Etiology, pathogenesis, and clinical presentation of Acute Rheumatic Fever in children
Pathogenesis of Acute Rheumatic Fever: Immune response and molecular mimicry
Acute Rheumatic Fever is an autoimmune reaction to Streptococcus infection that affects multiple organ systems in children. Understanding its pathogenesis and timely management is crucial to preventing long-term complications.
Etiology, Pathogenesis, and Clinical Presentation of Acute Rheumatic Fever in Children
Introduction
Acute Rheumatic Fever (ARF) is an autoimmune inflammatory disease triggered by a preceding Group A Streptococcus (GAS) infection. It primarily affects children and can lead to significant cardiac complications.
Etiology
ARF occurs due to an immune system response to a streptococcal throat infection. The body's antibodies mistakenly attack its own tissues, particularly the heart, joints, skin, and nervous system.
Pathogenesis: Immune Response and Molecular Mimicry
The pathogenesis of ARF revolves around molecular mimicry. Streptococcal M protein shares structural similarities with human tissue proteins, leading the immune system to mistakenly target self-antigens. This results in inflammatory damage.
Key Steps in Pathogenesis:
- Streptococcal infection triggers the immune system.
- Cross-reactive antibodies attack host tissues.
- Inflammatory cytokines are released, exacerbating tissue damage.
- Long-term cardiac complications such as rheumatic heart disease may develop.
Clinical Presentation
Children with ARF present with a range of symptoms affecting multiple systems:
Major Clinical Features:
- Carditis: Inflammation of the heart valves leading to murmurs and heart failure.
- Polyarthritis: Migratory joint inflammation, affecting large joints.
- Sydenham’s chorea: Neurological manifestations with involuntary movements.
- Erythema marginatum: Distinct rash with reddish ring patterns.
- Subcutaneous nodules: Small, firm nodules over joints and bony surfaces.
Diagnosis and Management
Diagnosis follows the Jones Criteria, integrating clinical, laboratory, and echocardiographic findings. Treatment includes antibiotics for streptococcal eradication, anti-inflammatory drugs, and long-term cardiac monitoring.
Conclusion
Early recognition and management of ARF are critical to preventing complications, particularly rheumatic heart disease. Public health interventions focusing on streptococcal infection prevention remain essential.
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