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.