Rheumatic Fever Overview
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Questions and Answers

What is the duration of acute rheumatic fever in most patients?

  • A few days
  • Less than a week
  • Several months (correct)
  • A year

    • Which of the following investigations is NOT commonly performed in cases of acute rheumatic fever?

  • CT scan (correct)
  • Plain chest x-ray
  • Throat culture
  • Electrocardiogram (ECG)

    • What complication is considered an early complication of acute rheumatic fever?

  • Adhesive pericarditis
  • Jaccoud’s arthropathy
  • Mitral stenosis
  • Heart failure (correct)

    • Which of the following markers is NOT indicated as part of laboratory investigations for acute rheumatic fever?

    <p>D-dimer</p> Signup and view all the answers

    According to the Jones Criteria, what is required for the diagnosis of acute rheumatic fever?

    <p>Two major and one minor criterion</p> Signup and view all the answers

    What indicates a recent streptococcal infection in adults regarding antistreptolysin O titer (ASO)?

    <p>Greater than 250 Todd units/ml</p> Signup and view all the answers

    Which of the following symptoms may be present in a patient with acute rheumatic fever?

    <p>History of sore throat 2-3 weeks prior</p> Signup and view all the answers

    What is the primary aim of treating acute rheumatic fever?

    <p>Eradication of group A beta-hemolytic streptococcal infection</p> Signup and view all the answers

    What is the primary reason that rheumatic carditis does not resolve without residual effects?

    <p>It causes irreversible stenosis due to gradual fibrosis.</p> Signup and view all the answers

    Which symptom is a key indicator of acute carditis related to rheumatic fever?

    <p>Disproportionate tachycardia.</p> Signup and view all the answers

    Which part of the heart is NOT typically involved in rheumatic carditis?

    <p>Epicardium</p> Signup and view all the answers

    What characteristic is associated with erythema marginatum?

    <p>It is a cutaneous lesion with migratory borders.</p> Signup and view all the answers

    Which manifestation is common in patients with rheumatic fever involving the heart muscle?

    <p>An abnormal third heart sound.</p> Signup and view all the answers

    What differentiates rheumatic fever's effect on the joints versus the heart?

    <p>The heart experiences irreversible changes while joints recover.</p> Signup and view all the answers

    Subcutaneous nodules associated with rheumatic fever are described as:

    <p>Firm, mobile, and painless.</p> Signup and view all the answers

    Which condition is characterized by fast, jerky, involuntary movements, and may be associated with rheumatic fever?

    <p>Sydenham's chorea.</p> Signup and view all the answers

    Which type of inflammatory lesion is characterized by a central area of fibrinoid degeneration surrounded by lymphocytes?

    <p>Proliferative lesion</p> Signup and view all the answers

    What is the most common age of onset for acute rheumatic fever?

    <p>5-15 years</p> Signup and view all the answers

    Which of the following is NOT a major criterion for diagnosing acute rheumatic fever?

    <p>Fever</p> Signup and view all the answers

    Which statement regarding rheumatic arthritis is correct?

    <p>It leads to complete recovery without deformities.</p> Signup and view all the answers

    What is a common symptom of rheumatic carditis?

    <p>Chronic heart disease</p> Signup and view all the answers

    In which layer of the heart does the myocardium belong?

    <p>Middle layer</p> Signup and view all the answers

    Which of the following contributes to the transmission of upper respiratory tract infections?

    <p>Overcrowding</p> Signup and view all the answers

    What characterizes arthralgia compared to arthritis?

    <p>Pain without signs of inflammation</p> Signup and view all the answers

    What type of infection commonly precedes rheumatic fever?

    <p>Group A B-hemolytic streptococcal infection</p> Signup and view all the answers

    What plays a significant role in the pathogenesis of rheumatic fever?

    <p>Antigenic similarity between streptococcal components and human tissues</p> Signup and view all the answers

    How long is the typical latent period between a streptococcal infection and the onset of rheumatic fever?

