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Indian Journal for the Practising Doctor

Diagnosis of Rheumatic Fever

Author(s): S.M. Kadri

Vol. 2, No. 1 (2005-03 - 2005-04)

Rheumatic fever causes chronic progressive damage to the heart and its valves. Until 1960, it was a leading cause of death in children and a common cause of structural heart disease. The disease has been known for many centuries. Baillou (1538-1616) first distinguished acute rheumatic arthritis from gout. Sydenham (1624-1668) described chorea but did not associate it with acute rheumatic fever (ARF). In 1812, Charles Wells associated rheumatism with carditis and provided the first description of the subcutaneous nodules. In 1836, Jean-Baptiste Bouillaud and, in 1889, Walter Cheadle published classic works on the subject.

The association between sore throat and rheumatic fever was not made until 1880. The connection with scarlet fever was established in the early 1900s. In 1944, the Jones criteria were formulated to assist disease identification. These criteria, with some modification, remain in use even today. The introduction of antibiotics in the late 1940s allowed for the development of treatment and preventive strategies. The dramatic decline in the incidence of rheumatic fever is thought to be largely owing to antibiotic treatment of streptococcal infection.

  • It must be remembered that diagnosis of ARF requires a high index of suspicion.
  • Guidelines of diagnosis used by the American Heart Association include major and minor criteria (ie, modified Jones criteria). In addition to evidence of a previous streptococcal infection, the diagnosis requires 2 major Jones criteria or 1 major plus 2 minor Jones criteria.
  • Guidelines of diagnosis used by the American Heart Association include major and minor criteria (ie, modified Jones criteria). In addition to evidence of a previous streptococcal infection, the diagnosis requires 2 major Jones criteria or 1 major plus 2 minor Jones criteria.

Major criteria

Carditis: This occurs in as many as 40% of patients and may include cardiomegaly, new murmur, congestive heart failure, and pericarditis, with or without a rub and valvular disease.

Migratory polyarthritis: This condition occurs in 75% of cases and is polyarticular, fleeting, and involves the large joints.

Subcutaneous nodules (ie, Aschoff bodies): These nodules occur in 10% of patients and are edematous, fragmented collagen fibers. They are firm, painless nodules on the extensor surfaces of the wrists, elbows, and knees.

Erythema marginatum: This condition occurs in about 5% of cases. The rash is serpiginous and long lasting.

Chorea (also known as Sydenham chorea and "St Vitus dance"): This characteristic movement disorder occurs in 5-10% of cases. Sydenham chorea consists of rapid, purposeless movements of the face and upper extremities. Onset may be delayed for several months and may cease when the patient is asleep.

Minor criteria

Clinical findings include arthralgia, fever and previous history of ARF

Laboratory findings include elevated acute phase reactants (eg, erythrocyte sedimentation rate, C reactive protein), a prolonged PR interval, and supporting evidence of antecedent group A streptococcal infections (ie, positive throat culture or rapid streptococcal screen and an elevated or rising streptococcal antibody titre).

Lab Studies:

  • No specific confirmatory laboratory tests exist. However, several laboratory findings indicate continuing rheumatic inflammation. Some are part of the Jones minor criteria.
  • Streptococcal antibody tests disclose preceding streptococcal infection.
  • Isolation of group A streptococci via throat culture.
  • Acute phase reactants (eg, erythrocyte sedimentation rate [ESR], C-reactive protein [CRP] in serum, and leukocytosis) may show an increase in serum complement, mucoproteins, alpha-2, and gamma globulins. Anemia usually is caused by suppression of erythropoiesis.
  • ECG- PR interval prolongation is present in approximately 25% of all cases and is neither specific to nor diagnostic of ARF.
  • Troponins have not been shown to be helpful in making the diagnosis since ischemia and necrosis are not the major cardiac problems.

Imaging Studies:

  • Echocardiography may be helpful in establishing carditis.
  • Synovial fluid analysis may demonstrate an elevated white blood cell count with no crystals or organisms.

References

  1. Andy JJ, Soomro RM: The changing incidence of juvenile mitral stenosis and natural history of rheumatic mitral valvulitis in Al Baha, Saudi Arabia. Ann Trop Paediatr 2001 Jun; 21(2): 105-9
  2. Birdi N, Hosking M, Clulow MK, et al: Acute rheumatic fever and poststreptococcal reactive arthritis: diagnostic and treatment practices of pediatric subspecialists in Canada. J Rheumatol 2001 Jul; 28(7): 1681-8
  3. Bisno AL, Gerber MA, Gwaltney JM, et al: Practice guidelines for the diagnosis and management of group A streptococcal pharyngitis. Infectious Diseases Society of America. Clin Infect Dis 2002 Jul 15; 35(2): 113-25
  4. Bronze MS, Dale JB: The re-emergence of serious group A streptococcal infections and acute rheumatic fever. Am J Med Sci 1996 Jan; 311(1): 41-54
  5. Cooper RJ, Hoffman JR, Bartlett JG, et al: Principles of appropriate antibiotic use for acute pharyngitis in adults: background. Ann Emerg Med 2001 Jun; 37(6): 711-9
  6. Hashkes PJ, Tauber T, Somekh E, et al: Naproxen as an alternative to aspirin for the treatment of arthritis of rheumatic fever: a randomized trial. J Pediatr 2003 Sep; 143(3): 399-401
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