RHEUMATIC FEVER
DR. [Link]
LECTURER
INTRODUCTION
• Rheumatic fever is an acute, immunologically
mediated, multisystem inflammatory disease which
may develop a few weeks after a Group A Beta
haemolytic streptococcal infection pharyngitis and
often involves the heart.
• Widespread use of antibiotics has reduced its
prevalence.
• In the third world countries and in the crowded
urban areas of the western world rheumatic fever is
an important public health problem.
INTRODUCTION
• Rheumatic fever primarily affects children between ages 5
and 15 years approximately 20 days after strep throat or
scarlet fever. In up to a third of cases, the underlying strep
infection may not have caused any symptoms.
• The rate of development of rheumatic fever in individuals
with untreated strep infection is estimated to be 3%.
• The rate of development is far lower in individuals who have
received antibiotic treatment. Persons who have suffered a
case of rheumatic fever previously have a tendency to
develop flare-ups with repeated strep infections.
PATHOGENESIS
• The exact pathogenesis is uncertain.
• It is proposed that the antibodies directed against the M
proteins of certain strains of streptococci cross-react with
tissue glycoproteins in the heart, joints and other tissues.
• Streptococcal infection evokes an autoimmune response
against self antigens.
• Genetic susceptibility regulates the hypersensitivity reaction.
• The chronic sequelae result from progressive fibrosis because
of both healing of the acute inflammatory lesions and the
turbulence induced by the on-going valvular deformities.
CLINICAL MANIFESTATIONS
• Diagnosis: modified Jones criteria
• T. Duckett Jones, MD, first published these criteria in
1944.
• They have been periodically revised by the American
Heart Association and other groups.
• Two major criteria, and one major and two minor
criteria, when there is also evidence of a previous
strep infection support the diagnosis of rheumatic
fever.
Major criteria
• Carditis: inflammation of the heart muscle which can
manifest as congestive cardiac failure with shortness
of breath, pericarditis with a rub, or a new heart
murmur’
• Migratory polyarthritis: temporary migrating
inflammation of the large joints, usually starting in
the legs and migrating upwards.
..MAJOR CRITERIA
• Sydenhams chorea (St. Vitus' dance): a
characteristic series of rapid movements of
the face and arms without purpose. The
movements are sudden, aimless, irregular
and involuntary. Its more common in girls than
in boys and is self limiting. It has no
permanent neural sequelae. This can occur
very late in the disease.
• Erythema marginatum: rash that begins on the trunk
or arms as macules and spread outward to form a
snakelike ring while clearing in the middle. This rash
never starts on the face and is made worse with
heat.
• Subcutaneous nodules): painless, firm collections of
collagen fibers on the back of the wrist, the outside
elbow, and the front of the knees. These now occur
infrequently.
Minor criteria
• Fever
• Arthralgia: Joint pain without swelling
• Laboratory abnormalities: increased ESR, increased
C reactive protein, leucocytosis
• Echocardiogram abnormalities: a prolonged PR
interval
• Evidence of Group A Strep infection: positive culture
for Group A Strep, elevated or rising antistreptolysin
O titres
• Previous rheumatic fever or inactive heart disease
Other signs and symptoms
• Abdominal pains
• Epistaxis
• Other antibody tests are sensitive
prognosticators of streptococcal infection
including DNase B, antihyaluronidase and
antistreptozyme.
MORPHOLOGY
• Aschoff bodies: found within the heart and
constitute foci of fibrinoid degeneration surrounded
by lymphocytes, occasional plasma cell and plump
macrophages called Anitschkow cells (
pathognomonic for rheumatic fever). During acute
rheumatic fever diffuse inflammation and aschoff
bodies may be found in any of the three layers of the
heart pericardium, myocardium or endocardium
hence pancarditis.
• In the pericardium they are accompanied by a
fibrinous pericardial exudates.
LABORATORY DIAGNOSIS
– Approximately 80% have an elevated anti-
streptolysin o titer. Only levels of over 300 units
are considered abnormal.
– Patients suspected of rheumatic fever with no
abnormal ASOT titers should be tested for anti-
DNase B and anti-DPNase. Streptozyme can also
be done.
– Tests generally used to measure inflammatory
process including ESR and CRP
..Lab diagnosis
– Mild to moderate anaemia is common in active
rheumatic fever.
– Prolongation of the PR interval in the ECG occur in
about one third of patients with rheumatic fever.
Other ECG changes include flattened or inverted T
waves or elevation of the S-T segment.
– CXR will detect cardiac enlargement and
pericardial effusion.
TREATMENT
• All patients with acute rheumatic fever should be placed at
bed rest. The duration and degree of bed rest should vary
with the severity of the disease.
• Anti-inflammatory drugs:
• -arthralgia . -- analgesics only
• -arthritis only and/or carditis without cardiomegally. --
Salicylates 100mg/kg/24hrs for 2 weeks and 75 mg/kg/24hrs
for 4-6 weeks.
• -carditis with cardiomegally or failure.—prednisone 2
mg/kg/24hrs for 2 weeks and taper over 2 weeks and
salicylates 75mg/kg/24hrs at 2 weeks and continue for 6
weeks.
PROGNOSIS
• Sequelae of rheumatic fever are essentially limited to
the heart and depends on the presence and the
severity of carditis. When there is no clinical
evidence of carditis during the acute attack
completerecovery is the rule. The prognosis is also
excellent if the findings are limited to prolongation of
the P-R interval.
Sample MCQ
• All of the following are features of rheumatic
fever EXCEPT:
• Carditis
• Subcutaneous nodules
• Elevated Antistreptolysin
• Aschoff bodies in the heart
• Erythema nodosum
•
•
Persistent sore throat may affect
the heart
Rheumatic fever