Background Juvenile dermatomyozitis (JDM) with vasculopathy is rare but the most severe form of JDM. Vasculopathy so far signified a poor prognosis with relapsing and chronic course and high risk of developing calcinosis, in other cases rapidly led to death.
Objectives To analyze characteristics of the presenting and cumulative clinical features, laboratory features, treatement modalities, disease activity and oucome of children with JDM with vasculopathy.
Methods All JDM patients and among them JDM patients with vasulopathy hospitalized during the period of 1991 – 2011 in Departement of Paediatric Rheumatology and Immunology, Zagreb University, School of Medicine, University Hospital Centre Zagreb, Croatia, were enrolled into the study.
Results There were 31 children with JDM hospitalised in our Departement: 15 boys (48.4%) and 16 girls (51.6%) with mean age at disease onset 12.1 years. Seven hospitalised children (26.9%) had severe form of JDM with vasculopathy: two with acute gastrointestinal perforation and bleeding, one with urinary tract involmenet and chronical hematuria and four with multysistem involvement – brain oedema with hallucinations, respiratory distress syndome, myocarditis, gastrointesinal bleeding, one of them also with retinal cotton-wool spots and papillar oedema.
In all JDM cases therapy included corticosteroids, in most cases combined with methotrexate and IV imunoglobulines, in severe vasculopathy cases also combined with cyclophosphamide and plasmaphereis.
In last case, 8 years old girl hospitalized in our Departement in september 2011. with rapid progression of respiratory insufficiency within severe acute multysistem involvement including retinal cotton-wool spots and papillar oedema, with poor response to multiple drug therapy combined with plasmaphereis, on 24th day of treatement we applyed first dose of anti-TNF therapy (infliximab).
Among all JDM patients with vasculopathy two patients had rapid progression with gastrointestinal perforations, profuse bleeding and lethal outcome, four patients has chronic course with calcinosis. In patient with severe acute multysistem involvement including retinal cotton-wool spots and papillar oedema, applyed anti-TNF therapy resulted in rapid complete resolution of eye changes and excellent, fast general recovery.
Conclusions In our opinion, an early apply of anti-TNF therapy in JDM patient with severe acute multysistem vasculopathy results in excellent outcome so further studies of anti-TNF therapy in these cases are reasonable.
Disclosure of Interest None Declared
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