Rheumatic fever

1. RHEUMATIC FEVER
/RHEUMATIC HEART
DISEASE

2. Acute , immunologically mediated, multisystem
inflammatory disease.
Occurs 10 days to 6 weeks after an episode of
group A streptococcal pharyngitis.
Affects usually children (5-15 yrs) but 20% cases
can be seen in adults.
Active phase of RF may progress to chronic
RHD.
RF does not occur after a streptococcal inf at
other sites eg skin.

3. Is an important public health problem in
poor socioeconomic localities
Incidence has < due to better living
standards, rapid diagnosis, good drug
therapy & unexplained < in virulence of gp
A streptococci

4. Systems involved are:
- Heart esp cardiac valves, endocardium.
- Joints esp large ones (migratory
polyarthritis).
- Skin (erythema marginatum).
- Subcutaneous nodules.
- Neurological disorder called SYNDENHAM
CHOREA (involuntary purposeless rapid
movements).

5. Morphology
HEART LESIONS:
- Acute RF: characterized by distinctive lesions
called ASCHOFF BODIES in the myocardial
interstitium.
- They are circumscribed lesions consisting of a
central focus of necrotic,swollen eosinophilic
collagen surrounded by T-lymphocytes,
occasional plasma cells & plump macrophages
called Anitschkow cells (abundant cytoplasm
central round to ovoid nuclei with chromatin
disposed in a central, slender, wavy ribbon).
- also called caterpillar cells - pathognomonic for
RF.
- some large macrophages become multinucleated
Aschoff Giant Cells.

6. During acute RF, diffuse inflammation &
Aschoff bodies may be found in all 3
layers of heart (PANCARDITIS)
PERICARDIAL LESIONS:
Fibrinous or serofibrinous pericardial
exudate called “bread & butter”
pericarditis.
This generally resolves without any
sequelae.

7. MYOCARDIAL LESIONS:
- Myocarditis
Scattered Aschoff bodies within the interstitial
connective tissue
ENDOCARDIAL LESIONS:
Simultaneous involvement of endocardium &
left sided cardiac valves by inflammatory foci
causes fibrinoid necrosis of cusps or chordae
tendinae.

8. Small 1-2 mm vegetations called “verruca”
situated along the line of closure of the valves.
They are irregular & wart-like
Cause little disturbance in cardiac function
Formed due to precipitation of fibrin & collagen
degeneration at sites of closure
Left atrium shows irregular, subendocardial
lesions called MacCallum plaques

9. PATHOGENESIS
Acute RF is a hypersensitivity reaction
caused by gp A Streptococci
Antibodies against M protein of certain
strains of streptococci cross-react with
glycoprotein antigens in the heart, joints &
other tissues
Streptococcal infection evokes an
autoimmune response against self-
antigens
Genetic suseptibility also plays a major
role in pathogenesis of this disease

10. Group A streptococci elaborate the
cytolytic toxins streptolysins S and O.
Streptolysin O induces persistently
high antibody titers that provide a useful
marker of group A streptococcal
infection and its nonsuppurative
complications (ASO Ab test).

11. Rheumatogenic strains often are
encapsulated mucoid strains rich in M
proteins and resistant to phagocytosis.
The presence of the M protein in the cell
wall is the most important virulence factor for
group A streptococcal infection in humans.

12. CHRONIC RHD
Characterized by organization of acute
inflammation & fibrosis
Permanent valvular deformity (esp mitral valve)
due to retraction & thickening of leaflets
Mitral & tricuspid valves show leaflet thickening,
commissural fusion, shortening, thickening &
fusion of tendinous cords

13. VALVULAR LESIONS of CH.RHD
Mitral valve alone is affected in 65-70%
cases of RHD.
RHD is responsible for 99% cases of
mitral stenosis
Concomitant involvement of mitral & aortic
valve is seen in 25% cases
Less severe damage may also occur in
the tricuspid & pulmonary valves

14. Fibrous bridging & calcification across
valvular commissures creates “fish
mouth” or “buttonhole” stenosis.
Mitral stenosis causes progressive left
atrial dilatation, harbouring a mural
thrombus in its appendage or along its
wall.
Lungs show congestive changes
ultimately leading to rt ventricular
hypertrophy.
Left ventricle is essentially normal with
isolated mitral stenosis

15. Microscopy (RHD)
Diffuse & dense fibrosis.
Neovascularization of valves.
Aschoff bodies are replaced by fibrous
scar therefore not seen in autopsy
specimens of ch CHD

16. Diagnosis of ARF
JONES CRITERIA:-
Clinical features &
Lab investigations

17. Major Criteria (JONES)
1.Migratory polyarthritis of large joints
2.Carditis
3.Subcutaneous nodules
4.Erythema marginatum of skin
5.Syndenham chorea

18. JONES (Major Criteria)
J =Joints (migratory polyarthritis).
O=(imagine heart shape) Carditis.
N=Nodules (s/c nodules), painless collections of
collagen fibres on back of wrists, front of knees.
E= Erythema marginatum (long lasting rash that
begins on trunk or arms.)
S= Syndenhams chorea ( rapid purposeless
movements of limbs & face.)