    <p>1-5 weeks</p> Signup and view all the answers

    What is the primary mechanism thought to contribute to the development of rheumatic fever?

    <p>Autoimmune response to streptococcal infection</p> Signup and view all the answers

    Which of the following tissues is least affected by rheumatic fever?

    <p>Liver</p> Signup and view all the answers

    Why do only a minority of individuals develop rheumatic fever after a streptococcal infection?

    <p>Host immune responses vary between individuals</p> Signup and view all the answers

    Rheumatic fever is classified as what type of disorder?

    <p>Immunologically mediated inflammatory disorder</p> Signup and view all the answers

    Which characteristic best describes rheumatic fever?

    <p>It is a multi-system disease that primarily affects connective tissues</p> Signup and view all the answers

    What is the primary proposed mechanism by which rheumatic fever develops following a streptococcal infection?

    <p>Cross-reactivity of antibodies with host tissues</p> Signup and view all the answers

    Which clinical feature is most strongly associated with the autoimmune nature of rheumatic fever?

    <p>Presence of anti-streptococcal antibodies in circulation</p> Signup and view all the answers

    What is the latent period between streptococcal infection and the onset of rheumatic fever for most patients?

    <p>1-5 weeks</p> Signup and view all the answers

    Which group of tissues is primarily affected by rheumatic fever?

    <p>Connective tissues</p> Signup and view all the answers

    What factor may contribute to why certain individuals develop rheumatic fever following infection?

    <p>Host immune responses</p> Signup and view all the answers

    Which bacterial strain is most likely to trigger rheumatic fever?

    <p>Group A beta-hemolytic streptococci</p> Signup and view all the answers

    What mechanism explains the damage to distant tissues in rheumatic fever?

    <p>Acute autoimmune reaction</p> Signup and view all the answers

    Which of the following is NOT characteristic of rheumatic fever?

    <p>It is a communicable disease</p> Signup and view all the answers

    Which factor is associated with the theory of molecular mimicry in rheumatic fever?

    <p>Similar amino acid sequences between M protein and human tissues</p> Signup and view all the answers

    What is the primary reason that rheumatic arthritis leads to joint inflammation?

    <p>Autoimmune response targeting joint tissues</p> Signup and view all the answers

    Which of the following is a characteristic of proliferative lesions in rheumatic fever?

    <p>Consists of Aschoff nodules</p> Signup and view all the answers

    What kind of arthritis is most commonly presented in acute rheumatic fever?

    <p>Polyarthritis</p> Signup and view all the answers

    What best describes the clinical feature of rheumatic chorea?

    <p>Involuntary muscle movements affecting limb coordination</p> Signup and view all the answers

    Which factor contributes to the incidence of acute rheumatic fever in populations?

    <p>Overcrowding and poverty</p> Signup and view all the answers

    In which layer of the heart is fibrinoid degeneration observed in rheumatic carditis?

    <p>Endocardium</p> Signup and view all the answers

    Which clinical presentation can indicate the severity of cardiac involvement in acute rheumatic fever?

    <p>Prolonged P-R interval</p> Signup and view all the answers

    What is one of the major criteria for diagnosing rheumatic fever?

    <p>Carditis</p> Signup and view all the answers

    Which inflammatory reaction is marked by a central area of degeneration surrounded by immune cells?

    <p>Aschoff nodule</p> Signup and view all the answers

    What distinguishes arthritis from arthralgia in the context of rheumatic fever?

    <p>Presence of joint swelling and redness</p> Signup and view all the answers

    What is a common laboratory finding in patients with acute rheumatic fever?

    <p>Leukocytosis</p> Signup and view all the answers

    Which of the following is considered a late complication of acute rheumatic fever?

    <p>Mitral stenosis</p> Signup and view all the answers

    Which laboratory test is indicative of a recent streptococcal infection?

