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Immunoglobulin G4 (IgG4) disease is a recently described entity with a growing list of associated disorders. The most common manifestations are pancreatitis, infiltration of the salivary or lacrimal glands (Mikulicz syndrome) and retroperitoneal fibrosis. We report the case of a patient whose polycythaemia was secondary to IgG4 disease. Through a review of the literature, we discuss the clinical, radiological and therapeutic aspects of this rare condition. [ABSTRACT FROM AUTHOR]

Retroperitoneal fibrosis (RPF) is a rare disease characterized by the formation of a fibroinflammatory 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 [ABSTRACT FROM AUTHOR]

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

Introduction: While IgG4-related disease (IgG4-RD) was initially described in the early 2000s, its polymorphic clinical manifestations were previously reported under different names ; they have in common the presence of IgG4+ oligoclonal plasma cells and fibrosis., State of the Art: Ruling out certain differential diagnoses, the diagnosis of IgG4-RD is based on a bundle of clinical, biological and histological features. Chest involvement is variable and can affect the mediastinum, bronchi, parenchyma, pleura and/or, more rarely, bones and (pericardium, aorta, coronary…) vascular structures. The most frequent radiological manifestations are peribronchovascular thickening, mediastinal lymphadenopathy, and nodular or interstitial patterns. Pleural involvement and posterior mediastinal fibrosis are less frequent, while thoracic paravertebral tissue thickening is highly specific. Systemic corticosteroids are the cornerstone of treatment. In case of relapse or as frontline therapy in case of risk factors for relapse and/or poor tolerance of corticosteroids), a steroid-sparing agent (most often rituximab) is added, and biannual maintenance infusions are associated with a lower risk of relapse., Perspectives: An international consensus has recently led to the development of classification criteria that should standardize the diagnostic approach and homogenize the enrolment of patients in epidemiological as well as therapeutic studies. Other treatments are also under evaluation, including biologics targeting T2 inflammation, CD-19 (inebilizumab, obexelimab), SLAMF7 (elotuzumab) surface proteins, Bruton's tyrosine kinase, and the JAK/STAT pathway., Conclusions: Substantial progress has been made over recent years in understanding IgG4-RD pathophysiology, and personalized patient care seems to be an achievable medium-term goal., (Copyright © 2023 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

La fibrose rétropéritonéale (ou maladie d'Ormond) est une pathologie rare et insidieuse. Elle réunit un ensemble d'affections caractérisées par la présence d'un tissu fibro-inflammatoire aberrant. Le défi, pour le clinicien, réside dans la distinction entre sa forme primitive et ses formes secondaires à des pathologies infiltratives, infectieuses ou malignes. La fibrose rétropéritonéale secondaire à la tuberculose est rarement rapportée dans la littérature, son diagnostic est difficile et ses mécanismes physiopathologiques ne sont pas clairs. Nous rapportons dans cette observation le cas d'un patient âgé de 53 ans présentant des rachialgies inflammatoires en rapport avec une fibrose rétropéritonéale secondaire à une spondylite tuberculeuse débutante traitée efficacement par les antibacillaires. [ABSTRACT FROM AUTHOR]

