga caudalmente a lo largo de los vasos ilíacos en retroperitoneo adyacente causando obstrucción ureteral como la complicación más frecuente.Reportamos el caso de un paciente varón de 53 años que se presentó con un dolor leve en ambas piernas. Al investigar más a fondo, descubrimos que había estado luchando con recaídas intermitentes cada 3-4 años durante los últimos 20 años desde que se le diagnosticó por primera vez fibrosis retroperitoneal idiopática. Tenía 33 años cuando desarrolló por primera vez los síntomas de anuria durante 48 horas y se le diagnosticó fibrosis retroperitoneal idiopática. Esto fue seguido por atrofia del riñón izquierdo e hipertensión 6 años después, luego hipotiroidismo después de otros 3 años y finalmente afectación de la vena cava inferior y trombosis venosa profunda aguda de las extremidades inferiores después de otros 3-4 años. Su trombosis venosa profunda se controló bien a tiempo. Le recetaron glucocorticoides cada vez que tenía una recaída y una complicación.Hicimos una revisión de la literatura para comprender los avances recientes sobre su patogenia, diagnóstico, investigaciones y manejo. Se realizaron búsquedas en PubMed utilizando términos como fibrosis retroperitoneal sola y en combinación con términos relacionados como trombosis de la vena cava inferior, trombosis venosa profunda, tamoxifeno, metotrexato. Este caso es único, ya que es muy raro encontrar trombosis venosa profunda aguda en fibrosis retroperitoneal idiopática sin desarrollo de colaterales cuando la luz de la vena cava inferior está comprometida hasta una obstrucción casi completa.Después de un seguimiento de 20 años, el paciente se encuentra bien en términos de actividad física y bienestar psicológico con antihipertensivos, tiroxina y anticoagulantes. ¿El intervalo libre de enfermedad está realmente libre de la enfermedad o simplemente disminuyó con inmunosupresores para activarse después de algún tiempo?

Characteristics of Adult Patients with Idiopathic Retroperitoneal Fibrosis and Assessment of Risk of Relapse at Diagnosis.

OBJECTIVES: To compare adult patients' characteristics suffering from idiopathic retroperitoneal fibrosis between "relapse-free" and relapsing patients at the diagnosis and identify factors associated with relapse at initial presentation. METHODS: We conducted a retrospective multicentric study in four hospitals in Eastern France, from 1993 to 2020, of adult patients suffering from idiopathic retroperitoneal fibrosis. We analyzed clinical, biological, and radiological features at diagnosis and during a forty-month follow-up. RESULTS: Of 47 patients suffering from retroperitoneal fibrosis, 21 patients had idiopathic retroperitoneal fibrosis. Among them, 13 experienced one or more relapses during follow-up. At diagnosis, clinical characteristics, relevant comorbidities, biological and radiological features were similar between groups. Smoking cessation seems associated with decreased relapse risk (p: 0.0624). A total of 8 patients developed chronic renal failure during follow-up. Ureteral infiltration at diagnosis was associated with evolution to chronic renal failure (p: 0.0091). CONCLUSION: No clinical, biological, or radiological features could predict relapse at retroperitoneal fibrosis diagnosis, but smoking cessation may prevent relapse.

Idiopathic retroperitoneal fibrosis: an update for nephrologists.

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 of IRF, which can lead to acute renal failure and, in the long term, to varying degrees of chronic kidney disease. IRF may be isolated or develop in association with autoimmune diseases (e.g. Hashimoto's thyroiditis and psoriasis) and other fibro-inflammatory disorders (often within the spectrum of immunoglobulin G4-related disease), which suggests that it should be considered as a potentially systemic condition. IRF is an immune-mediated disease: genetic variants (e.g. human leukocyte antigen (HLA)-DRB1*03) and environmental agents (mainly exposure to asbestos and smoking) are strongly associated with an increased risk of developing the disease, while a complex network of chemokines (e.g. CXCL12 and C-C moti chemokine 11 (CCL11)) and cytokines [e.g. interleukin (IL)-6, IL-12 and IL-13] is likely to orchestrate the inflammatory response and simultaneously promote fibrosis. Glucocorticoids, alone or in combination with traditional immunosuppressants such as methotrexate and mycophenolate mofetil, are usually efficacious and promptly induce disease remission; however, up to 50% of patients relapse, thus requiring repeat immunosuppressive courses. Biologic drugs, namely rituximab, are being explored for the treatment of IRF. In addition to medical therapies, interventional procedures (mainly ureteral stenting) are required to relieve ureteral obstruction, whereas surgical ureterolysis is generally reserved to refractory cases. If appropriately treated, then the overall and renal prognosis of IRF are good, with <5% patients developing end-stage renal disease.