    <p>Antistreptolysin O titer (ASO)</p> Signup and view all the answers

    Which statement accurately describes the diagnosis process for acute rheumatic fever?

    <p>It is diagnosed based on Jones Criteria and history of streptococcal infection.</p> Signup and view all the answers

    What type of anemia is commonly observed in patients with acute rheumatic fever?

    <p>Normocytic anemia</p> Signup and view all the answers

    What is the significance of elevated erythrocyte sedimentation rate (ESR) in lab investigations?

    <p>It indicates ongoing inflammatory processes.</p> Signup and view all the answers

    Which of the following is NOT considered an early complication of acute rheumatic fever?

    <p>Jaccoud’s arthropathy</p> Signup and view all the answers

    What is the effect of rheumatic activity in the body?

    <p>Potential for chronic exposure to infection</p> Signup and view all the answers

    Which investigation is least likely to be positive in cases of acute rheumatic fever?

    <p>Throat culture</p> Signup and view all the answers

    Which aspect of acute rheumatic fever is characterized by the absence of specific diagnostic tests?

    <p>Jones Criteria for diagnosis</p> Signup and view all the answers

    What is one of the primary outcomes of the gradual fibrosis process in rheumatic carditis?

    <p>Irreversible stenosis of heart valves</p> Signup and view all the answers

    Which sign can indicate the involvement of myocarditis in rheumatic carditis?

    <p>Abnormal third heart sound</p> Signup and view all the answers

    What characterizes erythema marginatum associated with rheumatic fever?

    <p>Erythematous macules with migratory borders</p> Signup and view all the answers

    Which type of heart complication is most directly related to rheumatic carditis?

    <p>Heart failure due to myocardial damage</p> Signup and view all the answers

    What symptom is commonly associated with the pericardial involvement in rheumatic carditis?

    <p>A pericardial rub</p> Signup and view all the answers

    Which statement correctly describes the subcutaneous nodules associated with rheumatic fever?

    <p>They are typically found along tendons and joint surfaces.</p> Signup and view all the answers

    What type of involuntary movements is characteristic of rheumatic chorea?

    <p>Fast, jerky, involuntary movements</p> Signup and view all the answers

    What differentiates the effects of rheumatic fever on joints versus the heart?

    <p>Joint symptoms resolve quickly without lasting effects.</p> Signup and view all the answers

    What is a common misconception regarding the onset of rheumatic fever symptoms?

    <p>Symptoms such as chorea can appear months after an infection.</p> Signup and view all the answers

    Which statement about the nature of carditis in rheumatic fever is accurate?

    <p>Carditis involves the gradual destruction of valvular function.</p> Signup and view all the answers

    Study Notes

    Rheumatic Fever

    • A sequel to group A β-hemolytic streptococcal pharyngeal infection (rheumatogenic strain)
    • Follows infection of upper respiratory tract especially pharyngitis
    • More likely to occur after severe & recurrent infections
    • Not communicable, but caused by a communicable disease

    Pathogenesis of Rheumatic Fever

    • Molecular mimicry: Molecular similarity between M protein of infecting streptococcus and human tissues like heart, joints, brain, connective tissue
    • The trigger is an autoimmune response
    • Antibodies produced against streptococci cross-react with human tissues
    • Damage to tissues is not due to direct bacterial invasion but an autoimmune reaction
    • Latent period between infection and onset is 1-5 weeks except in chorea, which may occur after several months
    • Occurs in a minority of patients after streptococcal infection, host immune responses likely play a role

    Clinical Presentation of Rheumatic Fever

    • Major Criteria:
      • Arthritis
      • Carditis
      • Rheumatic chorea (Sydenham’s)
      • Subcutaneous nodules
      • Erythema marginatum
    • Minor Criteria:
      • Fever
      • Arthralgia
      • History of rheumatic fever or evidence of rheumatic heart disease
      • Elevated ESR, C-reactive protein, polymorphonuclear leucocytosis
      • Prolonged P-R interval (>0.22s)

