PITFALLS IN DIAGNOSIS AND MANAGEMENT OF RHEUMATIC FEVER
Sujata Sawhney*
Department of Pediatrics.*
Number Myth Pearl
1. Elevated ASOT = rheumatic fever

Not in 95% of patients

2. Single ASOT is usually sufficient

A two fold rise is more meaningful

3.

Negative ASOT excludes acute rheumatic fever

Not in certain circumstances. Anti D Nase B adds value to the screen. Late onset chorea may not have positive titers. 15% of patients may have negative ASOT.

4.

Arthralgia and fever with high ASOT = ARF

At least one major criteria is needed for the diagnosis of ARF

5.

ASOT is not positive in other diseases

Positive in 30% of children with SOJIA

6.

Arthritis for more than 12 weeks needs PENIDURE

Arthritis for more than 8-12 weeks virtually excludes ARF

7. Neck, Back and small joints of the hands are involved commonly in ARF These joints are very infrequently involved in ARF
8. Carditis occurs in all patients

50% of patients don't develop carditis

9. Pericarditis in isolation occurs in ARF

It always occurs as a part of pancarditis

10. Steroids are needed for treatment of arthritis There is no indication for steroids for the treatment of arthritis
11. Echocardiography does not add value to patient assessment

If available and affordable it adds detail to examination and diagnosis.

12. Steroids alter the long term cardiac outlook

They don't impact on long term cardiac disease.



  • Early recognition of streptococcal sore throat is essential to treat this infection and dramatically reduce the new cases of

    acute rheumatic fever

    . This needs to go hand in hand with proper hygiene, improvement of living standards and reduction of overcrowding.
  • Similarly

    prompt treatment and management

    of acute rheumatic fever can reduce morbidity and appropriate treatment is needed to prevent recurrent attacks of ARF.
  • Over diagnosis of ARF, reliance on ASOT for

    diagnosis,

    unnecessary use of steroids and early initiation of NSAIDS causes

    diagnostic confusion

    and sentences many children to unwarranted use of PENIDURE for years on end.
  • It is the onus of the pediatrician to pick up the clinical signs and seek help where needed. This is a diagnosis that in the absence of carditis is notoriously difficult to make in retrospect.

Data From India:
Consider this example:

If the attack rate of rheumatic fever is 0.3-3% of all streptococcal sore throat then there would be between 50,000 and 500,000 new cases of acute rheumatic fever in India every year! According to recent epidemiological surveys among school children, the prevalence of

rheumatic heart disease

varies from 1-5.4 per 1000 school-age children and the incidence of acute rheumatic fever varies from 0.2-0.75 per 1000 school-age children 5-15 years of age. Using the 2001 National census Data information that 23.6% of the total population of India (1027 million) is between 5 and 15 years of age, one may estimate that approximately 600,000 children less than 15 years of age are currently suffering from chronic rheumatic heart disease, and that approximately 121,000 children are newly diagnosed with acute rheumatic fever every year. This is indeed a tremendous national health burden.

Rheumatic Fever:
Rheumatic fever is a delayed, nonsuppurative sequel of a pharyngeal infection with the group A beta hemolytic streptococcus. The onset of the disease is usually with an acute febrile illness. The Jones criteria for diagnosing rheumatic fever are as follows:

Table: Jones criteria for diagnosis of Acute Rheumatic Fever (Updated 1992)
Carditis Fever
Polyarthritis Arthralgia
Chorea Elevated acute phase reactants

Erythema marginatum

C-reactive protein

Subcutaneous nodules

Erythrocyte sedimentation rate Prolonged P-R interval rate


Supporting Evidence of Preceding Streptococcal Infection

Increased ASO or other streptococcal antibodies/antigens

Positive throat culture for group A hemolytic streptococci

  • The Jones criteria should be viewed as a guide to determine who is at high risk but cannot be used to define diagnosis with absolute certainty.
  • An exception includes chorea, which can present as the sole manifestation of ARF, in spite of negative laboratory data.

  • Another possible exception is indolent carditis.

Clinical features of acute rheumatic fever with important differences from other autoimmune conditions are detailed below:

Fever:


Most patients with acute rheumatic fever have moderate grade fever between 2 to 6 weeks. This is quite different from the quotidian fever seen in children with systemic onset JIA. With SOJIA the child looks well between the fever episodes and there is an evanescent rash maximal at the spike o fever.

Carditis:


This is potentially a life threatening condition in children with acute rheumatic fever. Rheumatic carditis is usually a pan carditis. Pericarditis in patients with rheumatic heart disease is seen only with pancarditis. On the other hand patients with systemic onset JIA may have pericardial effusions, without involvement of Endocardium. This is an important point to distinguish the two diseases, Echocardiography sharply increased the pick rate of endocardial disease, however this asymptomatic detection of valvular disease is not included in the diagnostic criteria of acute rheumatic fever. Advantages of echocardiographic assessment are as follows:
  • Clinical detection of soft murmurs may be difficult due to tachycardia.
  • Doppler and color flow mapping is more sensitive in picking up minor digress of valvular regurgitation than clinical examination.
  • The yield of carditis with valvular regurgitation increases with the use of echocardiography.
  • This is of great value in mixed valve lesions to determine the severity of each lesion.
  • Additional abnormalities that can be detected are:
    • Prolapse of the valve
    • Focal nodular thickening of leaflets
    • Pericardial effusion.

