Chronic periaortitis: A clinical approach.

Chronic periaortitis (CP) is a rare disease characterised by the presence of a fibro-inflammatory tissue typically enveloping the abdominal aorta, the iliac arteries and, in some cases, the nearby structures, such as the ureters and the inferior vena cava. Imaging plays a key role in the diagnosis and follow-up: computed tomography and magnetic resonance imaging scans are used to define the extension of the pathological tissue, whereas fluorodeoxyglucose positron emission tomography is the gold standard to establish the degree of its metabolic activity. CP must be distinguished from secondary forms of periaortic infiltration, which include malignant, infectious, and drug-related aetiologies. This review focuses on the clinical aspects of CP and the differential diagnosis with secondary cases, and aims to provide the clinician with a guide through this challenging clinical approach.

Multidisciplinary diagnosis and management of inflammatory aortic aneurysms.

BACKGROUND: Inflammatory abdominal aortic aneurysms (IAAAs) are a variant involving a distinct immunoinflammatory process, with nearly one half believed to be associated with IgG4-related disease (IgG4-RD). METHODS: MEDLINE and Google Scholar searches were conducted for English-language publications relevant to inflammatory aortic aneurysms from January 1970 onward. The search terms included inflammatory aortic aneurysms, aortitis, periaortitis, IgG4-related disease, and retroperitoneal fibrosis. Relevant studies were selected for review based on their relevance. RESULTS: Morphologically, IAAAs are characterized by a thickened aneurysm wall often displaying contrast enhancement and elevated metabolic activity on fluorine-18 fluorodeoxyglucose-positron emission tomography imaging. A strong association exists with perianeurysmal and retroperitoneal fibrosis. Although the rupture risk appears lower with IAAAs than with noninflammatory abdominal aortic aneurysms (AAAs), the currently recommended diameter threshold for operative management is the same. Open repair has been associated with increased morbidity compared with noninflammatory AAAs, and a retroperitoneal approach or minimal dissection transperitoneal approach has been recommended to avoid duodenal and retroperitoneal structural injuries. Endovascular aneurysm repair has been increasingly used, especially for patients unfit for open surgery. It is important to exclude an infectious etiology before the initiation of immunosuppressive therapy or operative repair. Multimodality imaging follow-up is critical to monitor disease activity and secondary involvement of retroperitoneal structures by the associated fibrotic process. Maintenance of immunosuppressive therapy will be needed postoperatively for most patients with active systemic disease, especially those with IgG4-RD and those with persistent symptoms. Additional interventions aimed at ureteral decompression could also be required, and lifelong follow-up is mandatory. CONCLUSIONS: Preoperative multimodality imaging is a diagnostic cornerstone for assessment of the disease extent and activity. IgG4-RD is an increasingly recognized category of IAAAs, with implications for tailoring adjunctive medical therapy. Open surgical repair remains the procedure of choice, although endovascular aneurysm repair is increasingly being offered. Maintenance immunosuppressive therapy can be offered according to the disease activity as assessed by follow-up imaging studies.

An international patient-centred study of retroperitoneal fibrosis.

BACKGROUND: The impact that rare chronic disorders, such as retroperitoneal fibrosis (RPF), can have on the physical and psychological aspects of a patient's health is poorly understood. Patient-related outcome measures and experiences provide a unique opportunity to understand the impact rare chronic disorders have on a patient's life as well as allowing healthcare providers to compare and improve performance. AIM: To understand the physical and psychosocial impact that RPF has upon peoples' lives. DESIGN: An international online questionnaire was therefore created to gain insights into how patients with RPF, a rare fibro-inflammatory condition, viewed their health and experiences. METHODS: An international online questionnaire comprising 62 questions/free text options, was designed in collaboration with two patient advocates and the multi-disciplinary Renal Association Rare Disease Registry (RaDaR) RPF Group the questionnaire was anonymous and freely accessible on a GOOGLE Form online platform for 6 months. RESULTS: A total of 229 patients from 30 countries across 5 continents responded. Four key issues were identified; (i) pain; (ii) therapy-related side effects; (iii) lack of informed doctors/information about their condition and its management; and (iv) psychological burden. Variations in diagnosis and management are highlighted with 55% undergoing a biopsy to reach a diagnosis of RPF; 75% of patients underwent a further interventional procedure with 60% concurrently treated medically. CONCLUSION: This study will guide further development of clinical and academic multi-disciplinary activity and shows the importance of trying to understand the impact of rare chronic disorders on the physical and psychological aspects of a patient's health.