Résumé Introduction La fibrose rétropéritonéale (FRP) est une affection rare caractérisée par l’engainement des structures rétropéritonéales par un tissu fibro-inflammatoire. Elle peut être isolée ou associée à une maladie sous-jacente. En l’absence d’attitude homogène et consensuelle, l’objectif de cette étude était, d’une part, d’analyser les caractéristiques principales au diagnostic et, d’autre part, d’évaluer la pertinence et la rentabilité des examens complémentaires réalisés au diagnostic à la recherche d’une cause ou maladie sous-jacente. Méthodes Soixante-dix-sept patients vus entre 1997 et 2011 ont été inclus dans cette étude rétrospective multicentrique. Le diagnostic de FRP reposait avant tout sur les données de l’imagerie. Les cas de fibrose péri-anévrysmale et ceux associés à un contexte évident de néoplasie sous-jacente étaient exclus. Résultats Au terme des explorations réalisées au diagnostic, la FRP était considérée comme primitive ou « idiopathique » dans 62 cas (80,5 %). Les biopsies chirurgicales ( n = 31) ou sous scanner ( n = 9) réalisées chez la moitié des patients ont montré dans 98 % des cas des lésions fibro-inflammatoires non spécifiques. Parmi les autres examens complémentaires réalisés, une biopsie ostéomédullaire a été réalisée chez 23 patients conduisant au diagnostic de lymphome malin non hodgkinien B de bas grade chez un seul patient porteur par ailleurs d’une IgM monoclonale. La recherche systématique d’auto-anticorps et le dosage de marqueurs tumoraux n’ont été d’aucun apport diagnostique. Conclusions Bien que la démarche diagnostique soit hétérogène, aucune cause et/ou maladie associée n’est retrouvée dans la majorité de cas de fibrose rétropéritonéale lorsque la présentation radiologique est typique. En l’absence d’éléments cliniques ou paracliniques d’orientation, la réalisation systématique d’examens non invasifs (marqueurs d’auto-immunité, marqueurs tumoraux et scintigraphie osseuse) ou invasifs (biopsie de la fibrose et biopsie ostéomédullaire) ne semble pas pertinente au vu de cette analyse. Introduction Retroperitoneal fibrosis (RPF) is a rare disorder characterized by the sheathing of retroperitoneal structures by fibro-inflammatory process. It can be either isolated or associated with an underlying disease or condition. In the absence of consistent and consensual approach, the objective of this study was to assess the relevance of diagnostic tests performed during the diagnostic work-up of RPF. Methods Seventy-seven patients were included in this retrospective multicenter study. The diagnosis of RPF was defined by the presence of a thickened circumferential homogeneous tissue unsheathing the infrarenal aorta, excluding peri-aneurysmal fibrosis and a clear evidence of a cancer. Results In 62 cases (80.5%), the RPF was considered as being primary or “idiopathic”. Surgical ( n = 31) or CT-guided ( n = 9) biopsies of the RPF were performed in half of the patients showing some fibrotic or non-specific inflammatory lesions in 98% of cases. A bone marrow biopsy was performed in 23 patients leading to diagnosis of low grade B cell non-Hodgkin lymphoma in a single patient who also had a monoclonal gammopathy IgM. The systematic search for autoantibodies or serum tumor markers was of no diagnostic value. Conclusions Although the diagnostic procedure was heterogeneous, no cause or associated disease was found in the majority of cases of FRP in this series. In the absence of any clinical or paraclinical evidence suggesting an underlying disease or any atypical features at presentation, a number of non-invasive tests (autoantibodies, tumor markers, bone scintigraphy) and also more invasive diagnostic tests (bone marrow and RPF biopsies) seem of little relevance. [ABSTRACT FROM AUTHOR]

Résumé Les aortites sont principalement retrouvées au cours des maladies inflammatoires comme l’artérite de Takayasu, l’artérite à cellules géantes ou la maladie de Behçet. Dans certains cas, l’aortite est dite idiopathique lorsqu’aucune de ces causes n’est retrouvée. Certains diagnostics différentiels doivent cependant être évoqués car leur prise en charge est bien différente. En premier lieu, il faut éliminer une cause infectieuse de l’aortite car leur rapidité d’évolution et leur pronostic à court terme en font une urgence thérapeutique. Ensuite, des diagnostics différentiels plus rares sont à connaître comme la fibrose rétropéritonéale le plus souvent idiopathique, mais parfois associée à une néoplasie ou une hémopathie. La maladie associée aux IgG4, la maladie d’Erdheim-Chester et l’anévrisme inflammatoire athéromateux sont d’autres diagnostics différentiels à évoquer devant une aortite inflammatoire afin d’adapter au mieux la prise en charge de ces patients. Aortitis are mainly described in inflammatory disorders such as Takayasu arteritis, giant cell arteritis or Behçet's disease. Aortitis is sometimes qualified as idiopathic. However, differential diagnoses must be searched since they need specific interventions. Infectious aortitis should be ruled out first as its rapid evolution and short-term poor prognosis makes it a therapeutic emergency. Furthermore, rarer differential diagnoses should be known as they require specific care that might sometimes differ from the treatment of inflammatory aortitis, such as retroperitoneal fibrosis mostly idiopathic but also secondary to neoplasia or malignant hemopathies. IgG4 related disease, Erdheim-Chester disease and inflammatory abdominal aortic aneurysm due to atherosclerosis are other differential diagnoses to mention in the presence of aortitis in order to adapt patients’ care consequently. [ABSTRACT FROM AUTHOR]

