Vascular and connective tissue diseases in the paediatric world.

Despite the fact that in recent decades many advances have been made in classification, outcomes measurements and therapeutic regimens, connective tissue diseases remain a challenge for paediatric rheumatologists. Corticosteroids are still the first choice therapy in juvenile systemic lupus erythematosus (SLE); however, it is time for a new therapeutic approach with immunosuppressant drugs in order to obtain a more prolonged survival and reduced organ system damage. A prevention of relapse of thromboembolic phenomena using low-dose aspirin is strongly suggested in children with anticardiolipin antibodies, particularly in those with SLE. The long-term prognosis in juvenile dermatomyositis is significantly improved due to a more aggressive treatment with corticosteroids and methotrexate. The treatment of Henoch-Schöenlein purpura is still under debate, and no agreement has been reached regarding the role of corticosteroids in the prevention of renal involvement. The management of the acute phase of Kawasaki disease is now well established, although there is controversy regarding the role of corticosteroids. It is still under debate how and whether to treat patients who do not fulfil the diagnostic criteria. Polyarteritis nodosa is a disease at risk of death and requires an aggressive approach with corticosteroids and immunosuppressants, while cutaneous polyarteritis needs a close control of an underlying streptococcus infection and prophylaxis with benzatin-penicillin to prevent relapses.