Cvs rhd-csbrp
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This document discusses rheumatic fever and rheumatic heart disease. It begins by describing rheumatic fever as an acute, immunologically mediated disease that occurs weeks after a Group A Streptococcal infection. It can lead to chronic rheumatic heart disease and valve damage. The document then covers the pathogenesis of rheumatic fever, its clinical manifestations like polyarthritis and Sydenham's chorea, its diagnosis using the Jones criteria, and the morphological features of both acute rheumatic fever and chronic rheumatic heart disease like Aschoff bodies. It concludes by describing the complications of rheumatic heart disease such as valve problems, heart failure, and arrhythmias.
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Cvs rhd-csbrp
- 1. May-2015-CSBRP Rheumatic Fever and Rheumatic Heart Disease CSBR.Prasad, MD.,
- 2. • Arthritis • Arthralgia • Types of Streptococci • What is beta hemolysis? • Markers for Streptococcal infection • What are the diseases caused by Streptococci ? • When do you clinically suspect pericarditis / pleurisy? • How to differentiate these two? May-2015-CSBRP
- 3. Diseases caused by Streptococcus • Pneumonia • Necrotizing fasciitis • Rheumatic fever • Poststreptococcal glomerulonephritis • Pharyngitis / tonsillitis • Neonatal meningitis (Group-B) • PANDAS / Tourette syndrome : Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infections May-2015-CSBRP
- 6. May-2015-CSBRP Rheumatic fever (RF) • It is an acute, immunologically mediated disease • Occur a few weeks after group A Streptococcal pharyngitis • Multisystemic disorder • May progress to chronic RHD (Valvular heart disease)
- 7. May-2015-CSBRP Rheumatic fever (RF) • It is an acute, immunologically mediated disease • Occur a few weeks after group A Streptococcal pharyngitis • Streptococcus strains: 1,3,5,6 & 18 [Griffith type]
- 8. May-2015-CSBRP Pathogenesis “Damage is mediated both by Abs and T-cells”
- 9. May-2015-CSBRP MORPHOLOGY Acute RF • Aschoff bodies • Pancraditis • Verrucous vegetations • MacCallum plaques Chronic RHD • Valvular changes – Leaflet thickening, – Commissural fusion and shortening, and – Thickening and fusion of the tendinous cords
- 10. May-2015-CSBRP RHD Valves affected are: decreasing order – Mitral – Aortic – Tricuspid – Pulmonary RHD is virtually the only cause of mitral stenosis Mnemonic: MAT
- 11. May-2015-CSBRP Clinical Features RF is characterized by: – Migratory polyarthritis of the large joints – Pancarditis – Subcutaneous nodules – Erythema marginatum – Sydenham’s chorea The diagnosis of RF is established by the “Jones criteria”
- 12. “Jones criteriaJones criteria” Required Criteria Evidence of antecedent Strep infection: ASO / Strep antibodies / Strep group A throat culture / Recent scarlet fever / anti-deoxyribonuclease B / anti-hyaluronidase Major Diagnostic Criteria Carditis Polyarthritis Chorea Erythema marginatum Subcutaneous Nodules Minor Diagnostic Criteria Fever Arthralgia Previous rheumatic fever or rheumatic heart disease Acute phase reactions: ESR / CRP / Leukocytosis Prolonged PR interval May-2015-CSBRP
- 13. “Jones criteriaJones criteria” Diagnostic : 1 Required Criteria and 2 Major Criteria and 0 Minor Criteria Diagnostic : 1 Required Criteria and 1 Major Criteria and 2 Minor Criteria May-2015-CSBRP
- 14. May-2015-CSBRP “Jones criteriaJones criteria” • Evidence of a preceding group A streptococcal infection + • Two of the major manifestations or • One major and two minor manifestations
- 15. May-2015-CSBRP
- 16. May-2015-CSBRP Rheumatic Heart Disease: Sreptococcal pharyngitis / tonsillitis
- 19. Sydenham's chorea: causes loss of muscle control, leading to awkward gait and distorted hand gestures May-2015-CSBRP
- 20. May-2015-CSBRP Acute RF • Appears 10 days to 6 weeks after a group A Streptococcal infection • Children between ages 5 -15yrs • Pharyngeal cultures for streptococci are negative • Indirect evidence of Streptococcal infection: – ASLO – DNase B
- 21. May-2015-CSBRP Acute RF • The predominant clinical manifestations are: – Carditis and – Arthritis • Arthritis: – More common in adults than in children – Migratory polyarthritis • “Acute carditis”: – Pericardial friction rubs – Tachycardia, and – Arrhythmias • Myocarditis: – Cardiac dilation with functional MR or – Heart failure • Approximately 1% of affected individuals die of fulminant RF involvement of the heart
