The 21st International Vasculitis Workshop, held in Barcelona, Spain, from April 7 to 10, 2024, highlighted advances in pediatric vasculitis, focusing on a holistic, multidisciplinary approach. Common childhood vasculitides, including IgA Vasculitis (IgAV) and Kawasaki Disease (KD), were discussed. The Ankara 2008 criteria for IgAV, endorsed by EULAR and PReS, were evaluated for their performance in adults, showing high sensitivity but necessitating further refinement for improved specificity. Studies on genetic associations, such as Human Leukocyte Antigen (HLA) polymorphisms in IgAV, and biomarkers like S100A8/A9, HMGB1, and RAGE, were presented. Kawasaki disease research included novel anti-apolipoprotein A-2 antibodies, showing promise in reducing coronary arteritis. Monogenic vasculitides, such as deficiency of ADA2, were addressed with new consensus-driven recommendations. The workshop underscored the importance of continued research and tailored therapeutic strategies to improve outcomes in pediatric vasculitis, paving the way for advancements in diagnosis, management, and understanding of these complex diseases.
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http://dx.doi.org/10.1093/rheumatology/keae406 | DOI Listing |
Rheumatology (Oxford)
March 2025
Division of Rheumatology, Department of Internal Medicine, Hacettepe University Faculty of Medicine, Altindag, Ankara, Türkiye.
Polyarteritis Nodosa (PAN), is the firstly described vasculitis and can be seen in paediatric and adult age. PAN has a heterozygous clinical picture including cutaneous, constitutional, musculoskeletal, gastrointestinal, and renal involvement. Description and splitting of other vasculitis, makes this medium vessel vasculitis, a very rare disease.
View Article and Find Full Text PDFRheumatology (Oxford)
March 2025
Pediatric Rheumatology Department, Hospital Sant Joan de Déu, Barcelona and Institut de Recerca Sant Joan de Déu, Barcelona, and Universitat de Barcelona, Barcelona, Spain.
Kawasaki disease (kDa) has remained a medical mystery for the last five decades with a wide array of hypothesis about potential aetiological factors, that have never been confirmed. In this brief note, I revised the state-of-the-art for the so-called 'wind hypothesis', claiming that the nature and types of aerosols, particularly fine ones, can account for a central part of this research avenue and the relation to kDa. Characterizing their chemical nature, in particular of the composition in trace elements, as well as their biological components (bacteria, fungi and viruses) stands up today as the most promising avenue towards constraining the range of environmental factors modulating or being responsible for this long-debated disease.
View Article and Find Full Text PDFRheumatology (Oxford)
March 2025
Department of Paediatric Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
The 21st International Vasculitis Workshop, held in Barcelona, Spain, from April 7 to 10, 2024, highlighted advances in pediatric vasculitis, focusing on a holistic, multidisciplinary approach. Common childhood vasculitides, including IgA Vasculitis (IgAV) and Kawasaki Disease (KD), were discussed. The Ankara 2008 criteria for IgAV, endorsed by EULAR and PReS, were evaluated for their performance in adults, showing high sensitivity but necessitating further refinement for improved specificity.
View Article and Find Full Text PDFPediatr Rheumatol Online J
March 2025
Department of Pediatric Nephrology, Gazi University Faculty of Medicine, Ankara, Turkey.
Background: Cyclophosphamide (CYC) is an inactive alkylating agent that transforms the alkyl radicals into other molecules and is used in combination with systemic corticosteroids in the treatment of many childhood rheumatic diseases, such as systemic lupus erythematosus (SLE), and ANCA-associated vasculitis (AAV). In recent years, rituximab (RTX), a B-cell-targeting anti-CD20 monoclonal antibody, has emerged as a new alternative treatment modality over CYC for induction therapy of childhood-onset rheumatic diseases. Clinicians adopt different practices for using CYC particularly in relation to indications, posology, pre-treatment laboratory work-up, post-treatment follow-up, and screening pre- and post-treatment vaccination status.
View Article and Find Full Text PDFCureus
February 2025
Pediatrics, Valley Children's Healthcare, Madera, USA.
Anti-glomerular basement membrane (anti-GBM) disease is an extremely rare small vessel vasculitis, which typically presents as rapidly progressive glomerulonephritis with or without pulmonary hemorrhage. Atypical anti-GBM disease varies in its clinical and laboratory presentation with insidious onset of symptoms. We present the case of a 16-year-old male child who presented with a two-week history of fever, weight loss, cough, hemoptysis, shortness of breath, and a five-year history of intermittent emesis.
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