Juvenile Systemic Connective Tissue Diseases

Juvenile Systemic Connective Tissue Diseases

What are the juvenile systemic connective tissue diseases?

SLE, juvenile dermatomyositis (JDM), overlap syndromes including mixed connective tissue disease (MCTD), SSc (scleroderma), vasculitis, juvenile Sjögren syndrome.

1. Childhood-onset systemic lupus erythematosus (SLE) is more severe, has more nephritis, and has a worse prognosis than adult-onset SLE.

2. Inflammatory myositis in childhood is almost always dermatomyositis and not polymyositis.

3. Henoch–Schonlein purpura (HSP) is an immunoglobulin A (IgA)-mediated process and the most common small-vessel vasculitis in childhood.

4. Localized scleroderma is the predominant childhood form of scleroderma (not systemic sclerosis [SSc]) and may respond to early aggressive immunosuppressive therapy.


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