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What type of disease is rheumatic fever considered?
What type of disease is rheumatic fever considered?
Which age group is most commonly affected by rheumatic fever?
Which age group is most commonly affected by rheumatic fever?
What is not a major manifestation of rheumatic fever according to Jones' criteria?
What is not a major manifestation of rheumatic fever according to Jones' criteria?
Which of the following is a primary mechanism in the pathogenesis of rheumatic fever?
Which of the following is a primary mechanism in the pathogenesis of rheumatic fever?
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Which parameter is a laboratory finding supporting the diagnosis of rheumatic fever?
Which parameter is a laboratory finding supporting the diagnosis of rheumatic fever?
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Which tissue is primarily affected by the autoimmune response in rheumatic fever?
Which tissue is primarily affected by the autoimmune response in rheumatic fever?
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What type of inflammation does pancarditis refer to?
What type of inflammation does pancarditis refer to?
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What is the role of the M protein in the pathogenesis of rheumatic fever?
What is the role of the M protein in the pathogenesis of rheumatic fever?
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What is a possible consequence of a mycotic aneurysm?
What is a possible consequence of a mycotic aneurysm?
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Which condition is NOT commonly associated with raised ESR?
Which condition is NOT commonly associated with raised ESR?
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Which diagnostic tool is most likely to show vegetations in cases of endocarditis?
Which diagnostic tool is most likely to show vegetations in cases of endocarditis?
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Which type of endocarditis is characterized by small, bland vegetations that do not cause destruction?
Which type of endocarditis is characterized by small, bland vegetations that do not cause destruction?
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Which procedure is important for prophylaxis in patients with cardiac abnormalities?
Which procedure is important for prophylaxis in patients with cardiac abnormalities?
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What is a common form of vegetative endocarditis involving the mitral valve?
What is a common form of vegetative endocarditis involving the mitral valve?
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What is a major difference between rheumatic endocarditis and Libman-Sacks endocarditis?
What is a major difference between rheumatic endocarditis and Libman-Sacks endocarditis?
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Which of the following is a characteristic feature of infective endocarditis?
Which of the following is a characteristic feature of infective endocarditis?
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What type of cells are characterized as 'caterpillar cells' in the context of Aschoff nodules?
What type of cells are characterized as 'caterpillar cells' in the context of Aschoff nodules?
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In which part of the heart do Aschoff nodules typically form during myocarditis?
In which part of the heart do Aschoff nodules typically form during myocarditis?
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What type of effusion is commonly associated with pericarditis due to Aschoff nodules?
What type of effusion is commonly associated with pericarditis due to Aschoff nodules?
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What is the typical clinical feature of arthritis associated with rheumatic fever?
What is the typical clinical feature of arthritis associated with rheumatic fever?
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What are the small vegetations called that may develop along the valve leaflets due to endocarditis in rheumatic fever?
What are the small vegetations called that may develop along the valve leaflets due to endocarditis in rheumatic fever?
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Which risk factor is least likely to contribute to the development of acute infective endocarditis?
Which risk factor is least likely to contribute to the development of acute infective endocarditis?
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What is a key feature of vegetations associated with acute endocarditis compared to those seen in subacute endocarditis?
What is a key feature of vegetations associated with acute endocarditis compared to those seen in subacute endocarditis?
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Which of the following is NOT a characteristic of Syndenham's Chorea?
Which of the following is NOT a characteristic of Syndenham's Chorea?
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Which organism is primarily associated with the pathogenesis of acute infective endocarditis due to its virulence and ability to proliferate rapidly?
Which organism is primarily associated with the pathogenesis of acute infective endocarditis due to its virulence and ability to proliferate rapidly?
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What type of necrosis is observed in Aschoff's nodules?
What type of necrosis is observed in Aschoff's nodules?
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What complication of infective endocarditis can result in the dislodgement of an artificial valve?
What complication of infective endocarditis can result in the dislodgement of an artificial valve?
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Which valves are most prone to developing severe lesions in the context of rheumatic fever?
Which valves are most prone to developing severe lesions in the context of rheumatic fever?
