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Rationale: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that affects multiple organ systems. Retroperitoneal fibrosis (RPF) is a rare condition characterized by the development of fibrous tissue in the retroperitoneal space. The coexistence of SLE and RPF is extremely uncommon, and this report aims to enhance understanding of this complex relationship.
Patient Concerns: A 32-year-old woman presented with sudden-onset syncope. Her medical history revealed a 5-year history of SLE, and imaging studies identified a retroperitoneal mass.
Diagnoses: A comprehensive diagnostic workup, including magnetic resonance imaging (MRI) and biopsy, confirmed retroperitoneal fibrosis secondary to SLE.
Interventions: The patient was treated with high-dose corticosteroids, immunosuppressive therapy, and the biologic agent rituximab.
Outcomes: The patient's symptoms markedly improved, and follow-up MRI demonstrated significant regression of the retroperitoneal lesion.
Lessons: RPF associated with SLE is exceptionally rare. This case underscores the importance of early diagnosis and a coordinated multidisciplinary approach in managing such complex conditions. Glucocorticoid therapy remains the cornerstone of treatment, augmented by immunosuppressants and biologic agents when necessary.
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http://dx.doi.org/10.1097/MD.0000000000041208 | DOI Listing |
Clin Nucl Med
September 2025
Hepatology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Hepatocellular carcinoma is one of the leading causes of cancer-related death worldwide. Immune checkpoint inhibitors (ICI) have improved progression and overall survival in patients progressing on sorafenib therapy. But activation of the immune system can lead to numerous immune-related adverse events.
View Article and Find Full Text PDFEur J Intern Med
September 2025
Nantes Université, CHU Nantes Department of Internal and Vascular Medicine, CNRS UMR 6291, INSERM UMR 1087, l'institut du thorax Team III Vascular and Pulmonary Diseases, F-44000 Nantes, France. Electronic address:
BMJ Case Rep
September 2025
Department of Internal Medicine, Hospital General Universitario Gregorio Marañón, Madrid, Community of Madrid, Spain.
Uveitis is a significant cause of visual impairment and is often linked to underlying systemic immune-mediated conditions. Retroperitoneal fibrosis, characterised by the proliferation of fibrotic tissue in the retroperitoneum, can similarly be associated with autoimmune or autoinflammatory diseases. While both conditions have been reported in the context of systemic disorders, their co-occurrence in the absence of a defined systemic diagnosis is exceedingly rare, with only two such cases previously documented.
View Article and Find Full Text PDFRadiol Case Rep
November 2025
Loma Linda University Medical Center, 11234 Anderson St., Loma Linda, CA 92354, USA.
We present a case of a 59-year-old female with headaches and memory loss. Her history includes surgeries for hydronephrosis and a mediastinal mass initially diagnosed as retroperitoneal fibrosis. Imaging performed a few years later revealed multiple bilateral supratentorial masses.
View Article and Find Full Text PDFDiagnostics (Basel)
August 2025
Center for Clinical and Translational Science, Beijing Tsinghua Changgung Hospital, School of Clinical Medicine, Tsinghua Medicine, Tsinghua University, Beijing 102218, China.
: Retroperitoneal fibrosis (RPF), a rare fibroinflammatory disorder, is classified into idiopathic (iRPF) and secondary (sRPF) forms, with the latter posing significant diagnostic challenges in routine clinical pathway due to atypical presentations, especially in malignancy-associated (maRPF) cases. : Here, we report a 38-year-old female with congenital pancreatic hypoplasia presenting with elusive hypometabolic retroperitoneal masses, initially suggestive of iRPF. Persistent CA19-9 elevation prompted histopathological evaluation, revealing poorly differentiated adenocarcinoma of indeterminate origin.
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