Different pathophysiological conditions, clinical picture and therapeutic methods of juvenile systemic lupus erythematosus
Journal Title: Postępy Nauk Medycznych - Year 2013, Vol 26, Issue 5
Abstract
Systemic lupus erythematosus is a multisystem inflammatory disease of autoimmune aetiology, the development of which involves genetic, hormonal and environmental factors. Approximately 20% of lupus cases have their onset during childhood or adolescence due to immune system immaturity and sex hormone synthesis initiation, both of which promote the development of the disease. Juvenile systemic lupus erythematosus differs from that of adult-onset.The disease is characterised by a very high clinical and serological activity, rapid development of organ complications as well as the necessity of an aggressive immunosuppressive therapy. The most common symptoms include: nephropathy, involvement of the central nervous system and the hematopoietic system. In 90% of cases, nephropathy develops during the first two years of the disease with its course and its pathomorphological etiology being the major determinants of treatment and prognosis. It is recommended that early renal biopsy is performed and repeated after 6 months. Young people usually develop severe proliferative forms of glomerulopathy (WHO class III or IV), which lead to an end-stage renal failure in 25% of cases, even though treated. Poor prognostic indicators include as follows: the presence of antiphospholipid antibodies, low complement C3 and C4 levels, high titer of anti-dsDNA antibodies, which requires intensive immunosuppressive therapy. Induction therapy involves an intravenous administration of methylprednisolone, cyclophosphamide or mycophenolate mofetil, cyclosporin A. MMF is recommended for maintaining remission. Rituximab has been described to have positive effects in treatment-resistant cases. The treatment of lupus entails a number of complications affecting the processes of growth, maturation, systemic metabolism as well as the mental state of a young patient. Furthermore, so far unsatisfactory treatment results are to a large extent the outcome of cumulative adverse effects of the therapy.When referring an adolescent patient to an internist (rheumatologist, nephrologist), the specialist’s knowledge on the dissimilarities associated with the paediatric lupus as well as familiarity with patient’s special needs ought to be considered.
Authors and Affiliations
Lidia Hyla-Klekot, Grażyna Kucharska, Karina Słonka, Katarzyna Karwicka
Keywords
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