    Rheumatic Arthritis

    • Most common presentation
    • Characterized by:
      • Polyarthritis
      • Migratory or fleeting character
      • Predilection for large joints (knees, ankles, elbows, wrists)
      • Dramatic response to salicylates within 24-48 hours
      • Complete recovery with no residual deformities

    Rheumatic Carditis

    • Most serious manifestation
    • Can lead to chronic rheumatic heart disease & severe heart failure
    • Unlike arthritis, carditis does not resolve without residue effects
    • Gradual fibrosis progresses, leading to valvular stenosis and irreversible damage
    • Involvement of all layers of the heart: Pericardium, myocardium, endocardium (Pancarditis)

    Manifestations of Rheumatic Carditis

    • Disproportionate tachycardia, especially if associated with weak or muffled first heart sound
    • Rapid development of functional murmurs
    • Abnormal third heart sound due to myocarditis
    • Pericardial rub due to pericarditis or pericardial effusion
    • Heart failure in severe cases

    Subcutaneous Nodules

    • Small, mobile, painless nodules
    • Firm, non-tender, isolated or in clusters
    • Most common along extensor surface of joints, bony prominences, tendons, dorsal surface of feet
    • Last a few days, with complete resolution

    Erythema Marginatum

    • Highly specific to acute rheumatic fever
    • Erythematous macules with migratory borders and blanching centers (reddish pink border, pale center)
    • Round or irregular shape
    • Often on the trunk, abdomen, inner arms, or thighs
    • Highly suggestive of carditis

    Rheumatic (Sydenham's) Chorea

    • Extrapyramidal disorder (affecting basal ganglia)
    • Fast, jerky, involuntary, purposeless movements especially face, tongue and limbs (hands & feet)
    • Involuntary movements increase with stress
    • Emotional instability may be associated, but mentality remains normal
    • May be the only manifestation of ARF
    • Usually a late manifestation, months after infection
    • Occurs in 30% of patients with ARF
    • Affects females more than males
    • Long duration (several months) but resolves completely with no cerebral damage

    Investigations for Rheumatic Fever

    • Laboratory Investigations:

      • CBC: Leukocytosis and anemia
      • ESR: Markedly elevated, non-specific
      • CRP : Markedly elevated, non-specific
      • ASO: Elevated in 80% of cases, >250 Todd units/ml in adults & 500 Todd units/ml in children (indicative of recent streptococcal infection)
      • Streptozyme test: Positive in more than 95% of cases
      • Throat culture: Positive in only few cases

    • Cardiac Investigations:

      • ECG
      • Chest X-ray
      • Echocardiography

    Diagnosis of Acute Rheumatic Fever

    • Jones Criteria: Developed to prevent overdiagnosis
    • Diagnosis requires presence of two major criteria or one major and two minor criteria, in the presence of data of recent streptococcal infection
    • Evidence of Streptococcal Infection:
      • History of sore throat 2-3 weeks before
      • Residual infection in throat at examination
      • Positive throat swab culture
      • Elevated ASO titer (>250 Todd units/ml)
      • History of recent scarlet fever

    Aim of Treatment

    • Eradication of group A β-hemolytic streptococcus infection
    • Avoid chronic exposure of immune system to streptococcal bacteria
    • Treatment according to presentation

    Complications of Rheumatic Fever

    • Early Complications:
      • Heart failure
      • Arrhythmias
      • Heart Block
    • Late Complications:
      • Rheumatic valvular lesions (e.g mitral stenosis)
      • Rheumatic activity
      • Rarely; adhesive pericarditis & Jaccoud’s arthropathy

    Rheumatic Fever

    • Preceded by infection with group A B-hemolytic streptococci, particularly pharyngitis.
    • More likely to develop after severe and recurrent infections.
    • The mechanism of rheumatic fever development is unknown, but the prevailing theory suggests an autoimmune process.

