Rheumatic fever is an inflammatory disease that occurs as a result of a prior streptococcal infection. It commonly affects children between 5-15 years old. The major systems involved are the heart, joints, skin, and brain. In the heart, it can cause inflammation of the tissues and valves, leading to scarring and deformities over time. On pathology, it is characterized by lesions called Aschoff bodies in the heart muscle. Long term, it can progress to chronic rheumatic heart disease with permanent valve damage and heart failure. Prompt treatment of streptococcal infections can help prevent recurrent cases of 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.