Retroperitoneal Fibrosis Is Still an Underdiagnosed Entity with Poor Prognosis.

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.

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?

[Retroperitoneal fibrosis at the Ibn Sina University Hospital of Rabat: about 18 cases]. / Fibrose rétropéritonéale au CHU Ibn Sina de Rabat: à propos de 18 cas.

Retroperitoneal fibrosis (RPF) is a rare disease characterized by the formation of a fibro-inflammatory plaque in the retroperitoneal space in front of the abdominal aorta. It is responsible for the sheathing of the ureters. It is characterized by unspecific clinical signs, and it is often detected based on obstructive uropathy. We conducted a retrospective, descriptive study in the Department of Urology B and in the Department of Nephrology of the Ibn Sina University Hospital over a period of 10 years from January 2006 to December 2016. The study enrolled 18 patients, including 11 men and 7 women, with an average age of 51.4 years ± 11.2. Diagnosis was based on lumbar pain in 14 patients. Obstructive renal failure was reported in 15 patients and the diagnosis of RPF was based on uroscanner. Etiological assessment revealed 2 cases with a history of neoplasia, 2 cases of inflammatory disease and 1 case of retroperitoneal surgery; chronic drug intake was noted in more than half of the patients. In all patients, treatment was based on double J stent placement, while systemic treatment with corticosteroids and immunosuppressants was used on the basis of the evolutionary profile. Ureterolysis was immediately performed in 3 patients. Outcome was favorable, with significant improvement in renal function in 12 patients. A relapse occurred in 2 patients after 2 years of follow-up. Retroperitoneal fibrosis (RPF) should be suspected in patients with general signs associated with obstructive renal disease. Secondary causes should be systematically investigated, with particular emphasis on hyper IgG4 disease and neoplastic diseases.

Idiopathic Retroperitoneal Fibrosis.

The world of surgery has been concerned with idiopathic retroperitoneal fibrosis (RPF) ever since 1948, when J. K. Ormond published the first case of ureteral stenosis following a retroperitoneal scleroinflammatory process due to unknown reasons. Until 1960, 82 such cases were reported in the literature. The authors present two new cases of RPF, located exclusively in the pelvic (subperitoneal) compartment of the retroperitoneal space. The literature on this subject is reviewed, current etiological theories are mentioned and treatment opportunities are discussed.

Into Clinical Practice: Diagnosis and Therapy of Retroperitoneal Fibrosis.

PURPOSE OF THE REVIEW: We aim to review the most relevant diagnostic features and treatment options of retroperitoneal fibrosis, in order to provide a useful guide for clinical practice. RECENT FINDINGS: The recent literature highlights the role of imaging studies such as computed tomography, magnetic resonance imaging and positron emission tomography as useful tools for the diagnosis of retroperitoneal fibrosis, with retroperitoneal biopsy being reserved to atypical cases. The treatment approach is mainly conservative and is based on the use of medical therapies plus urological interventions. Medical therapies essentially comprise glucocorticoids and immunosuppressants-either traditional or biological agents such as rituximab. Surgical ureterolysis is only left for refractory cases. Recent findings in retroperitoneal fibrosis highlight the possibility of a non-invasive diagnostic approach and a conservative treatment strategy.

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.

La fibrose rétropéritonéale idiopathique.