- 22. RHD - Microscopy • Characteristic feature of RHD is Aschoff’s body • Aschoff’s body composed of: – Swollen eosinophilic collagen – T-cells – Plasma cells – Plump macrophages – Anitschkow cells – They are perivascular in location May-2015-CSBRP
- 25. May-2015-CSBRP
- 26. May-2015-CSBRP Catterpillar chromatin in nuclei
- 27. May-2015-CSBRP Catterpillar chromatin in nuclei
- 28. May-2015-CSBRP Aschoff’s body – perivascular in location
- 29. Fibrinous pericarditis “Bread and butter” pericarditis May-2015-CSBRP
- 31. Fibrinous pericarditis “Bread and butter” pericarditis May-2015-CSBRP
- 32. RHD - Microscopy • Fibrinoid necrosis: seen in the endocardium, cusps, along the tendinous cords • Vegetations: Small projections on the lines of closure • MacCollum’s patches: Irregular thickening in the left atrial wall in the presence of MR May-2015-CSBRP
- 33. May-2015-CSBRP Gross appearance of heart showing dilated left atrium with MacCallum plaque and vegetations
- 34. May-2015-CSBRP Gross appearance of heart showing dilated left atrium with MacCallum plaque and vegetations
- 36. May-2015-CSBRP Acute RF • After an initial attack there is increased vulnerability to reactivation of the disease with subsequent pharyngeal infections • Damage to the valves is cumulative • Clinical manifestations appear years or even decades after the initial episode of RF
- 37. Chronic RHD • Characterized by organization of acute inflammation and subsequent fibrosis • Valves show thickening, commissural fusion and shortening, • Cordae tendinae shows thickening and shortening • Mitral valve: MS [Button hole, Fish mouth] May-2015-CSBRP
- 38. May-2015-CSBRP Mitral valve: MS [Button hole, Fish mouth]
- 39. May-2015-CSBRP Rheumatic mitral stenosis • “Fish mouth” or “Button hole” stenoses • Left atrial enlargement • Mural thrombi in left atrium • Long standing MS: pulmonary vascular and parenchymal changes > RVH • Valves: – Organization of the acute inflammation – Neovascularization and – Transmural fibrosis
- 40. May-2015-CSBRP Acute and chronic rheumatic heart disease
- 41. May-2015-CSBRP Mitral valve: MS [Button hole, Fish mouth]
- 42. May-2015-CSBRP Rheumatic heart disease (shortening and thickening of chordae)
- 43. May-2015-CSBRP Complications • Cardiac murmurs • Cardiac hypertrophy and dilation • Valvular heart disease • Heart failure • Arrhythmias (particularly AF in the setting of mitral stenosis) • Thromboembolic complications • Infective endocarditis
- 44. May-2015-CSBRP Rupture of chordae tendinae
- 45. May-2015-CSBRP Mural Thrombus in the left atrium
- 46. Rheumatic fever: “Licks the joints and Bites the heart” May-2015-CSBRP
- 48. Sydenham’s Chorea • Extrapyramidal disorder: • Fast, clonic involuntary movements (especially face and limbs) • Muscular hypotonus • Emotional lability • First sign: difficulty walking, talking, writing • Usually a late manifestation, can be months after infection • May be the only manifestation of ARF • Often associated with carditis • Usually benign and resolves in 2-3 months • But can last for more than 2 years May-2015-CSBRP
- 49. May-2015-CSBRP • marantic endocarditis is a/w... • hypercoagulable states; involves deposition of fibrin and platelets on leaflets of cardiac valves
- 50. May-2015-CSBRP • this endocarditis has vegetations on both sides of the valve surface • libman-sacks endocarditis (a/w lupus)
- 51. May-2015-CSBRP • these vegetations have fibrinoid necrosis and inflammation and are located on both sides of the valve surface • libman-sacks endocarditis (lupus)
Editor's Notes
- Rheumatic fever (RF) is an acute, immunologically mediated, multisystem inflammatory disease classically occurring a few weeks after an episode of group A streptococcal pharyngitis; occasionally, RF can follow streptococcal infections at other sites, such as the skin. Acute rheumatic carditis is a common manifestation of active RF and may progress over time to chronic rheumatic heart disease (RHD), mainly manifesting as valvular abnormalities. Valvular heart disease. RHD is characterized principally by deforming fibrotic valvular disease, particularly involving the mitral valve; indeed, RHD is virtually the only cause of mitral stenosis. The incidence and mortality rate of RF and RHD have declined remarkably in many parts of the world over the past century, as a result of improved sanitation, and rapid diagnosis and treatment of streptococcal pharyngitis. Nevertheless, in developing countries, and in many crowded, economically depressed urban areas, RHD remains an important public health problem, affecting an estimated 15 million people.
