Renal amyloidosis (RA) is known to be one of the many complications of Behcet's disease (BD) but its frequency has been often underestimated. In this paper, we report on three cases of RA in a series of 105 patients with BD. RA was clinically suspected in our patients by the presence of edema and/or hypotension. The nephrotic syndrome was present in all of them and one patient had associated renal failure. Renal biopsy showed amyloid deposits type AA in all cases.Our patients received Colchicine at doses of 1 mg per day but with disappointing results. Their prognosis was significantely impaired with our three patients dying at ten years, six months and four weeks after diagnosis of RA. We will try, through this clinical experience and based on the relevant literature to better understand this kind of morbid association.
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J Plast Reconstr Aesthet Surg
November 2024
Bagcilar Training and Research Hospital, Department of Plastic, Reconstructive and Aesthetic Surgery, Istanbul, Turkey.
Objective: Autoimmune diseases are systemic conditions that can have negative effects on wound healing. The objective of the present study was to investigate the efficacy of combining bone marrow-derived mesenchymal stem cells (BM-MSCs), acellular dermal matrix (ADM), split-thickness skin graft (STSG), and negative-pressure wound therapy (NPWT) for treating patients with autoimmune diseases and chronic non-healing wounds.
Methods: Thirty-four patients with autoimmune diseases and non-healing chronic wounds of the lower extremities between 2012 and 2023 were included in the study.
Eur J Pediatr
December 2024
Department of Medical Genetics, Dr. Behçet Uz Children's Hospital, Izmir, Turkey.
Unlabelled: The RASopathies are a group of disorders resulting from a germline variant in the genes encoding the Ras/mitogen-activated protein kinase pathway. These disorders include Noonan syndrome (NS), cardiofaciocutaneous syndrome (CFC), Costello syndrome (CS), Legius syndrome (LS), and neurofibromatosis type 1 (NF1), and have overlapping clinical features due to RAS/MAPK dysfunction. In this study, we aimed to describe the clinical and molecular features of patients exhibiting phenotypic manifestations consistent with RASopathies.
View Article and Find Full Text PDFImmun Inflamm Dis
December 2024
Department of Rheumatology and Immunology, Huadong Hospital Affiliated to Fudan University, Shanghai, China.
Objective: This study aimed to investigate the efficacy and safety of a triple therapy consisting of colchicine, thalidomide and total glucosides of paeony (TGP) in Behcet's disease (BD) patients with mucocutaneous involvement.
Methods: Totally 355 newly diagnosed BD patients with mucocutaneous involvement were recruited, who received dexamethasone and colchicine for the first 2 weeks, then they were categorized into "sustained triple-therapy (ST)" (n = 231) and "colchicine to triple-therapy (CT)" (n = 124) groups respectively: for ST group, patients received colchicine, thalidomide plus TGP from Month (M)0.5 to M12; for CT group, patients received colchicine from M0.
Radiol Case Rep
February 2025
Department of Internal Medicine, An Najah National University Hospital, Nablus, Palestine.
Behçet's Disease (BD) is a multisystem inflammatory disorder that can lead to severe vascular complications, including Budd-Chiari Syndrome (BCS), a rare but life-threatening condition characterized by hepatic vein obstruction. The co-occurrence of BD and inherited thrombophilia, such as Factor V Leiden mutation, significantly increases the risk of thrombosis, complicating the clinical management of affected individuals. In this case, a 16-year-old female initially presented with nonspecific symptoms of generalized fatigue and bone pain, which later progressed to abdominal distension and significant hepatosplenomegaly.
View Article and Find Full Text PDFFront Med (Lausanne)
December 2024
Department of Oral Medicine, Royal London Hospital, Barts Health NHS Trust, London, United Kingdom.
Introduction: Crohn's disease (CD) is a chronic inflammatory bowel disease which can affect any area of the gastrointestinal tract, including oral tissues. The complex nature of this disease demands interdisciplinary management, especially when both intestinal and oral manifestations are present.
Case: This report presents the case of a 28-year-old male patient with oral, ileo-caecal and peri-anal CD managed jointly between Gastroenterology and Oral Medicine.
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