Retroperitoneal fibrosis (RPF) is a rare disease characterized by the presence of fibro-inflammatory tissue around the aorta entrapping the adjacent structures. RPF can be idiopathic or secondary to many disorders. The physiopathology is unknown but can be part of the spectrum of IgG4 related diseases. Imaging studies and inflammatory markers are essential for initial evaluation and follow-up. Biopsy is usually not recommended. The first line of treatment is corticosteroids associated or not with immunosuppressive drugs. In case of ureteral obstruction with renal failure, ureteral stent placement or nephrostomies are recommended. Initial response to treatment is usually good but relapses are frequent.

Current approach to diagnosis and management of retroperitoneal fibrosis.

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.

Characteristics and prognosis of IgG4-related periaortitis/periarteritis: A systematic literature review.

OBJECTIVE: Immunoglobulin G4 (IgG4)-related disease is a systemic chronic fibroinflammatory disease that can affect almost every organ of the body. IgG4-related periaortitis/periarteritis is a newly recognized subset of IgG4-related disease, and its characteristics and prognosis remain unclear. We investigated the clinical characteristics and prognosis of IgG4-related periaortitis/periarteritis. METHODS: We performed a systematic literature review of IgG4-related periaortitis/periarteritis. Additionally, we have summarized the characteristics and prognosis of IgG4-related coronary arteritis. RESULTS: We investigated 248 patients with IgG4-related periaortitis/periarteritis. All studies reported the condition in elderly patients, and male predominance was observed. The infra-renal abdominal aorta and iliac arteries were the most commonly affected sites. Most reports showed the serum C-reactive protein elevation in this disease entity, in contrast to non-vascular IgG4-related disease. Based on radiological findings observed in 27 patients with IgG4-related coronary arteritis, vasculitic lesions were classified into 3 types: stenotic (67% of patients), aneurysmal (42%), and diffuse wall thickening type (92%). Serum IgG4 level, but not C-reactive protein level, was associated with the number of affected organs in IgG4-related coronary arteritis. Corticosteroid treatment with or without cardiac surgery or percutaneous coronary intervention was effective in most patients with IgG4-related coronary arteritis; however, 33% of patients showed an unfavorable clinical course including disease progression, relapse, or death. Pre-treatment stenosis and/or aneurysms were associated with progression of stenosis or aneurysm after corticosteroid treatment. CONCLUSION: Most clinical characteristics were similar between the IgG4-related periaortitis/periarteritis and the non-vascular IgG4-related disease groups; however, serum C-reactive protein level elevation was observed only in the former. Although corticosteroid treatment was effective, this disease can be life-threatening secondary to myocardial infarction, aortic dissection, and aneurysmal rupture. Pre-treatment evaluation of stenosis or aneurysms is important for predicting progression of stenosis or aneurysm after corticosteroid treatment.

Risk factors for relapse and long-term outcome of idiopathic retroperitoneal fibrosis.

