Background: Behcet's disease (BD) is an inflammatory disorder of unknown cause that is characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. Local vasculitis can cause damage to the visceral system, but it is rare in kidney patients, especially those with IgA nephropathy (IgAN). In China, a small number of related cases have been reported. Here we present a case of co-occurrence of BD and IgAN.
Case Presentation: An 18-year-old female who presented with a history of recurrent oral ulcers was found ten years ago. Four years later, the patient presented with reddish nodules on the skin of both lower limbs and then presented with vulvar ulcers. This patient was clinically diagnosed with Behcet's disease after left calf skin biopsy and presented severe proteinuria and hematuria during this period. IgAN was diagnosed after percutaneous renal biopsy. The patient was treated with hormonal, anti-inflammatory, immunomodulatory, kidney protective, and protein-lowering urine agents. After 3 years of follow-up, the patient reappears oral ulcers, reddish nodules on the skin of both lower limbs and renal dysfunction.
Conclusions: BD is less common in China and is clinically prone to missed diagnosis and misdiagnosis. BD with IgAN is rarer. We should regularly pay attention to the routine urine and renal function of BD patients for early detection and treatment and to prevent further progression of the disease.
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http://dx.doi.org/10.1186/s12882-024-03748-y | DOI Listing |
Korean J Intern Med
January 2025
Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
Background/aims: This study applied the 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology (ACR/EULAR) criteria for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) to patients with Behçet's disease (BD) to investigate the proportion and clinical implications of the reclassification to the overlap syndrome of BD and AAV (OS-BD-AAV).
Methods: We included 280 BD patients presenting with ANCA positivity but without medical conditions mimicking AAV at diagnosis. Demographic data, items from the 2014 revised International Criteria for BD and 2022 American College of Rheumatology and European Alliance of Associations for Rheumatology criteria for AAV, ANCA positivity, and laboratory results were recorded as clinical data at diagnosis.
Mol Biol Rep
January 2025
Connective Tissue Diseases Research Center, Tabriz University of Medical Sciences, Tabriz, Iran.
Background: Recent studies have highlighted the potential role of several long non-coding RNAs (lncRNAs) in the pathogenesis of Behçet's disease (BD). This study investigated the expression profiles of lncRNA NEAT1 and lncRNA HOTAIR, and their target cytokine genes, IL-6 and TNF-α, in active and inactive BD patients.
Methods: This cross-sectional study was conducted on peripheral blood mononuclear cells (PBMCs) obtained from 25 BD patients and 25 age-sex-matched healthy controls (HCs).
Clin Exp Immunol
January 2025
Department of Clinical Laboratory, State key Laboratory of Complex, Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China.
Neuro-Behçet's disease (NBD) is a more severe but rare symptom of Behçet's disease (BD), which is mainly divided into parenchymal NBD (p-NBD) involving brain stem, spinal cord, and cerebral cortex. Non-p-NBD manifests as intracranial aneurysm, cerebral venous thrombosis, peripheral nervous system injuries, and mixed parenchymal and non-parenchymal disease. P-NBD is pathologically characterized by perivasculitis presenting with cerebrospinal fluid (CSF) pleocytosis, elevated total protein, and central nervous system (CNS) infiltration of macrophages and neutrophils, which are subdivided into acute and chronic progressive stages according to relapsing-remitting courses and responses to steroids.
View Article and Find Full Text PDFJ Clin Med
December 2024
Department of Internal Medicine and Clinical Immunology, University Hospital of Rennes, 35000 Rennes, France.
Current guidelines recommend systematic screening for rheumatic diseases (RDs), including antiphospholipid syndrome (APS), in patients with recurrent pregnancy loss (RPL). However, these recommendations are based on limited evidence, as data on the prevalence of RD in this specific population remain scarce. In particular, the impact of the recent update to the ACR/EULAR classification criteria for APS on the prevalence of RD among RPL patients has yet to be clarified.
View Article and Find Full Text PDFChildren (Basel)
December 2024
Department of Pediatrics, Division of Pediatric Gastroenterology, University of Health Sciences Dr. Behçet Uz Children's Hospital, Izmir 35210, Turkey.
Background: Cholelithiasis is a rare disease in infants, and there is limited data on its risk factors and management.
Objectives: To evaluate the risk factors, management, and response to medical treatment of cholelithiasis in infants.
Methods: Infants diagnosed with cholelithiasis by ultrasound between 2018 and 2023 were retrospectively analyzed.
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