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Which symptom is most likely associated with the embolic complications of left-sided lesions in infective endocarditis?
Which symptom is most likely associated with the embolic complications of left-sided lesions in infective endocarditis?
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In an immunocompromised patient, which condition is most likely to predispose them to acute infective endocarditis?
In an immunocompromised patient, which condition is most likely to predispose them to acute infective endocarditis?
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What morphological characteristic distinguishes the vegetations seen in acute infective endocarditis?
What morphological characteristic distinguishes the vegetations seen in acute infective endocarditis?
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Which of the following statements about the morphology of vegetations in infective endocarditis is accurate?
Which of the following statements about the morphology of vegetations in infective endocarditis is accurate?
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What is a distinguishing characteristic of erythema marginatum compared to rheumatoid nodules?
What is a distinguishing characteristic of erythema marginatum compared to rheumatoid nodules?
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Which condition is least likely to cause death as a result of acute rheumatic fever?
Which condition is least likely to cause death as a result of acute rheumatic fever?
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Which statement accurately reflects the chronic rheumatic heart disease outcomes?
Which statement accurately reflects the chronic rheumatic heart disease outcomes?
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What pathological feature is associated with rheumatic heart disease?
What pathological feature is associated with rheumatic heart disease?
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Which of the following is considered a type of infective endocarditis?
Which of the following is considered a type of infective endocarditis?
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What characterizes nonbacterial thrombotic endocarditis (NBTE)?
What characterizes nonbacterial thrombotic endocarditis (NBTE)?
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In which condition would you most likely observe the presence of sterile thrombi on heart valves?
In which condition would you most likely observe the presence of sterile thrombi on heart valves?
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What common long-term consequence is associated with chronic rheumatic heart disease?
What common long-term consequence is associated with chronic rheumatic heart disease?
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Which of the following best describes the lesions associated with Libman-Sacks endocarditis?
Which of the following best describes the lesions associated with Libman-Sacks endocarditis?
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What bacterium is known as a common causative agent of subacute bacterial endocarditis?
What bacterium is known as a common causative agent of subacute bacterial endocarditis?
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What is a key factor in the pathogenesis of infective endocarditis?
What is a key factor in the pathogenesis of infective endocarditis?
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Which condition is less likely to lead to platelet thrombi formation?
Which condition is less likely to lead to platelet thrombi formation?
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What is a common microscopic finding in lesions of Libman-Sacks endocarditis?
What is a common microscopic finding in lesions of Libman-Sacks endocarditis?
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How does acute infective endocarditis differ from subacute endocarditis?
How does acute infective endocarditis differ from subacute endocarditis?
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What appearance do the vegetations of Libman-Sacks endocarditis typically have?
What appearance do the vegetations of Libman-Sacks endocarditis typically have?
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Study Notes
Rheumatic Fever and Rheumatic Heart Disease
- Rheumatic fever is an acute, immunologically mediated, post-streptococcal, multisystem inflammatory disease.
- It primarily affects the heart, joints, central nervous system, skin, and subcutaneous tissues.
- It usually occurs 1-5 weeks after a streptococcal infection (group A β-hemolytic streptococcal infection).
- The disease predominantly affects children aged 5-15 years.
- Tissue lesions in rheumatic fever are sterile, meaning they don't contain streptococci.
- Recurrence of rheumatic fever is always preceded by a streptococcal infection.
Pathogenesis
- Rheumatic fever is a post-infectious immunologic disease resulting from the immune system's reaction to streptococcal antigens.
- The body's antibodies cross-react with human tissue antigens, leading to inflammation.
- Key antigens involved include the cell wall polysaccharide of group A Streptococcus, which can cross-react with cardiac valves.
- The cell wall M protein shows cross-reactivity with sarcolemma and myosin in cardiac muscle.
- The hyaluronate capsule of group A Streptococcus is identical to human hyaluronate present in joint tissues.
- There may be autoantibodies in the brain that target basal ganglia and dopaminergic neurons.
Environmental Factors and Susceptible Hosts
- Environmental factors like overcrowding and socioeconomic status can influence the risk of rheumatic fever.