BACKGROUND: Retroperitoneal fibrosis (RF) is a rare disease of unclear etiology characterized by the presence of fibroinflammatory tissue in the retroperitoneal space, which can entrap and obstruct retroperitoneal structures, notably the ureters. The disease responds well to steroid therapy, but tends to recur even after years. The aim of our study was to evaluate the long-term renal outcome of patients affected by idiopathic retroperitoneal fibrosis looking for predictive risk factors for recurrence of the disease and progression to end-stage renal disease. METHODS: Retrospective observational study of patients with idiopathic RF diagnosed from 2004 to 2017 and follow-up of at least 1 year after the end of first course therapy with steroid, with or without tamoxifen (TMX) and with urological procedures when applicable. RESULTS: Forty-three patients were included in the study. The follow-up was 93 ± 52 months. All the patients obtained remission after therapy that was maintained until the last observation in 26 of them. In 17 patients, there was at least one recurrence. Risk factors associated with relapse were identified and resulted in smoking habit, onset with acute kidney injury (AKI), low back pain and antinuclear antibodies (ANA) positivity. Renal function remained fairly stable during the long-term follow-up. The renal end-point (doubling of serum creatinine or ESRD) occurred in 8% of the patients; however, eGFR in patients with relapse was similar to that of non-recurrent at the diagnoses, but it decreased over time more in the relapsing than in non-relapsing patients (p group = 0.20; p time = 0.001; p time × group interactions = 0.04). Based on these 4 predictor conditions, patients were divided into "low risk" (with 0-1 risk factor), and "high risk" (3-4 risk factors). The renal end-point occurred in 40% of high-risk patients, while none of the low-risk patients reached it (p = 0.02). CONCLUSIONS: Smoking habit, AKI at diagnosis, ANA positivity and lumbar pain were associated with relapse of RF after initial remission due to steroid and/or TMX therapy; the combination of these conditions was also predictive of worse renal function outcome. Identification of risk factors for relapse can be useful not only to modulate the choice, the dosage of first-line treatment and the duration of maintenance therapy but also for preventing a progressive loss of kidney function, as well.

IgG4-related Retroperitoneal Fibrosis: An Emerging Masquerader With a Sinister Presentation.

OBJECTIVE: Retroperitoneal fibrosis (RPF) is a rare proliferative fibro-inflammatory disease involving the soft tissues of the retroperitoneum. IgG4 related retroperitoneal fibrosis is an emerging entity which needs to be distinguished from idiopathic RPF. We describe a clinical case of IgG4 related RPF highlighting the importance of clinching this diagnosis. METHODS: A 70 year old female was referred to the outpatient department of our institute with complaints of fatigue, bilateral flank pain and loss of appetite for the past 1 month. The CT and PET scan demonstrated a uniformly enhancing bulky retroperitoneal mass causing bilateral hydroureteronephrosis. The biopsy from the mass lesion revealed IgG4 related disease. The patient was started on corticosteroids after percutaneous nephrostomy placement. RESULTS: Three months post induction of therapy, repeat PET-CT shows resolution of the mass with no FDG avid lesion. Serum IgG4 levels were reduced to normal (27 mg/dL) suggestive of response to treatment. The percutaneous nephrostomies were removed and the patient is doing well on maintenance dose of corticosteroids for her disease. CONCLUSION: The availability of serum IgG4 levels for monitoring treatment response and follow-up can curtail the repeated radiological imaging and associated contrast exposure as compared to idiopathic RPF. Secondly, the diagnosis of IgG4-related RPF shall alert the clinician to look out for extra-retroperitoneal diseases on follow up of this multi-organ disease.

Clinical and Prognostic Significance of Serum IgG4 in Chronic Periaortitis. An Analysis of 113 Patients.

Objective: Chronic periaortitis (CP) is a rare fibro-inflammatory disorder that incorporates idiopathic retroperitoneal fibrosis, inflammatory abdominal aortic aneurysms, and perianeurysmal retroperitoneal fibrosis. CP is included in the spectrum of IgG4-related disease. Since CP patients rarely undergo diagnostic biopsies, serum IgG4 levels are often used to classify CP as IgG4-related. However, the clinical and prognostic significance of serum IgG4 in CP is unknown. Methods: We measured serum IgG4 in active CP patients and compared the clinical characteristics, response to therapy and outcome of patients with high and normal levels. We also tested the diagnostic significance of IgG4 by comparing its levels in CP patients, healthy and disease controls (malignancies, Erdheim-Chester disease, large-, and small-vessel vasculitis). Results: We studied 113 consecutive patients with active CP. Twenty-four (21.2%) had high serum IgG4 (>135 mg/dL). The demographic, laboratory, and clinical characteristics of patients with high and normal IgG4 were similar, and so were the rates of ureteral obstruction and the disease characteristics on CT, MRI, and 18F-FDG-PET. Patients with high IgG4 only had a higher frequency of extra-retroperitoneal fibro-inflammatory lesions (p = 0.005). There were no significant differences in response to therapy and relapses between the two groups. Serum IgG4 levels did not discriminate CP from controls. Conclusions: Serum IgG4 levels are high in a minority of CP patients and do not identify specific clinical or prognostic subgroups; only a higher frequency of extra-retroperitoneal lesions is found in high-IgG4 patients. Serum IgG4 levels do not help in the differential diagnosis between CP and its mimics.