    Pathogenesis

    • An autoimmune response is triggered by molecular mimicry between M protein of the infecting streptococcus and human tissues like the heart, joints, brain, and connective tissue.
    • The body produces antibodies against streptococci.
    • These antibodies cross-react with human tissues due to antigenic similarity between streptococcal components and human connective tissues.
    • The damage to tissues is not due to direct bacterial invasion but to an autoimmune reaction caused by anti-streptococcal antibodies cross-reacting with host tissues.
    • The latent period between streptococcal infection and rheumatic fever onset is 1-5 weeks, except for chorea, which may occur after several months.

    Factors Influencing Development

    • Host immune responses may play a role in determining who develops rheumatic fever after streptococcal infection.
    • The virulence of the streptococcal strain (rheumatogenic serotype) is a significant factor.

    Definition

    • Rheumatic fever is an inflammatory disorder caused by an immune response to group A-beta hemolytic streptococcal pharyngeal infection (rheumatogenic strain).
    • It affects connective tissue, particularly the heart, joints, brain, and skin (cutaneous and subcutaneous tissue).
    • It is not a communicable disease, but rather a consequence of a communicable disease (streptococcal pharyngitis).

    Pathology

    • Involves inflammatory processes in joints, heart, CNS, and skin & subcutaneous nodules.
    • Two types of inflammatory reactions occur:
      • Exudative lesions mainly affect serous membranes and heal without residual effects.
      • Proliferative lesions ("Aschoff nodules") primarily affect the heart and consist of a central area of fibrinoid degeneration surrounded by lymphocytes, macrophages, Aschoff giant cells, and an outer layer of fibroblasts. They heal with fibrosis.

    Epidemiology

    • Incidence and mortality have declined in the past 30 years due to:
      • Improved socioeconomic conditions.
      • Rapid diagnosis and treatment of strep pharyngitis with effective antibiotics.
    • Commonest age of onset is 5-15 years, but it can occur up to 25-30 years.
    • Males and females are equally affected.
    • Overcrowding, poverty, and lack of access to medical care contribute to the transmission of upper respiratory tract (URT) infections.

    Clinical Presentation

    • Major criteria include Arthritis, Carditis, Rheumatic chorea (Sydenham's), Subcutaneous nodules, and Erythema marginatum.
    • Minor criteria include Fever, Arthralgia, History of rheumatic fever or evidence of rheumatic heart disease, Acute phase reactants (high ESR, C-reactive protein, polymorphnuclear leucocytosis), and Prolonged P-R interval more than 0.22 sec.

    Rheumatic Arthritis

    • Most common presentation of rheumatic fever.
    • Characterized by:
      • Polyarthritis.
      • Migratory or fleeting character.
      • Predilection for large joints.
      • Dramatic response to salicylates within 24-48 hours.
      • Complete recovery with no residual deformities.

    Arthralgia vs. Arthritis

    • Arthritis is inflammation of the joint, presenting with all signs of inflammation (redness, heat, swelling, pain, tenderness, and limited movement). It is a major criteria of rheumatic fever.
    • Arthralgia is joint pain without signs of inflammation. It is a minor criteria of rheumatic fever.

    Rheumatic Carditis

    • Most serious manifestation of rheumatic fever.
    • May lead to chronic rheumatic heart disease and severe heart failure.
    • Unlike arthritis, carditis does not resolve without residual effects.
    • Gradually progresses with slow fibrosis, leading to irreversible stenosis.
    • Rheumatic fever can be gentle on joints but harsh on the heart (the saying "rheumatic fever licks the joints and bites the heart").

    Rheumatic Carditis (cont.)

    • All layers of the heart may be involved (pancarditis).
    • Symptoms and signs depend on the involvement of the pericardium, myocardium, or endocardium.

