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.
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http://dx.doi.org/10.11604/pamj.2017.28.194.10092 | DOI Listing |
Dis Mon
January 2025
Department of Pharmaceutical Sciences, Babasaheb Bhimrao Ambedkar University, Vidya Vihar, Raebareli Road, Lucknow-226025, (U.P.), India. Electronic address:
Erdheim-Chester disease (ECD) is an extremely rare non-Langerhans cell disorder that is believed to include both inflammatory and neoplastic characteristics. It is caused due to genetic mutations in proto-oncogenes like BRAF and MEK, while immunological pathways have an essential role in the onset and progression of the disease. Despite its rarity, ECD poses significant diagnostic and therapeutic challenges due to its heterogeneous clinical presentation and limited understanding of its underlying pathophysiology.
View Article and Find Full Text PDFClin Rheumatol
January 2025
Department of Rheumatology and Immunology, The First Affiliated Hospital of Naval Medical University, Shanghai, 200433, China.
Objective: Retroperitoneal fibrosis (RPF) is a rare condition marked by inflammation and fibrosis affecting the peritoneal and retroperitoneal soft tissues. In recent years, the identification of IgG4-related diseases has brought to light a significant association with fibrous disorders, including RPF, which were once considered independent. In this comprehensive cohort study, we performed a comparative analysis of the demographic, clinical, laboratory, histopathological, and therapeutic characteristics between patients with IgG4-related RPF and those with idiopathic retroperitoneal fibrosis (iRPF).
View Article and Find Full Text PDFCureus
November 2024
Department of Internal Medicine/Rheumatology, Texas Tech University Health Sciences Center Paul L. Foster School of Medicine, El Paso, USA.
Retroperitoneal fibrosis (RPF) is a rare disease with a nonspecific presentation. RPF can be classified into Idiopathic, the most common, or secondary due to malignancy and various medications resulting in chronic inflammation and fibrosis in the retroperitoneum. The complications arise due to the compression of structures in the retroperitoneum.
View Article and Find Full Text PDFFront Med (Lausanne)
December 2024
Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing, China.
Objective: Retroperitoneal fibrosis (RPF) is a rare disease characterized by the presence of fibroinflammatory tissue that surrounds the abdominal aorta and the iliac arteries and often entraps the ureters. Hydronephrosis is a common complication of RPF, however, its clinical features and outcomes have not been well elucidated.
Methods: A total of 115 RPF-related hydronephrosis patients have been recruited from 9 clinical centers in China since March 2010.
Rheumatology (Oxford)
December 2024
Department of Rheumatology and Clinical Immunology, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), The Ministry of Education Key Laboratory, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.
Objectives: Idiopathic retroperitoneal fibrosis (IRF) is a rare autoimmune-mediated condition characterized by fibro-inflammatory tissue development around the abdominal aorta and iliac arteries. Ureteral entrapment and hydronephrosis are the most common manifestations and acute renal failure or chronic renal insufficiency may occur as the consequence. Glucocorticoids and immunosuppressants may be effective but the therapeutic response and outcome are heterogeneous and hard to predict.
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