Arthritis:


Rheumatic arthritis is the commonest major criteria of acute rheumatic fever seen worldwide. It is also the single most important cause of diagnostic confusion and therapeutic misadventures. It is well known that is arthritis in these children is very sensitive to NSAID/Aspirin. Thus if a patient is given any of these medications early in the cause of the disease the classic migratory polyarthritic pattern is quickly lost. The pain is usually more prominent than the objective signs of inflammation. It is only when the disease is allowed to express itself fully (no anti-inflammatory treatment is given) that over half the patients show a true polyarthritis. Classically, each joint is maximally inflamed for only a few days or a week or the most. The arthritis is transient and seldom lasts for more than four to six weeks. Typically the patient has large joint disease involving the lower limbs. The back and small joints of the hands and feet are not involved in the acute rheumatic fever. Thus fleeting transient polyarthritis is the hallmark of the arthritis of acute rheumatic fever. This contrasts sharply with additive and sustained arthritis as seen in JIA.

Chorea:


Also known as St. Vitus' dance, is because of involvement of the basal ganglia and occurs in 15% patients. This is a delayed onset major criteria of acute rheumatic fever with patients presenting up to 12 months after exposure to streptococcus. Patients with Sydenham's chorea present with persistent involuntary and purposeless movements of extremities. Speech is halting and explosive. There are various clinical tests that point to a diagnosis of chorea.

Erythema Marginatum:


This is uncommon manifestation of acute rheumatic fever, seen in only 5% of patients. The rash is characteristically non-pruritic and macular with a serpiginous erythematous border. This is very different from the rash of SOJIA, which is evanescent, macular and salmon pink.

Subcutaneous Nodules:


They are located on the extensor surfaces of the joints, especially knees and elbows. These nodules are common in patients with chronic rheumatic heart disease and signify active carditis. In many respects they resemble benign rheumatoid nodules.

Minor manifestations of the Disease:


Non-specific manifestations of fever, arthralgia, elevated ESR and CRP are also common in other rheumatic disorders. The fever is variable in severity; between 38 to 40o C may be present during the acute phase of the disease. A prolonged P-R interval on electrocardiography occurs in patients with acute rheumatic carditis.
In summary acute rheumatic fever has a specific clinical sign that must be recognized so the patient is treated adequately and long-term cardiac morbidity is prevented.
Features that suggest a diagnosis other than acute rheumatic fever are:
  • Absence of joint erythema
  • Recurrent course with poor response to NSAID.
  • Absence of evidence for antecedent groups A streptococcal infection.

Supporting Evidence of Antecedent Group A Streptococcal Infection:
The supporting Evidence of Antecedent Group A Streptococcal infections are two:
  • A positive throat culture because of the latency period between the initial pharyngitis and the onset of ARF reduces the frequency of positive throat cultures to about 30%.
  • An elevated ASO or anti-Dnase B is however expected in up to 85% of patients. More than 90% of patients show an elevated titer for one of these tests. A two-tube increase and decrease in titers should be demonstrated over time.
  • The three acute phase reactants in acute rheumatic are ESR, CRP and leukocyte count. Of these the leukocyte count is the most variable and least dependable.
It is important to note that patients with systemic onset JIA may also have an elevation in the ASO titers. Up to one third of children with systemic onset arthritis may have a moderate elevation of the ASO titers. This is a reflection of inflammation rather than evidence of recent streptococcal infection.

Post streptococcal reactive arthritis (PSRA):



This is a post streptococcal arthritic condition that does not fulfill the Jones criteria for diagnosis of acute rheumatic fever. The chief clinical features of this disorder are:
  • Additive rather than migratory arthritis.
  • Relatively poor response to salicylates.
  • Persistence for mean of two months.
  • Elevated acute phase reactants
  • Serologic evidence of recent group A streptococcal infection.
  • 6% of these patients may develop mitral valve disease, it is thus recommended that antistreptococcal prophylaxis be given for 1 year and then discontinued if there is no evidence of cardiac involvement.

Management of Acute Rheumatic Fever:


The management of acute rheumatic fever depends on the problems that the patient has and focuses on the following principles:
  • All patients receive secondary prophylaxis of acute rheumatic fever with penicillin given three weekly parenterally (I.M.), or orally. If the patient is allergic to penicillin he may be given erythromycin. Management of current infection does not alter the course of the disease. The decision to withdraw anti bacterial prophylaxis should be carefully undertaken in each patient per the risk profile of repeated infections. Patients with cardiac lesions need life long prophylaxis.
  • Anti-inflammatory treatment for the arthritis or carditis: The tried and tested drug for this condition is aspirin. The use is per the patient's clinical problem. Typically 80-100 mg/kg/day in 4-6 divided doses may be needed.
  • Use of steroids: In difficult to control carditis or for treatment of carditis with congestive failure. A Cochrane database review in 2003 has revealed that there is no significant difference in the risk of cardiac disease at one year between the corticosteroid-treated and aspirin-treated groups (relative risk 0.87, 95% confidence interval 0.66 to 1.15). Most of the trials studied were between 1950 and 1965; only two were done in the last 2 years. The antiquity of most of the trials restricted adequate. Statistical analysis of the data and acceptable assessment of clinical outcomes by current standards. The use of digoxin, diuretics and vasodilators are needed on occasions.
  • Use of haloperidol for movement disorder i.e., chorea. All patients with chorea should get treatment for secondary prophylaxis of streptococcal infections. Glucocorticoids and salicylates have no effect on chorea. Sedation is sometimes needed to tide over a difficult period.
  • Surgery is reserved for special situations.
  • Valve replacement in patients' refractory to medical treatment.
  • Regurgitant lesions respond well to valve replacement.
  • Pure stenotic lesions respond to conservative commissurotomy.

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