- Rheumatic fever (RF) is an acute, immunologically mediated, multisystem inflammatory disease classically occurring a few weeks after an episode of group A streptococcal pharyngitis; occasionally, RF can follow streptococcal infections at other sites, such as the skin. Acute rheumatic carditis is a common manifestation of active RF and may progress over time to chronic rheumatic heart disease (RHD), mainly manifesting as valvular abnormalities. Valvular heart disease. RHD is characterized principally by deforming fibrotic valvular disease, particularly involving the mitral valve; indeed, RHD is virtually the only cause of mitral stenosis. The incidence and mortality rate of RF and RHD have declined remarkably in many parts of the world over the past century, as a result of improved sanitation, and rapid diagnosis and treatment of streptococcal pharyngitis. Nevertheless, in developing countries, and in many crowded, economically depressed urban areas, RHD remains an important public health problem, affecting an estimated 15 million people.
- Vegetation is assemblages of plant species and the ground cover they provide. Vegetations in RHD too resemble this hence the name.
- Rheumatic fever (RF) is an acute, immunologically mediated, multisystem inflammatory disease classically occurring a few weeks after an episode of group A streptococcal pharyngitis; occasionally, RF can follow streptococcal infections at other sites, such as the skin. Acute rheumatic carditis is a common manifestation of active RF and may progress over time to chronic rheumatic heart disease (RHD), mainly manifesting as valvular abnormalities. Valvular heart disease. RHD is characterized principally by deforming fibrotic valvular disease, particularly involving the mitral valve; indeed, RHD is virtually the only cause of mitral stenosis. The incidence and mortality rate of RF and RHD have declined remarkably in many parts of the world over the past century, as a result of improved sanitation, and rapid diagnosis and treatment of streptococcal pharyngitis. Nevertheless, in developing countries, and in many crowded, economically depressed urban areas, RHD remains an important public health problem, affecting an estimated 15 million people.
- Carditis: All the layers of the heart namely, epicardium, myocardium and endocardium are involved so, it’s pancarditis. Pericardium is also involved and shows fibrinous pericarditis so called “bread and butter pericarditis”. Alvualr involvement is very common in acute rheumatic heart disease, the most common vulvar lesion is MR and in chronic RHD it’s MS. Migratory polyarthritis: There is involvement of large jpoints. It;’s more commonly seen in adults than children. Arthritis involves one joint after the other (migratory) and subsides spontaneously with out residual deformity (non-erosive arthritis). The pain dramatically responds to salicylates. Subcutaneous nodules: these are painless subcutaneous nodules found on the extensor surfaces of ELBOW, SHIN and OCCIPUT. Erythema marginatum: There is presence of red macular rash more easily appreciated in fair skinned individuals sparing the face and without residual scarring. Sydenham’s chorea: It’s a late manifestation of the disease characterized by presence of involuntary, purposeless movements associated with emotional lability of the patient. ------------------------------------- REF: Special Writing Group of the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council on Cardiovascular Disease in the Young of the American Heart Association. Guidelines for the diagnosis of rheumatic fever. Jones Criteria, 1992 update. JAMA. 1992 Oct 21;268(15):2069-73. Erratum in: JAMA 1993 Jan 27;269(4):476.
- Erythema marginatum: Highly specific to ARF Cutaneous lesion: reddish pink border, pale centre, round or irregular shape Often on trunk, abdomen, inner arms or thighs Highly suggestive of carditis.
- Subcutaneous nodules: Usually 0.5-2cm in diameter: Firm, non-tender, isolated or in clusters Most common along on the extensor surfaces of the joint: knees, elbows, wrist Also on bony prominences, tendons, dorsum of feet, occiput or cervical spine. Last a few days only Often associated with carditis
- Sydenham's chorea (St.Vitus dance) causes loss of muscle control, leading to awkward gait and distorted hand gestures. Other disorders that may be accompanied by chorea include abetalipoproteinemia, ataxia-telangiectasia, Fahr disease, glutaric aciduria, Wilson disease, Lesch-Nyhan syndrome, hyperthyroidism, lupus erythematosus, pregnancy (Chorea gravidarum), and certain anticonvulsants or psychotropic agents.
- Anitschkow cells: These distinctive cells have abundant cytoplasm and central round to oval nculei in which the chromatin is dispersed in a central, slender, wavy ribbon – Hence the name “CATTER PILLAR CELLS”
- A window of adherent pericardium has been opened to reveal the surface of the heart. There are thin strands of fibrinous exudate that extend from the epicardial surface to the pericarial sac. This is typical for a fibrinous pericarditis.
