Novelties in the diagnosis and treatment of the most common systemic vasculitides in childhood

Authors

  • Marija Jelušić Klinika za pedijatriju, Medicinski fakultet Sveučilišta u Zagrebu, Klinički bolnički centar Zagreb, Zavod za imunologiju, reumatologiju, respiracijske i alergološke bolesti, Referentni centar za pedijatrijsku i adolescentnu reumatologiju Republike Hrvatske, Kišpatićeva 12, Zagreb
  • Mario Šestan Klinika za pedijatriju, Medicinski fakultet Sveučilišta u Zagrebu, Klinički bolnički centar Zagreb, Zavod za imunologiju, reumatologiju, respiracijske i alergološke bolesti, Referentni centar za pedijatrijsku i adolescentnu reumatologiju Republike Hrvatske, Kišpatićeva 12, Zagreb
  • Marijan Frković Klinika za pedijatriju, Medicinski fakultet Sveučilišta u Zagrebu, Klinički bolnički centar Zagreb, Zavod za imunologiju, reumatologiju, respiracijske i alergološke bolesti, Referentni centar za pedijatrijsku i adolescentnu reumatologiju Republike Hrvatske, Kišpatićeva 12, Zagreb

DOI:

https://doi.org/10.13112/pc.1075

Keywords:

SYSTEMIC VASCULITIS; CHILD; IgA VASCULITIS; MUCOCUTANEOUS LYMPH NODE SYNDROME; TAKAYASU ARTERITIS; ANTI-NEUTROPHIL CYTOPLASMIC ANTIBODY-ASSOCIATED VASCULITIS; POLYARTERITIS NODOSA

Abstract

Vasculitis is an inflammatory process that affects the blood vessel walls as the primary site of inflammation, which can lead to tissue damage due to vascular stenosis, occlusion, aneurysm, and/or rupture. The most common systemic vasculitis in children is IgA vasculitis (IgAV) and Kawasaki disease (KD). Gastrointestinal complications are the most common acute complications of IgAV, while kidney involvement (nephritis) is the most important chronic complication. The most important novelties in the field of IgAV relate to the follow-up of patients, whereby children at higher risk of developing nephritis are singled out. These are children with IgAV who have had severe gastrointestinal symptoms (severe abdominal pain, intussusception, hematochezia, and/or massive gastrointestinal bleeding), children with severe skin symptoms (ulcerations and necrosis, persistent purpura), male children and older children at the time of diagnosis of IgAV. Such children should be followed up for at least a year. According to the revised recommendations for the diagnosis of KD, it should be suspected in any child with unexplained fever lasting 4 or more days, with the presence of four or more criteria: bilateral conjunctival injection, changes in the oral cavity and lips, polymorphic rash, cervical lymphadenopathy, redness of the palms and soles, swelling of the hands and feet in the acute phase, and peeling of the skin around the nails in the later stages. According to the newer, revised recommendations, treatment of KD is not started equally in all patients, but those with a standard risk of developing coronary artery aneurysms should receive intravenous immunoglobulins and acetylsalicylic acid, and those with a high risk should receive additional anti-inflammatory therapy, i.e. systemic glucocorticoids or TNF-alpha blockers (infliximab or etanercept).

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Published

2025-04-02

How to Cite

Jelušić, M., Šestan, M., & Frković, M. (2025). Novelties in the diagnosis and treatment of the most common systemic vasculitides in childhood. Paediatria Croatica, 69(Suppl 2), 214-227. https://doi.org/10.13112/pc.1075

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