Résumé: Propos: La fibrose rétropéritonéale (FRP) est une maladie rare aux étiologies multiples. Notre objectif était d’analyser les stratégies diagnostiques, la répartition nosologique, le suivi, le traitement et le pronostic. Méthodes: Étude rétrospective de cohorte d’un centre universitaire. Résultats: Dix-huit patients (11 hommes) ont été suivis entre 1996 et 2009. Le chiffre inaugural de CRP était élevé dans tous les cas. Le scanner, associé ou non à l’IRM et au TEP 18-FDG, a confirmé le diagnostic chez 15 patients. Trois patients ont eu une biopsie à visée diagnostique. Dix patients présentaient une forme secondaire : vascularite systémique (granulomatose avec polyangéite, n =1, maladie de Takayasu, n =2), fibrose systémique disséminée avec thyroïdite de Riedel (n =1), périaortite athéromateuse (n =6). Quinze patients ont été traités par corticoïdes avec une durée moyenne de traitement de 60 mois (12–228 mois). Les patients présentant une vascularite étaient, au diagnostic, plus jeunes avec un syndrome inflammatoire plus important ; ils ont présenté des rechutes et une corticodépendance plus fréquentes. Un traitement immunosuppresseur n’a été associé que dans les formes secondaires à une vascularite. Il n’y a eu aucun décès avec un recul de 6±2 ans. Conclusion: Un panel large d’examens complémentaires a permis de définir le caractère secondaire de la FRP dans 60 % des cas. Le risque de rechutes et la réponse au traitement semblent conditionnés par la cause de la fibrose, justifiant un bilan initial systématique. [ABSTRACT FROM AUTHOR]

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Résumé: L’atteinte aortique est une complication potentiellement mortelle de la spondylarthrite ankylosante (SA), qui survient généralement tardivement au cours de l’évolution de cette maladie fréquente. Des lésions inflammatoires évoluant jusqu’à la fibrose sont localisées essentiellement dans la racine aortique entraînant un reflux, mais ce processus fibreux peut s’étendre jusqu’à l’oreillette gauche (bombement sous-aortique) touchant la valve mitrale et le système de conduction cardiaque. Des blocs auriculoventriculaires de premier, deuxième et troisième degré sont les anomalies de la conduction les plus fréquemment rapportées, parfois de façon transitoire. Une périaortite chronique a été décrite chez les patients atteints de SA. Elle se caractérise par une inflammation évoluant jusqu’à la fibrose dans le rétro péritoine périaortique et péri iliaque, à l’origine d’une compression des uretères, des veines, des artères et des canaux lymphatiques. Le traitement est endoscopique et/ou chirurgical, avec administration de glucocorticoïdes (GC), en association à des agents immunosuppresseurs. Les praticiens devraient penser à une aortite (avec des anomalies du système de conduction) et à une périaortite au cours de la SA car elles peuvent influencer significativement le pronostic de la maladie et nécessiter un traitement urgent. [ABSTRACT FROM AUTHOR]

Résumé: La survenue d’une fibrose rétropéritonéale a été rapportée chez plusieurs patients atteints de granulomatose de Wegener (GW), mais seuls trois cas isolés de lésions dorsales prévertébrales, ressemblant fortement à une médiastinite fibrosante, ont été publiés à ce jour. Nous rapportons quatre nouveaux cas de patients atteints de GW (deux hommes, deux femmes), âgés de 49 à 59ans au moment du diagnostic, présentant des lésions dorsales prévertébrales, localisées principalement à droite, associées à un épaississement de la plèvre dans deux cas. Ces lésions ont été détectées sur le scanner réalisé au moment du diagnostic chez deux patients et sont survenues plus tard chez les deux autres. Seul un des patients avait des rachialgies modérées. Deux patients ont eu une biopsie qui a mis en évidence une inflammation granulomateuse. Chez un patient, la lésion a régressé sous traitement de la GW. La taille des lésions n’a pas diminué chez les autres patients. Des calcifications intralésionnelles sont apparues dans deux cas. Aucun des patients n’a eu d’érosion osseuse locale, de complications vasculaires ou neurologiques. Ces lésions prévertébrales pourraient représenter une forme dorsale de fibrose rétropéritonéale au cours de la GW, mais avec une présentation et une évolution en général moins sévère. La GW doit être évoquée dans le diagnostic différentiel de médiastinite fibrosante (avec la tuberculose, les néoplasies, la sarcoïdose, l’histiocytose et les pseudotumeurs inflammatoires) qui peut avoir une présentation radiologique similaire. [ABSTRACT FROM AUTHOR]

