Pediatr. praxi. 2008;9(4):216-221
Systemic connective tissue diseases in childhood
- Klinika dětského a dorostového lékařství 1. LF UK a VFN, Praha
Systemic connective tissue diseases are characterised by chronic inflammation and variable clinical manifestations. Despite their rarity in childhood their health impact may be serious. Timely diagnosis and early appropriate therapy are important favourable prognostic factors. Systemic lupus erythematosus (SLE) and inflammatory myopathies, namely juvenile dermatomyositis, are main representatives. SLE is a typical autoimmune multisystem disease where glomerulonephritis and neuropsychiatric lupus belong to the most serious manifestations. At disease onset fevers, fatigue, photosensitive rash and arthritis together with high sedimentation rate and cytopenia are most common. Proximal skeletal muscles and skin are the main target for chronic inflammation and often prominent vasculopathy in juvenile dermatomyositis. Progression of muscle weakness may cause respiratory insuficiency. Therapy principles are further discussed.
Keywords: Key words: systemic lupus erythematosus, juvenile dermatomyositis, clinical manifestations, therapy.
Published: November 1, 2008 Show citation
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