- Microscopically, the pericardial surface here shows strands of pink fibrin extending outward. There is underlying inflammation. Eventually, the fibrin can be organized and cleared, though sometimes adhesions may remain.
- Anitschkow cells: These distinctive cells have abundant cytoplasm and central round to oval nuclei in which the chromatin is dispersed in a central, slender, wavy ribbon – Hence the name “CATTER PILLAR CELLS” MacCollum’s patches: Irregular thickening in the left atrial wall in the presence of MR. indicative of exaggerated response of the Aschoff body at the site regurgitant jet injury.
- On opening the pleural and pericardial cavities, about one liter of pus was drained. The heart weighed 282 g. The left atrium showed dilatation, with a map-like thickened, roughened, and wrinkled area in the posteromedial surface [Figure 1], which was subsequently identified as a MacCallum plaque The mitral valve showed narrowing and diffuse thickening, with a fish- mouth appearance, and multiple vegetations hanging into the left ventricle. Tendinous cords showed shortening, thickening, and fusion. The tricuspid and aortic valves were unremarkable. The left ventricle wall showed thickening. Photomicrograph of the adjacent areas of the MacCallum plaque showing mixed inflammatory cell infiltrate, with focal areas of interstitial edema and neovascularization (H and E, 10× magnifications)
- On opening the pleural and pericardial cavities, about one liter of pus was drained. The heart weighed 282 g. The left atrium showed dilatation, with a map-like thickened, roughened, and wrinkled area in the posteromedial surface [Figure 1], which was subsequently identified as a MacCallum plaque The mitral valve showed narrowing and diffuse thickening, with a fish- mouth appearance, and multiple vegetations hanging into the left ventricle. Tendinous cords showed shortening, thickening, and fusion. The tricuspid and aortic valves were unremarkable. The left ventricle wall showed thickening. Photomicrograph of the adjacent areas of the MacCallum plaque showing mixed inflammatory cell infiltrate, with focal areas of interstitial edema and neovascularization (H and E, 10× magnifications)
- In rheumatic mitral stenosis, calcification and fibrous bridging across the valvular commissures create “fish mouth” or “buttonhole” stenoses. With tight mitral stenosis, the left atrium progressively dilates and may harbor mural thrombi that can embolize. Long-standing congestive changes in the lungs may induce pulmonary vascular and parenchymal changes; over time, these can lead to right ventricular hypertrophy. The left ventricle is largely unaffected by isolated pure mitral stenosis. Microscopically, valves show organization of the acute inflammation, with post-inflammatory neovascularization and transmural fibrosis that obliterate the leaflet architecture. Aschoff bodies are rarely seen in surgical specimens or autopsy tissue from patients with chronic RHD, as a result of the long intervals between the initial insult and the development of the chronic deformity.
- Figure 12-23 Acute and chronic rheumatic heart disease. A, Acute rheumatic mitral valvulitis superimposed on chronic rheumatic heart disease. Small vegetations (verrucae) are visible along the line of closure of the mitral valve leaflet (arrows). Previous episodes of rheumatic valvulitis have caused fibrous thickening and fusion of the chordae tendineae. B, Microscopic appearance of an Aschoff body in a patient with acute rheumatic carditis. The myocardium exhibits a circumscribed nodule of mixed mononuclear inflammatory cells with associated necrosis; within the inflammation, large activated macrophages show prominent nucleoli, as well as chromatin condensed into long, wavy ribbons (caterpillar cells; arrows). C and D, Mitral stenosis with diffuse fibrous thickening and distortion of the valve leaflets and commissural fusion (arrows, C), and thickening of the chordae tendineae (D). Note neovascularization of anterior mitral leaflet (arrow, D). E, Surgically resected specimen of rheumatic aortic stenosis, demonstrating thickening and distortion of the cusps with commissural fusion. (E, Reproduced from Schoen FJ, St. John-Sutton M: Contemporary issues in the pathology of valvular heart disease. Hum Pathol 18:568, 1967.)
- The heart has been sectioned to reveal the mitral valve as seen from above in the left atrium. The mitral valve demonstrates the typical "fish mouth" shape with chronic rheumatic scarring. Mitral valve is most often affected with rheumatic heart disease, followed by mitral and aortic together, then aortic alone, then mitral, aortic, and tricuspid together.
- what is a McCallum's patch? ---- Inflammation of the endocardium in acute rheumatic heart disease so you can see underlying (brown) myocardium
- Sydenham’s Chorea Extrapyramidal disorder: Fast, clonic involuntary movements (especially face and limbs) Muscular hypotonus Emotional lability First sign: difficulty walking, talking, writing Usually a late manifestation, can be months after infection May be the only manifestation of ARF Often associated with carditis Usually benign and resolves in 2-3 months But can last for more than 2 years