Childhood primary large vessel CNS vasculitis: single-centre experience and review of the literature

Publikation: Beitrag in FachzeitschriftÜbersichtsartikel (Review)BeigetragenBegutachtung

Abstract

Ischaemic brain injuries are rare conditions in the paediatric age group. Main causes include non-arteriosclerotic arteriopathies, which in childhood usually result from primary vasculitis of large or small vessels and lead to impaired perfusion and subsequent ischaemic brain lesions. In accordance with the nomenclature of systemic forms, CNS vasculitis is subdivided into groups, based on the size of affected vessels: angiography-positive primary angiitis of medium-sized and large vessels (pPACNS), and angiography-negative angiitis of small vessels (svPACNS). We report the clinical presentation, diagnostic approach, and therapy of four children with progressive pPACNS. Patients were treated with high-dose corticosteroids and anticoagulation with unfractionated heparin in the acute phase, followed by immune modulatory treatment with mycophenolate mofetil (MMF) and dual antiplatelet therapy with acetylsalicylic acid and clopidogrel. In this manuscript, we illustrate the experience gained in our hospital, resulting in significantly faster diagnosis and treatment initiation, and discuss the applied immune modulating treatment regimen in the context of the literature. Based on our observations, we conclude that immune modulating therapy with initial high-dose corticosteroids, followed by steroid-sparing maintenance treatment with MMF, may be safe and effective in childhood progressive pPACNS.

Details

OriginalspracheEnglisch
Seiten (von - bis)213-220
Seitenumfang8
FachzeitschriftClinical and Experimental Rheumatology
Jahrgang35
Ausgabenummer1, Suppl 103
PublikationsstatusVeröffentlicht - 2017
Peer-Review-StatusJa

Externe IDs

Scopus 85028512073
PubMed 28375836
researchoutputwizard legacy.publication#79499
researchoutputwizard legacy.publication#78475
ORCID /0000-0002-3666-7128/work/147143654

Schlagworte

Schlagwörter

  • Adrenal Cortex Hormones/administration & dosage, Age of Onset, Anticoagulants/administration & dosage, Aspirin/administration & dosage, Brain Ischemia/diagnostic imaging, Cerebral Angiography/methods, Child, Child, Preschool, Clopidogrel, Diffusion Magnetic Resonance Imaging, Drug Therapy, Combination, Female, Germany, Heparin/administration & dosage, Humans, Immunosuppressive Agents/administration & dosage, Magnetic Resonance Angiography, Male, Mycophenolic Acid/administration & dosage, Platelet Aggregation Inhibitors/administration & dosage, Ticlopidine/administration & dosage, Time Factors, Treatment Outcome, Vasculitis, Central Nervous System/complications