Rheumatic fever I00.x1

Immunologically triggered febrile sequelae of angina tonsillaris with ß-hemolytic streptococci or streptococcal pharyngitis, which has become rare in industrialized countries.

In developing countries: Most common cause of heart disease in childhood. Rare in industrialized countries.

An autoimmune reaction is discussed, which occurs as a secondary disease about 2-3 weeks after an infection of the pharynx and leads to an exudative inflammatory reaction in the heart, joints and skin. Toxins of the beta-hemolytic group A streptococci are responsible, which lead to the formation of autoantibodies against myocardial and endocardial structures in the organism (molecular mimicry).

Predominantly (but not exclusively) limited to childhood (age of illness between the 3rd and 16th year of life).

• Major symptoms: Carditis, Polyarthritis (migratory), Chorea minor (Sydenham), Erythema anularerheumaticum(Erythema marginatumrheumaticum), and Subcutaneous nodules(Rheumatic nodules). SPECK: S=Subcutaneous nodules, P=Polyarthritis, E=Erythema anulare, C=Chorea, K=Carditis
  Secondary criteria: fever, arthralgias, elevated ESR/leukocytes, prolonged PQ or PR time.
• In less than 5% of patients, urticarial lesions and erythematous papules form on the knee and elbow (non-anular) and regress within a few weeks(erythema papulatum - Cockayne 1912).
• Other: Myocarditis (in infants), pancarditis (in young children), periendocarditis (in school children). Development of mitral stenosis or aortic regurgitation is possible. Occasional renal involvement (glomerulonephritis).

The diagnosis of "rheumatic fever" is most likely if 2 major criteria or 1 major criterion and 2 minor criteria are met with confirmed previous streptococcal infection. The oligo- or polyarthritis of rheumatic fever practically never becomes chronic, i.e. there is no "secondary chronic polyarthritis".

Treatment of streptococcal infection with penicillin G i.v. (e.g. penicillin Grünenthal) 3-5 million IU over 10 days. In case of penicillin allergy: alternatively use erythromycin or antibiosis according to antibiogram.

In case of severe symptoms with arthritides and carditis additionally glucocorticoids such as prednisone (e.g. Decortin) 60-100 mg/day, gradual dose reduction over several weeks, maintenance dose according to clinic. In addition, antiphlogistic treatment with acetylsalicylic acid (e.g. ASS Tbl.) can be used in high doses of 500-1000 mg 3 times/day (in milder cases, treatment with acetylsalicylic acid alone is also possible). For severe pain, etofenamate 1 g deep i.m. as ED.

Continuous chemoprophylaxis is obligatory: 2-4 times/day 0.25 million IU penicillin V (e.g. Megacillin Filmtbl.) in rheumatic fever in childhood up to 25 years of age, in disease after 15 years of age over 5-10 years. Thereafter, only interim antibiosis in surgical procedures.

Recurrences are frequent and increase the risk of developing a heart valve defect

Heart involvement is the decisive prognostic factor

Rheumatoid chorea: Cures in 90% of cases within 10 weeks with appropriate therapy

Residuals (e.g. restlessness) can remain, recurrences are even possible despite prophylaxis

Antibiotic administration (e.g. penicillin V) after a positive streptococcal rapid test (e.g. for angina tonsillaris)

Antibiotic reinfection prophylaxis

Duration of antibiotic prophylaxis:

• Rheumatic fever without carditis: usually until the 21st year of life, but at least for 5 years
• Rheumatic fever with carditis: usually until the 21st year of life, but at least for 10 years
• Rheumatic fever with carditis and permanent heart valve damage: usually up to the 40th LJ, but at least for 10 years

1. Kadavath S et al(2015) Adult-onset Still's disease-pathogenesis, clinical manifestations, and new treatment options. Ann Med 47:6-14
2. Lehndorff H, Leiner C (1922) Erythema annulare. Z Kinderheilkd (Berlin) 32: 46
3. Martin WJ et al (2015) Post-infectious group A streptococcal autoimmune syndromes and the heart.autoimmune Rev 14:710-725
4. Müller W, Zeitler H (1998) Differential diagnosis of rheumatic diseases. Springer, Berlin Heidelberg New York, S. 143-144
5. Rullan E et al (2001) Rheumatic fever. Curr Rheumatol Rep 3: 445-452Sato
   S et al. (2017) A retrospective study: Acute rheumatic fever and post-streptococcal reactive arthritis
   in Japan. Allergology international: official journal of the JapaneseSociety
   of Allergology 66: 617-620.
6. Walz B et al (2015) Fever, skin changes, myalgia--from early symptom to diagnosis. German Med Weekly 140:1137-1144