Summary: Retroperitoneal fibrosis (RF) is a rare disease in children. We report the case of a 15-year-old boy who presented with a 2-month history of dorsal pain and a 2-week history of fever. The erythrocyte sedimentation rate and serum C-reactive protein value were high. Magnetic resonance imaging revealed a large heterogeneous retroperitoneal mass, suggestive of RF. Percutaneous biopsy of inguinal lymph node provided the diagnosis of anaplastic large cell lymphoma. This is the first report of RF revealing malignancy in childhood. It emphasizes that malignancy must be carefully searched for in children with unexplained RF. [Copyright &y& Elsevier]

Abstract: Background: Nowadays it is quite easy to diagnose idiopathic retroperitoneal fibrosis (IRF), particularly with the help of medical imaging. However there is no guideline about the treatment. Purpose: Looking for data about an evidence-based management. Methods: Screening of the database Medline. Titles and abstracts of articles published between 01/01/1985 and 31/12/2004 have been read to identify clinical trials and series about more than ten patients. Results: No record of any therapeutic trials has been found. Eight series in total, which included 177 patients, were identified. Two of the patients have been treated by an ureteral desobstruction only (endoscopy or nephrostomy), 45 by surgery (ureterolysis), 65 by corticotherapy and 64 both by surgery and steroids. For 38 patients, immunosuppressive drugs were combined with corticotherapy (azathioprine, cyclophosphamide or D-penicillamine). According to the authors, doses and duration of corticotherapy varied. Median follow-up lasted 56 months. The outcome is satisfactory in 73% for surgery alone, 86% for medical treatment alone and 73% for both. The association between steroids therapy and immunosuppressive drugs is efficient in 97% of the cases. No clear data about side effects was mentioned. Discussion: Treatment of the IRF is still empirical, based on surgery and corticotherapy. There is no guideline about the treatment strategy. Although tamoxifen has been proposed, efficacy evidence is lacking. Prospective multicenter studies will help us to progress in the management of the IRF. [Copyright &y& Elsevier]

Abstract: Occurrence of deep PETCO2 drop during surgical lumbar disk repair is rare but dramatic. This case report leads to the diagnosis of retroperitoneal vessels lesions. We review the different diagnosis related to the drop of the PETCO2 during surgery in the genupectoral position. We recommend that the diagnosis of retroperitoneal vessels lesion have to be suspected early if air embolism occurs during lumbar disk surgery. [Copyright &y& Elsevier]

Abstract: Ureteral obstruction due to idiopathic retroperitoneal fibrosis is a rare but severe clinical problem. The open approaches, as well as surgical techniques used to prevent stenosis recurrence, are described. Ureterolysis remains the procedure to relieve ureteral obstruction. The ureter is dissected and freed from the fibrotic process, and then separated to prevent the recurrence of the stenosis. Recently, the development of laparoscopic urology has allowed for minimal invasive treatment of many urological problems. We present our technique of ureterolysis for extrinsic ureteral obstruction. Advantages and complications of each method are considered and indications are proposed. [Copyright &y& Elsevier]

The case of a 52-year-old man with retroperitoneal fibrosis and ankylosing spondylitis is described. Inflammatory low back pain and acute renal insufficiency prompted a computed tomography scan of the abdomen with contrast agent injection. A fibrous sheath surrounding the aorta and attracting the ureters toward the midline was seen, strongly suggesting retroperitoneal fibrosis. The diagnosis of ankylosing spondylitis was based on the presence of inflammatory low back pain responsive to nonsteroidal anti-inflammatory drugs, syndesmophytes at the lumbar and cervical spine, bilateral sacroiliitis, and presence of the HLA-B27 antigen. Prednisone therapy in a daily dosage of 1 mg/kg induced a marked improvement. Only nine cases of concomitant retroperitoneal fibrosis and ankylosing spondylitis have been reported. These two conditions share similarities in some of the etiologic factors and anatomic localizations, suggesting that both may stem from a predisposition to fibrotic diseases. [ABSTRACT FROM AUTHOR]