19. CANCER (Major Jones
Criteria)
C=Carditis
A= Arthritis
N=Nodules
C=Chorea
ER=ERythema marginatum.

20. Minor Criteria (clinical features &
Lab investigations)
Nonspecific signs & symptoms:
1.Fever
2.Arthralgias - jt pain without swelling.
3.Lab abn -↑ESR, ↑CRP, ↑WBC.
4.ECG- prolonged PR interval.
5.Previous rheumatic fever.
6.Evidence of gpA Streptococcal inf:-
+ culture
↑ ASO titre

21. DIAGNOSIS
Jones criteria:-
***TWO MAJOR MANIFESTATIONS
OR
***ONE MAJOR & TWO MINOR CRITERIA
in order to establish the diagnosis.

22. Clinical features: arthritis & carditis
- arthritis is more common in adults than
children
-begins as migratory polyarthritis
accompanied with fever
One large joint is involved after another
becomes painful & swollen for some days
Subsides spontaneously
Leaves no residual deformity

23. Carditis: pancarditis
Pericardial friction rubs
Weak heart sounds
Tachycardia & arrhythmias
New heart murmurs.
other clinical features include epistaxis &
abdominal pain.

24. Myocarditis may cause cardiac dilatation
leading to mitral valve insufficiency or
even heart failure.
PROGNOSIS
In cases of primary attack is excellent with
only 1% cases dying from fulminant RF

25. COMPLICATIONS
RECURRENCES are liable to occur after
each subsequent pharnygeal attack with
similar clinical manifestations.
CARDITIS TENDS TO MORE SEVERE in
recurrent episodes.
EMBOLIZATION from mural thrombi
within atria or their appendages.
INFECTIVE ENDOCARDITIS
superimposed on deformed valves.

26. Ch rheumatic carditis does not cause
clinical manifestations for years or even
decades after the initial episode of RF
CARDIAC MURMURS
CARDIAC HYPERTROPHY &
DILATATION
HEART FAILURE
ARRHYTHMIAS LIKE ATRIAL
FIBRILLATION

27. Treatment
Surgical repair by incising the diseased
mitral stenotic valve commissures &
replacement by prosthetic devices has
greatly improved the outcome of this
disease.

28. Rheumatic fever- vegetations
(verrucae)

29. Acute Rheumatic Fever: small verrucous
vegetations on line of closure of mitral valve

30. Rheumatic fever vegetations

31. Ch.rheumatic valvulitis affecting the mitral valve & dev due to organization &
fibrosis of ac endocardial inflammation. Note the shortened & thickened
chordae tendinae

32. Chronic rheumatic scarring: fish mouth
deformity of mitral valve

33. AORTIC STENOSIS (RHEUMATIC FEVER)

34. RHEUMATIC NODULES ON BACK

35. RHEUMATIC NODULES

36. Erythema marginatum

37. Ac rheumatic carditis: ASCHOFF NODULES best
seen in myocardial interstitium & centered around
a b.v.

38. L/M: ASCHOFF NODULE COMPOSED OF
GIANT CELLS & MONONUCLEAR CELLS

39. AC.RHEUMATIC CARDITIS SHOWING A PECULIAR CELL CALLED
ANITSCHOW MYOCYTE :ELONGATED THIN CELL WITH A THIN
ELONGATED NUCLEUS (CATERPILLAR CELL).

41. Aschoff nodule in myocardium

42. Rheumatic myocarditis (aschoff nodule)

43. ACUTE RH.HEART DISEASE
Fig 5-6. Rheumatic Heart Disease: A: In acute rheumatic heart disease. There is necrosis and
an associated inflammatory reaction in the myocardial interstitium.

44. RH.HEART DISEASE
Fig 5-6. Rheumatic Heart Disease: B: The Aschoff body is pathognomonic for rheumatic
heart disease and is composed of a necrotic focus infiltrated by mononuclear and
multinucleated giant cells, Anitschkow cells.

45. RH.HEART DISEASE
Fig. 5-6. Rheumatic Heart Disease: C: Healed rheumatic valvulitis is characterized by blood vessels
proliferation and an associated inflammatory reaction.