Acute Rheumatic Fever: Pathophysiology, Clinical Manifestations, Complications, Diagnosis, Management, and Prevention

Acute Rheumatic Fever (ARF) is an inflammatory disease that occurs as a delayed complication of untreated or inadequately treated streptococcal throat infection. It primarily affects the heart, joints, skin, and central nervous system.

Pathophysiology

ARF is an autoimmune response triggered by Group A Streptococcus (GAS) infection. Molecular mimicry leads to immune-mediated damage to various tissues, particularly the heart valves, resulting in rheumatic heart disease (RHD).

Clinical Manifestations

• Carditis (inflammation of the heart)
• Polyarthritis (migratory joint inflammation)
• Chorea (neurological disorder causing involuntary movements)
• Erythema marginatum (skin rash)
• Subcutaneous nodules

Complications

Major Complications

• Rheumatic heart disease (RHD) leading to valvular damage
• Heart failure due to progressive valve dysfunction
• Endocarditis (infection of damaged heart valves)

Minor Complications

• Persistent arthritis
• Neurological sequelae such as Sydenham’s chorea
• Chronic fatigue and recurrent episodes

Diagnosis

Diagnosis is based on the modified Jones criteria, which include major and minor clinical manifestations along with evidence of preceding streptococcal infection.

Management

• Antibiotic therapy (penicillin to eradicate GAS)
• Anti-inflammatory treatment (aspirin or corticosteroids)
• Supportive care for cardiac and neurological symptoms

Prevention

Primary prevention involves early treatment of streptococcal pharyngitis with antibiotics. Secondary prevention includes long-term prophylactic antibiotics to prevent recurrence in individuals with a history of ARF.