Therapy of vasculitis syndromes varies considerably according to different forms and gravity. Mild cases often heal spontaneously. In other cases, when the causal factors are known, the removal of the offending antigen or the treatment of an underlying primary disease are sufficient to obtain remission. In the vasculitis syndromes, in which the causal factors are unknown, therapy is based on various associations of nonsteroidal antiinflammatory drugs, antiplatelet drugs, anticoagulants, corticosteroids and cytotoxic drugs, depending on the type, extention and gravity of disease. In most severe systemic necrotizing vasculitis, therapy with corticosteroids and cytotoxic drugs must be protracted for months, with inevitable side-effects, particularly severe in children. Pulse therapy with intravenous bolus of high doses of methylprednisolone seems to offer a new valid therapeutic approach, but is still under investigation. Plasmapheresis may be successful in acute and grave forms. High dose intravenous gammaglobulin therapy is indicated in the prevention of coronary involvement of Kawasaki disease. In this review these problems and therapeutic regimes suggested by different Authors are discussed, with particular attention to their application in childhood.
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Pediatr Blood Cancer
January 2025
Pediatrics Department, Jean Verdier Hospital, APHP, Bondy, France.
Background: Systemic inflammatory diseases (SIDs) have been reported in patients with sickle cell disease (SCD), but clinical data in children are scarce.
Objectives: To identify clinical and laboratory features at diagnosis of SID in children with SCD and to describe their evolution.
Methods: Data from children with SCD and SIDs were retrospectively collected in a French multicenter study from 1991 to 2018.
Cureus
December 2024
Medicine, Faculdade de Medicina da Universidade do Porto (FMUP), Porto, PRT.
Anti-glomerular basement membrane disease is a rare small vessel vasculitis caused by the deposition of immunoglobulin G (IgG) autoantibodies in the basement membrane of glomerular capillaries and lung alveoli, leading to rapidly progressive renal failure and/or alveolar hemorrhage. We report the case of an 83-year-old female patient presenting with uremic symptoms, rapidly progressive kidney failure, and a high titer of anti-glomerular basement membrane antibodies. Given the urgent need for kidney replacement therapy, the substantial fibrosis and glomerular scarring observed in the kidney biopsy suggesting a chronic process, and the absence of pulmonary involvement, neither immunosuppressive treatment nor plasmapheresis was initiated, since a low likelihood of a favorable response to these interventions was expected.
View Article and Find Full Text PDFPharmaceuticals (Basel)
January 2025
Department of Paediatrics, "Carol Davila" University of Medicine and Pharmacy, 050474 Bucharest, Romania.
The introduction of anti-tumor necrosis factor-α (anti-TNF-α) agents, particularly infliximab (IFX) and adalimumab (ADA), has significantly expanded the therapeutic arsenal for inflammatory bowel disease (IBD). While these biologics have demonstrated substantial efficacy, they are associated with a spectrum of potential adverse events (AEs). This study aims to evaluate and document these AEs to facilitate optimal patient selection and monitoring strategies of patients undergoing these therapies.
View Article and Find Full Text PDFJ Clin Med
January 2025
Department of Pulmonary Medicine, European Hospital Georges Pompidou, 75015 Paris, France.
: Cryoglobulinemia (CG) syndrome is a heterogeneous condition characterized by the presence of cryoglobulins in serum, often leading to vasculitis with protean clinical manifestations. Understanding the presentation of cryoglobulinemia-related symptoms based on cryoprecipitate levels, GC type, and severity at diagnosis is essential for effective management. Hence, this study aimed to provide a comprehensive analysis of patients with positive cryoglobulin detection to investigate these aspects.
View Article and Find Full Text PDFInt J Mol Sci
January 2025
Department of Dermatology, Sexually Transmitted Diseases and Clinical Immunology, Collegium Medicum, University of Warmia and Mazury, Al. Wojska Polskiego 30, 10-229 Olsztyn, Poland.
Vascular cell adhesion molecule-1 (VCAM-1) and E-selectin are involved in different inflammatory diseases and may be potential cardiovascular risk biomarkers in psoriasis. They play an important role in regulating the recruitment and adhesion to endothelial cells during inflammation, affecting various conditions like vasculitis, atherosclerosis, and cardiovascular diseases. Positive outcomes have been observed when using Tumor Necrosis Factor Alpha (TNF-α) inhibitors and biological therapies that target selectins to control the functioning of endothelial cells and reduce inflammation in psoriasis and related conditions.
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