Retroperitoneal fibrosis is an uncommon inflammatory condition in which the retroperitoneal tissue is transformed into a tight sheet of fibrous tissue. It can occur in association with various diseases, including rheumatic conditions. We report a new case in a 66-year-old man with spondyloarthropathy. Nine similar cases have been reported. Axial manifestations predominated, and half the patients carried the HLA-B27 antigen. The relation between retroperitoneal fibrosis and spondyloarthropathy remains controversial. [ABSTRACT FROM AUTHOR]

The authors report six cases of retroperitoneal fibrosis. The mean age is 32 years. Two patients were treated by corticosteroid and ureteric stents with a good outcome; they have normal kidney function. Four patients underwent ureterolysis by wrapping the ureters in omentum. The mean follow-up is 60 months. In the light of theses six cases and review of the literature, the clinical, diagnosis and therapeutic aspects are discussed. [Copyright &y& Elsevier]

Erdheim-Chester disease (EC) is a rare disease that is included in Group L in the 2016 revised classification of Langheransian histiocytoses. This disease may be clinically asymptomatic or manifest as a multi-systemic and life-threatening condition. All organs can be affected but typically there is bone involvement, retroperitoneal fibrosis, pituitary involvement, involvement of large vessels, lung, pleura or central nervous system. We are reporting a 70-year-old patient who, as of 2014, had a pelvic mass with retroperitoneal fibrosis and large vessel vasculitis without a definite diagnosis. Histological and molecular examination of the surgical specimen of the pelvic mass with the discovery of the BRAF V600E mutation provided new elements for the definitive diagnosis of Erdheim-Chester disease. We will describe the clinical, histological and molecular features to be known in EC disease., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

Abstract: Retroperitoneal fibrosis may reveal many diseases, including neoplasias. An 83-year-old man presented with an acute renal failure due to compressive retroperitoneal fibrosis. Clinically the only abnormal feature was a cutaneous subclavicular infiltrated lesion and laboratory features included hypereosinophilia, anemia and elevated acute phase reactants. A thoracic CT-scan showed pleuritis and para-oesophagal lymph node and the 18FDG-PET-scan an hypermetabolism lesion of the oesophagus. Gastroscopy and colonoscopy found a gastric linitis, already multimetastatic at diagnosis. The gastric linitis can present with many decepting clinical forms, increasing the risk of delayed diagnosis. [Copyright &y& Elsevier]

Résumé: La fibrose rétropéritonéale est une maladie rare, inflammatoire et fibrosante du tissu rétropéritonéal périaortique, associée à un engainement des uretères et des organes adjacents. Nous rapportons un nouveau cas de fibrose rétropéritonéale primitive chez un patient de 55 ans hospitalisé pour lombalgie inflammatoire. Avec un recul d''un an, la corticothérapie s''est associée à une très bonne réponse clinique, biologique et paraclinique. Cette observation nous permet de discuter de l''étiopathogénie et de la stratégie thérapeutique de la fibrose rétropéritonéale primitive. [Copyright &y& Elsevier]

The retroperitoneal fibrosis is a rare pathology, whose physiopathologic mechanism is poorly known. It characterizes by the training of a benign fibrous prevertebral plate. This plate is in rule limited between the renal pedicle in high and the promontory anointed down. However, extensions to the great peritoneal cavity, the mediastinum and especially the pelvis have been brought. Authors bring a case of retroperitoneal fibrosis at a woman of 54 years, that spreads to the pelvic region complicated of an obstructive renal insufficiency. The ureteral catheterism has allowed the standardization of the renal function. The uroCT-scan has allowed to make a complete lesion statement. The patient has been operated on with realization of ureterolysis and ureteral intraperitonization. Through this observation, therapeutic and diagnostic aspects will be discussed. [Copyright &y& Elsevier]

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., (Copyright © 2020. Published by Elsevier Masson SAS.)

Competing Interests: Les auteurs déclarent ne pas avoir de lien d’intérêts.