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Polyarteritis nodosa in children


Médecine thérapeutique / Pédiatrie. Volume 14, Number 5-6, 362-70, Septembre-Décembre 2011, Dossier

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Author(s) : Richard Mouy

Summary : Childhood polyarteritis nodosa (cPAN) is a very rare disease. The two main forms are the systemic (60%) and the cutaneous form (30%), which is usually less severe. Some symptoms are common to both types: spiking fever, myalgia, arthralgia, abdominal pain, various dermatological symptoms (subcutaneous nodulus, soft tissue swelling, skin rash) associated with a biological inflammation. Other symptoms may be encountered in the systemic cPAN: renal disease (glomerulonephritis of bad prognosis), intestinal vascularitis, cardiac disease, neurological abnormalities (mono- or multinevrititis, seizure). The diagnosis is based upon histopathological and/or radiological data. The histological findings show a necrotizing panarteritis of the wall of small and medium-size vessels. Aneuvrysms of renal, hematic or mesenteric arteries may be displayed by vascular Doppler or angiography. The treatment of the systemic cPAN is based upon oral and/or parenteral steroids with immunosuppressive drugs such as azathioprine and cyclophosphamide. The cutaneous form can be treated with non-steroidal anti-inflammatory drugs, but oral corticosteroids are frequently required. Intravenous immunoglobulins seem to be efficient, as is long-term prophylactic penicillinotherapy when streptococcus is involved.

Keywords : vasculitides, necrotizing vasculitis, streptococcus pyogenes, cutaneous periarteritis nodosa, intravenous immunoglobulin

 

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