89. Rheumatic Fever and Rheumatic Heart Disease (RHD)
90. Acute Pericarditis
91. Myocarditis
92. Infective Endocarditis (IE)
93. Congenital Heart Disease
94. Cardiomyopathies
95. Arteriosclerosis
96. Atherosclerosis
97. Inflammatory Disease of Blood Vessels
98. Aneurysms and Dissection
99. Congestive Heart Failure
100. Iron Deficiency Anaemia
101. Megaloblastic anaemia
102. Pancytopenia
103. Leucocytosis and Leucopenia
104. Aplastic anaemia
105. Haemolytic anaemia
106. Hereditary Spherocytosis
107. Haemoglobinipathies
108. Thalassemia syndrome
109. Sickle Cell Disease
110. Leukaemia
111. Leukemoid reaction
112. Lymphadenitits
113. Hodgkin lymphoma
114. Non-hodgkin lymphoma
115. Myeloproliferative disorders
116. Myelofibrosis
117. Multiple myeloma
118. Bleeding disorders
119. Coagulation disorders
120. any
121. Blood grouping
122
Microbiology
122. Introduction of Blood borne infections
123. Infective Endocarditis
124. Brucella
125. Rickettsiae
126. Leishmania donovani
127. Plasmodium
128. Wuchereria bancrofti
129
Biochemistry
129. Metabolism in Blood Cells
130. Iron metabolism
131. Haemoglobin
132. Lipoprotein metabolism
133. Biochemical aspect of MI
134
Pathology
89. Rheumatic Fever and Rheumatic Heart Disease (RHD)
1.
Write short notes on: (a) Morphology of Rheumatic Heart Disease.
[2072]
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2.
Describe the microscopic features of rheumatic heart disease.
[2059]
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3.
Describe the gross and microscopic features of rheumatic carditis. List out its complications.
(4)
[2055]
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RHEUMATIC FEVER
Introduction
Definition
Rheumatic fever (RF) is an acute, immunologically mediated, multisystem inflammatory disease classically occurring a few weeks after an episode of group A streptococcal pharyngitis.
Occurs in children between 5 and 15 years.
Pathogenesis of Rheumatic Fever
graph TD
1["Pharyngitis by group-A, β hemolytic <i>Streptococcus</i>."]
2["M<sub>1</sub> protein of <i> Streptococcus pyogenes</i> released in circulation."]
3["Symptoms of pharyngitis subside in 97%."]
4["In 0.3 to 3% people, after 3-5 weeks development of <br>antibodies against M<sub>1</sub> protein by host immune system."]
5["Molecular similarity between M<sub>1</sub>-protein and human cell membrane,<br> hence antibodies directed against M<sub>1</sub>-protein cross react with <br>glycoprotein antigens in Brain, Heart, Joint, Skins and Subcutaneous nodules."]
6["Rheumatic fever"]
7["On second attack, with same bacteria, reactivation of immune system."]
8["Cross reaction takes place"]
9["Continuous progressive damage to heart result<br> in permanent damage to heart valves and myocardium."]
10["Rheumatic Heart Disease"]
1 --> 2
2 --> 3
3 --> 4
4 --> 5
5 --> 6
6 --> 7
7 --> 8
8 --> 9
9 --> 10
Clinical features
M1-protein in bacterial surface resembles the following proteins of human body and thus affects these organs whose symptoms becomes the diagnostic criteria of John's criteria.
Lyso gangliosides of Brain
Keratin of Skin
Vimentin of Joint
Tropomyosin of Endocardium
Laminin of Heart valves
John's criteria
Evidence of a preceding group A Streptococcus infection + (2 major or (1 major + 2 minor criteria))
Major criteria
Brain
Syndenham's chorea (Saint Vitus dance)
Skin
Erythema marginatum
Joint
Migratory polyarthritis
Subcutaneous nodules
Enlarged
Heart
Pancarditis
Minor criteria
High fever
History of Rheumatic fever
Leukocytosis
Increased ESR/ CRP
Prolonged PR interval
Arthralgia (Joint pain)
RHEUMATIC HEART DISEASE
Definition
Rheumatic Heart Disease is a condition in which permanent damage occurs to heart as a consequence of rheumatic fever.
Classification of Rheumatic Fever
Acute Rheumatic Heart Disease
Morphology
Acute rheumatic carditis is manifested in all three layers of heart so called Pancarditis.
Acute rheumatic pericarditis
Gross
Fibrinous and serofibrinous exudate in pericardial sac, bread butter appearance.
Microscopic
Fibrin on surface
Infiltration by lymphocytes, plasma cells, histiocytes and few neutrophils.
Acute rheumatic myocarditis
Gross
Early stage: Myocardium of especially left ventricle become soft and flabby.
Intermediate stage: Interstitial tissue of the myocardium show small foci of necrosis.
Late stage: Tiny pale foci of Aschoff bodies throughout the myocardium.
Microscopic
Aschoff bodies, a Distinctive lesion, are found which consists of following
Foci of lymphocytes (primarily T-cells)
Occasional plasma cells
Anitschkow cells
Plump activated macrophages
Abundant cytoplasm
Central round to ovoid nuclei in which the chromatin is disposed in a central, slender, wavy ribbon hence called as Caterpillar cells.
May become multinucleated i.e., Aschoff giant cells.
Anitschkow cell is pathognomonic for Rheumatic fever.
Acute rheumatic endocarditis
Acute rheumatic valvulitis
Gross
Fibrinoid necrosis within the cusps or tendinous cords.
Overlying these necrotic foci and along the lines of closure are small (1 to 2 mm) vegetations, called Verrucae.
Microscopic
Acute rheumatic submural endocarditis
Gross
Subendocardial lesions, perhaps exacerbated by regurgitant jets, can induce irregular thickenings called MacCallum plaques, usually in left atrium.
Microscopic
Chronic Rheumatic Heart Disease
Morphology
Valves
◉ Involved valves:
Mitral valve is almost always affected.
Mitral valve in isolation in 75%
Mitral valve + Aortic valve in 25%
Tricuspid and pulmonary valve is rarely affected.
◉ Changes in Mitral valve:
◈ Thickening of leaflet: Due to fibrosis
◈ Fusion and shortening of Commissures: The valve orifice becomes reduced to a fixed narrow opening that gives the characteristic appearance of Fish mouth (or Button hole)
◈ Thickening and fusion of the chordae tendinae:
Dilation of left atrium with mural thrombi
Long standing stenosis may lead to right ventricular hypertrophy
