Acute rheumatic fever

• Disease Description (Causes and Symptoms)

  • Cause: Occurs through an immunologic cross-reaction after a pharyngeal or tonsillar infection (streptococcal pharyngitis) caused by Group A Streptococcus (GAS). The immune response to antigens (e.g., M protein) attacks the heart, joints, central nervous system, and subcutaneous tissues (molecular mimicry, autoimmune mechanism), causing nonpurulent inflammation.
  • Pathophysiology (Summary): Antibody and cellular immune responses cross-react with the myocardium, pericardium, valve tissue, and nervous tissue → valve damage (long-term scarring → rheumatic heart disease), systemic inflammation (arthritis, chorea, etc.).
  • Main Symptoms (Typical): Migratory (polyarticular) arthritis of the large joints (knees, ankles, wrists, etc.), cardiac involvement (valvulitis, myocarditis → tachycardia, heart murmur, signs of heart failure), high fever, Sydenham chorea, subcutaneous nodules, and erythema marginatum. According to the information in the table, epistaxis (nosebleed), abdominal pain, and other symptoms may occasionally accompany it.
  • Diagnostic Essentials: Revised Jones criteria (major manifestations plus evidence of an elevated or inappropriate antibody response or evidence of a streptococcal infection are required). Evidence of streptococcal infection includes a throat culture, rapid antigen test, or elevated ASO/anti-DNase B levels.

• Treatment (Standard Treatment, Medication, Procedures/Surgery, Lifestyle Management, and Precautions)

  • Acute Phase (Eradication of Infection)
    • Antibiotics: Elimination/eradication of GAS — penicillin is recommended (e.g., a single IM dose of benzathine penicillin G or oral penicillin V for 10 days). If allergic to penicillin, substitute a macrolide (e.g., erythromycin or azithromycin).
    • Purpose: Treatment of the acute infection and prevention of subsequent reinfection and recurrence (secondary prevention).
  • Anti-inflammatory and Symptomatic Treatment
    • Arthritis: High-dose aspirin (children: 60–100 mg/kg/day in divided doses; adults: several g/day) or an NSAID (at the physician’s discretion). When aspirin is used, monitoring for bleeding, gastrointestinal adverse effects, and liver function is necessary (including clinical judgment regarding the risk of Reye syndrome in children in situations where a viral infection is suspected).
    • Cardiac involvement (myocarditis/heart failure): Steroids (e.g., prednisone) may be considered in severe cases. In congestive heart failure, standard heart failure treatment (diuretics, ACE inhibitors, etc.) and intensive care-level support may be required.
    • Sydenham chorea: If symptoms are severe, some anticonvulsants or antipsychotics may be used (in consultation with neurology or pediatric neurology).
  • Secondary Prevention (Prevention of Recurrence, Important)
    • Long-term maintenance with IM benzathine penicillin G injections (e.g., 1.2 MU for adults; weight-based dosing for children) at 3- to 4-week intervals. The recommended duration varies depending on whether there is cardiac involvement:
      • No cardiac involvement: At least 5 years or until age 21 (whichever is longer).
      • Cardiac involvement but no residual lesions: At least 10 years or until age 21.
      • Permanent cardiac valve lesion (residual lesion): At least 10 years or, in some cases, lifelong prophylaxis is recommended.
  • Surgical Treatment
    • If there is severe valve damage that does not improve with medication or complications (heart failure, decreased cardiac function), valve surgery (valve replacement or valvuloplasty) may be considered.
  • Lifestyle Management and Precautions
    • Rest and reduced activity are recommended during the acute phase, especially with strenuous activity restricted when there is cardiac involvement.
    • Monitor for adverse effects of anti-inflammatory drugs such as aspirin (gastrointestinal bleeding, changes in liver enzyme levels).
    • Check the history of penicillin allergy and sensitivity before using an alternative antibiotic.
    • Assess valve lesions and function through long-term follow-up (echocardiography, electrocardiography, etc.).

• Points to Check Before an Outpatient Visit (Items to Bring, Test Records, Symptom Records, Including Sample Questions)

  • Items to Bring (Helpful for Treatment and Communication)
    • A list of all medications currently being taken (including prescription drugs, OTC drugs, and supplements)
    • Records of past medical conditions and medication allergies (especially whether there is a penicillin allergy)
    • If available, reports of recent throat culture or rapid antigen test results, or blood tests (such as an ASO titer)
    • Recent electrocardiogram (ECG), chest X-ray, and echocardiogram (Echo) results (if available)
    • A symptom diary including the date symptoms began, temperature records, the migratory pattern of joint pain (locations and duration), and whether movement is restricted
  • Useful Information to Show the Doctor at the Outpatient Visit
    • Recent throat infection or contact with someone who had an infectious disease (e.g., scarlet fever or a person with a streptococcal infection)
    • Antibiotics recently received and the response to treatment
  • Sample Questions for Testing and Additional Preparation (For the Patient to Check Personally)
    • “Have you ever experienced a reaction such as hives or difficulty breathing after receiving penicillin, even once?”
    • “Have you had symptoms of pharyngitis (sore throat or fever) recently, within the past 6 weeks?”

• General Sample Questions to Ask the Doctor

  • What are the major adverse effects of this medication (e.g., penicillin, aspirin, or steroids), and how should I respond if they occur?
  • What is the condition of my heart (based on the echocardiogram results), and what is the prognosis going forward?
  • How long will secondary prevention (benzathine penicillin) be necessary in my case?
  • Are there any special activities or exercises I should avoid in everyday life?
  • If I am allergic to penicillin, what alternative antibiotics are available, and how effective are they?

• Examples of Disease-Specific Questions (1–2 Questions Appropriate to the Condition)

  • If a valve lesion (regurgitation or stenosis) was seen on a recent echocardiogram: “Does the current degree of valve regurgitation (or stenosis) meet the criteria for surgery? How often should follow-up be performed?”
  • For chorea (when motor and emotional symptoms are present): “What medications can be used when chorea significantly interferes with daily life, and what are their adverse effects? Is additional evaluation by neurology necessary?”

Additional Recommendations (Brief)

• If acute rheumatic fever is suspected (high fever, migratory arthritis, heart murmur, chorea, etc.), immediate medical evaluation is recommended. If severe cardiac symptoms (difficulty breathing, hypotension, severe chest pain, or altered consciousness) occur, go to the emergency room.
• When symptoms of pharyngitis are present, appropriate diagnosis (rapid antigen testing or culture) and timely antibiotic treatment are important for primary prevention (prevention of rheumatic fever).