Impact of hybrid molecular imaging in retroperitoneal fibrosis: a systematic review.

The aim of this article was to critically assess the usefulness of hybrid molecular imaging (FDG PET/CT and FDG PET/MR) procedures in the evaluation of inflammatory activity in retroperitoneal fibrosis (RPF). A systematic review of the literature was performed using PubMed without timeline restriction and using the following keywords: retroperitoneal fibrosis, disease activity, diagnostic techniques, PET/CT, PET/MR. We evaluated full text articles written in the English language. Case reports, review articles or editorials and articles not in the field of interest of this review were excluded. Nine articles comprising a total of 186 patients met the inclusion criteria and were included and described in this systematic review. The new hybrid molecular imaging methods give promising results in the evaluation of the activity of the disease, quantification and prediction of therapeutic response and in tailoring medical therapy in RPF. FDG PET/CT can be a valuable tool in detecting disease activity, particularly in asymptomatic patients with RPF with acute phase reactant increase. Hybrid imaging can predict therapy response outcome and the best time for stent removal. Although PET/MR has potential advantage in small lesions and has reduced radiation exposure in comparison to PET/CT, PET quantification parameters have potentially higher diagnostic value over MR parameters in the evaluation of RPF. Acute phase reactants alone may not be reliable for the management and follow-up assessment of the disease. Hybrid imaging in RFP could be more comfortable, more accurate, with less radiation burden than different separate imaging studies acquired at different points in time.

Chronic Tubulo-interstitial Pyelonephritis with Retroperitoneal Fibrosis.

A large proportion of patients with retroperitoneal fibrosis (RPF) have been labelled as having idiopathic RPF historically. Recent reports of a subset of these patients having a fibro-inflammatory condition with characteristic histology and good response to therapy has helped in renewed understanding of an old disease. A 40 year old man presented with vague abdominal discomfort of 5 months duration. Ultrasound examination revealed soft tissue thickening with calcification over lower abdominal aorta. CT scan characterized the lesion as a retroperitoneal mass encasing the aorta with an associated lesion in upper pole of left kidney. A laparoscopic surgery and biopsy of the mass lesions were performed. It was reported as chronic tubulo-interstitial disease with retroperitoneal fibrosis. In the post-operative period, patient developed fever with weight loss. An 18F-FDG PET-CT done revealed FDG-avid retroperitoneal mass lesion. Serum IgG-4 levels were elevated and review of biopsy revealed features of IgG4 related disease. Patient had good response to treatment with steroids and azathioprine.

[IgG4-Related Retroperitoneal fibrosis]. / FIBROSIS RETROPERITONEAL RELACIONADA IgG4.

OBJECTIVE: To report two new cases of IgG4-related retroperitoneal fibrosis, a recently described pathology. METHODS: We analyze two cases diagnosed in our center and performed a literature review. RESULT: IgG4 related disease is a recently described entity that includes previously not related pathologies. The clinical manifestations are highly variable and its presentation is usually subacute. The treatment of choice is glucocorticoids. In our first case the outcome was favorable with corticosteroids and azathioprine. However, the second case required surgery on 2 occasions with radical nephrectomy. The diagnosis of the latter was made nine years after the onset of symptoms when the biopsy was reviewed; at that moment immunosuppressive therapy was not started. CONCLUSIONS: It is very important to know and diagnose this disease because of the good response to treatment that prevents complications.