- Factors within the susceptible host, such as family history of the disease or genetic predisposition also play a significant role.
- Repeated or continuous streptococcal infections increase susceptibility to rheumatic fever.
Clinical Manifestations
- Cardiac: Pancarditis (inflammation of the endocardium, myocardium, and pericardium).
- Extracardiac: Fever, arthritis (migratory polyarthritis), arthralgia, skin lesions (erythema marginatum, subcutaneous nodules), and chorea.
Jones Criteria for Diagnosis
- To diagnose rheumatic fever, two major or one major and two minor criteria are needed.
- Major criteria: Carditis, arthritis (migratory polyarthritis), chorea, subcutaneous nodules, and erythema marginatum.
- Minor criteria: Clinical manifestations like fever, arthralgia, history of rheumatic fever or rheumatic heart disease. Laboratory findings like elevated acute-phase reactants (ESR, C-reactive protein, leukocytosis), prolonged PR interval on ECG. Supporting evidence of recent streptococcal infection (e.g., increased streptococcal antibody titers, positive throat culture for group A strep, or history of scarlet fever).
Morphology
-
Acute phase of rheumatic fever: Characterized by Aschoff nodules, which are small lesions:
- Foci of fibrinoid necrosis (immune complex reactions).
- Surrounding lymphocytes, macrophages, and plasma cells.
- "Activated" histiocytes (Anitschkow cells or Aschoff cells) with caterpillar-like appearance and central nuclei.
- Found in pericardium, myocardium, and valves.
- Eventually heal through fibrosis.
- Microscopic appearance of Aschoff bodies: Spherical or fusiform structures with central necrosis, surrounded by lymphocytes (primarily T cells), scattered plasma cells, and activated macrophages.
- Anitschkow cells have abundant cytoplasm and nuclei with centrally condensed wavy ribbon chromatin.
Pancarditis
- Pericarditis: Aschoff nodules form in the pericardium, potentially causing pericardial effusion, and serous exudate.
- Myocarditis: Aschoff nodules develop in the myocardium with interstitial oedema and mild inflammation, which can lead to left ventricular failure.
- Endocarditis: Aschoff nodules lead to irregularities in valve surfaces, with fibrin and platelet aggregations forming small vegetations ("verrucae"), often along the valve lines. Aortic and mitral valves are most susceptible to significant damage.
Extracardiac Manifestations
- Joints: Affects large joints like the knees, characteristically causing pain that subsides with aspirin.
- Syndenham's Chorea: A disorder characterized by involuntary dance-like movements, which are commonly seen in patients with rheumatic fever. Restlessness, difficulty maintaining still posture, involuntary movements, disappearing during sleep, and some emotional instability are characteristic.
Subcutaneous Nodules
- Painless nodules beneath the skin found on extensor surfaces of joints.
- Usually found on elbows or wrists.
- Typically resolve within a few days to several weeks.
Erythema Marginatum
- Flat or slightly raised, painless rash with pale centers and rounded or serpiginous margins.
- Common in children.
Chronic Rheumatic Heart Disease (RHD)
- Permanent valve deformity due to post-inflammatory fibrosis occurring 10-30 years after repeated acute RF attacks.
- 70% of cases have just mitral involvement; some have both mitral and aortic.
- Endocarditis heals through progressive fibrosis; valvular leaflets fibrosis leads to stenosis. Cordae tendonae fibrosis leads to regurgitation.
- Valves thicken and retract, often fusing and with calcium deposits.
- Fibrous bridging across valvular commissures with calcification creates "fish mouth" stenosis.
- Microscopic examination shows neovascularization and diffuse fibrosis that obliterates normal leaflet architecture.
Long-Term Outcome of RHD
- Depends on chronic valve changes' severity.
- Potential heart failure from mitral stenosis, mitral incompetence, or mixed mitral valve disease.
- Susceptibility to infective endocarditis.
- Left atrial thrombus formation can occur.
Types of Endocarditis
- Non-infective (Non-microbial): Rheumatic heart disease, Libman-Sacks endocarditis (SLE), Non-bacterial thrombotic endocarditis (NBTE).