    Manifestations of Rheumatic Carditis

    • Early signs:
      • Disproportionate tachycardia, particularly with a weak or muffled first heart sound and prolonged P-R interval.
      • Rapid development of functional murmurs.
      • Abnormal third heart sound due to myocarditis.
      • Pericardial rub due to pericarditis or pericardial effusion.
    • Severe cases may present with heart failure due to myocardial damage.

    Subcutaneous Nodules

    • Usually small, mobile, painless nodules.
    • Firm, non-tender, and isolated or in clusters.
    • Most common along extensor surfaces of joints, bony prominences, tendons, and feet.
    • Last a few days with complete resolution.

    Erythema Marginatum

    • Present in 7% of patients.
    • Highly specific to rheumatic fever.
    • Cutaneous lesion characterized by erythematous macules with migratory borders and blanching centers (reddish pink border, pale center).
    • Often found on the trunk, abdomen, inner arms, or thighs.
    • Highly suggestive of carditis.

    Rheumatic (Sydenham's) Chorea

    • Extrapyramidal disorder (affecting basal ganglia).
    • Damage is typically not permanent.
    • Fast, jerky, involuntary, purposeless movements, particularly affecting the face, tongue, and limbs (hands and feet).
    • Involuntary movements increase with stress.
    • May be associated with emotional instability, but mentality remains normal.
    • Can be the only manifestation of rheumatic fever.
    • Usually a late manifestation months after infection.
    • Occurs in 30% of patients with rheumatic fever.
    • Affects females more than males.
    • Lasts for several months but resolves completely without cerebral damage.

    Investigations

    • Complete blood count (CBC): leukocytosis and anemia.
    • Acute phase reactants: elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP).
    • Anti-streptococcal O titer (ASOT).
    • Throat culture.
    • Electrocardiogram (ECG).
    • Plain chest x-ray.
    • Echocardiogram.

    Complications

    • Early complications:
      • Heart failure.
      • Arrhythmias.
      • Heart block.
    • Late complications:
      • Rheumatic valvular lesions (e.g., mitral stenosis).
      • Rheumatic activity.
      • Rarely: adhesive pericarditis and Jaccoud's arthropathy.

    Laboratory Investigations

    • Blood picture:
      • Polymorphnuclear leucocytosis.
      • Normocytic anemia.
    • Erythrocyte sedimentation rate (ESR): markedly elevated, used for follow-up.
    • C-reactive protein (CRP): markedly elevated, used for follow-up.
    • Antistreptolysin O titer (ASO): elevated in 80% of cases, with titers greater than 250 Todd units/ml in adults and 500 Todd units/ml in children indicative of recent streptococcal infection.
    • Streptozyme test: positive in more than 95% of cases.
    • Throat culture: positive in only a few cases.

    Cardiac Investigations

    • ECG.
    • Chest x-ray.
    • Echocardiography.

    Diagnosis

    • No specific diagnostic test exists for acute rheumatic fever.

    Jones Criteria

    • Developed to prevent overdiagnosis.
    • Diagnosis requires:
      • Two major criteria or one major and two minor criteria.
      • Evidence of recent streptococcal infection.

    Evidence of Streptococcal Infection

    • History of sore throat 2-3 weeks prior to presentation.
    • Sometimes a residual throat infection is present at the time of examination.
    • Throat swab culture is often positive for streptococcus.
    • Elevated antistreptolysin O titer (ASOT) more than 250 Todd units/ml or other antistreptococcal antibodies.
    • History of recent scarlet fever.

    Aim of Treatment

    • Eradication of group A beta-hemolytic streptococcal infection.
    • Avoid chronic exposure of the immune system to streptococcal bacteria.
    • Treatment according to the presentation.

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    Description

    This quiz covers the key aspects of rheumatic fever, including its connection to group A streptococcal infections, the pathogenesis involving molecular mimicry, and the clinical presentation criteria. Understand the autoimmune response and its implications for diagnosis and treatment.

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