- Infective (Microbial): Primarily bacterial or fungal.
Non-bacterial Thrombotic Endocarditis (NBTE)
- Characterized by the deposition of small, sterile thrombi on cardiac valve leaflets.
- Occurs more commonly in mitral valves in debilitated patients (cancer or sepsis), often in those with mucinous adenocarcinomas.
- Sterile, bland, non-destructive vegetations.
- Doesn't cause tissue destruction nor show inflammation.
Endocarditis of Systemic Lupus Erythematosus (Libman-Sacks Endocarditis)
- Occurs in approximately 10% of patients with lupus.
- Lesions from immune complex deposition.
- Affects mitral and tricuspid valves, as small (1-4 mm), sterile vegetations.
- Appear warty (verrucous) in appearance.
- Microscopically, composed of fibrin and platelet thrombi with fibrinoid necrosis. Capillaries and infiltration by histiocytes, plasma cells, lymphocytes, and neutrophils occur in the endocardium.
Infective Endocarditis (IE)
- Microbial infection affecting heart valves or mural endocardium.
- Leads to vegetation formation (thrombotic debris and microorganisms), and often destroys underlying cardiac tissue.
- Classified into acute (ABE) and subacute (SABE) forms.
IE Pathogenesis
- Bacteria entering the bloodstream implant on cardiac valves or endocardium due to surface adhesion molecules.
- Bacteria preferentially lodge on damaged valves (especially in RHD and congenital heart disease) over healthy ones
- Conditions causing stress to valves lead to endothelial damage, favoring platelet thrombi formation, which then get infected by circulating bacteria.
Sub-acute Bacterial Endocarditis
- Damage to the pre-existing valve leads to platelet-fibrin deposits and thrombotic vegetations.
- Bacteria invade thrombotic vegetations and slowly proliferate causing gradual valve destruction.
- Poorly virulent causative agents like Streptococcus viridans.
Acute Bacterial Endocarditis
- Patients with normal valves affected by highly virulent causative agents (e.g., Staphylococcus aureus).
- Bacteria enter the bloodstream resulting in rapid proliferation of organisms on heart valve leading to necrosis and valve destruction.
- Rapidly forming vegetations depend on both virulence of the organism and the size of the bacterial invasion. Host immunity plays a role as well.
Risk Factors for IE
- Rheumatic heart disease, congenital heart malformations, other cardiac malformations, and various other heart conditions.
- Iatrogenic factors like prosthetic valves, indwelling catheters; immunocompromised patients.
- Infections like neutropenia, AIDS or, IV drug use.
IE Morphology
-
Macroscopic: Friable (easily disintegrating), bulky vegetations that are bacteria laden.
- Vegetations can be single or multiple and range in size from millimeters to centimeters.
- Microscopic: Acute endocarditis vegetations tend to be bulkier, can cause perforation or erosion of the valve leaflet, and potentially spread to myocardial abscesses. Subacute endocarditis vegetations tend to be smaller, don't usually cause perforation, and extend to adjacent mural endocardium. IE shows mixed inflammatory cells and colonies of bacteria.
IE Complications
- Cardiac: Valvular insufficiency, myocardial abscess, perforation of IV septum, suppurative pericarditis.
- Embolic: Left-sided lesions (brain, spleen, kidneys, limbs), and less commonly mycotic aneurysms in vessels. Right-sided lesions can affect the lung. Artificial valve dislodgement also a risk.
IE Diagnosis
- Clinical suspicion based on known risk factors, unexplained fever, septicemia, heart failure, and embolic infarcts.
- Investigations include raised ESR, white blood cell count, normochromic, normocytic anaemia, and blood cultures to identify the causative organism (if no antibiotics are being taken).
- Echocardiogram may show vegetation.
Major Forms of Vegetative Endocarditis
- Information on the location, pathogens, and microscopic/pathological features of different endocarditis types.
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Test your knowledge about rheumatic fever and related cardiac conditions in this quiz. Answer questions about its pathology, manifestations, age groups affected, and diagnostic features. Ideal for medical students and healthcare professionals looking to reinforce their understanding of these topics.