Rheumatic FeverRheumatic Fever

Normal Heart AnatomyNormal Heart Anatomy

Rheumatic Fever (RF)Rheumatic Fever (RF)

Definition:Definition: Rheumatic fever (RF) is an Rheumatic fever (RF) is an

autoimmune disease affecting the autoimmune disease affecting the heart and extra- cardiac sites (joints, heart and extra- cardiac sites (joints, brain, skin and others)brain, skin and others)

The incidence of RF has been The incidence of RF has been lowered in the developed countries lowered in the developed countries but is still high in poor communitiesbut is still high in poor communities

The disease affects children and The disease affects children and young adults (5-15years)young adults (5-15years)

The disease follows upper respiratory The disease follows upper respiratory infection (tonsillitis) with Group A infection (tonsillitis) with Group A Beta hemolytic streptococciBeta hemolytic streptococci

Theories of Pathogenesis:Theories of Pathogenesis:

Toxic products of streptococci Toxic products of streptococci Immunologic cross-reactivity between Immunologic cross-reactivity between

Streptococcal substances and heart Streptococcal substances and heart muscle (heart reactive antibodies) muscle (heart reactive antibodies)

Sensitized T-lymphocytes may lead to Sensitized T-lymphocytes may lead to cardiac injury cardiac injury

JONES' CRITERIA FOR JONES' CRITERIA FOR DIAGNOSIS OF RF:DIAGNOSIS OF RF:

Major Major ManifestationsManifestations Carditis (friction rub, Carditis (friction rub,

murmur, cardiomegaly, murmur, cardiomegaly, CHF) CHF)

Arthritis (migratory Arthritis (migratory polyarthritis, swollen, polyarthritis, swollen, red, tender) red, tender)

Chorea Chorea Subcutaneous nodules Subcutaneous nodules Erythema marginatumErythema marginatum

Minor Minor ManifestationsManifestations Clinical Clinical

Fever Fever Arthralgia Arthralgia History of rheumatic History of rheumatic

fever or rheumatic fever or rheumatic heart disease heart disease

Laboratory Laboratory Acute phase reactants Acute phase reactants

(ESR, C-reactive (ESR, C-reactive protein, leukocytosis) protein, leukocytosis)

Prolonged P-R interval Prolonged P-R interval on ECGon ECG

PATHOLOGY OF RHEUMATIC FEVERPATHOLOGY OF RHEUMATIC FEVER Cardiac Disease (Rheumatic heart Cardiac Disease (Rheumatic heart

disease)disease) Extra-Cardiac DiseaseExtra-Cardiac Disease

RHEUMATIC HEART RHEUMATIC HEART DISEASEDISEASE

Rheumatic heart disease: all the Rheumatic heart disease: all the heart layers are affected heart layers are affected (pancarditis)(pancarditis)

1.1. Rheumatic myocarditisRheumatic myocarditis

2.2. Rheumatic pericarditisRheumatic pericarditis

3.3. Rheumatic endocarditisRheumatic endocarditis

11 - -Rheumatic myocarditisRheumatic myocarditis::

Acute phaseAcute phase:: it is characterized by the it is characterized by the development of pathognomonic lesions called development of pathognomonic lesions called AschoffAschoff’’s Bodiess Bodies within the myocardium. within the myocardium.

Gross featuresGross features:: Aschoff bodies are multiple tiny nodules (1-2 Aschoff bodies are multiple tiny nodules (1-2

mm in diameter)mm in diameter)

Microscopic features:Microscopic features: Aschoff body is a lesion composed of:Aschoff body is a lesion composed of:

Fibrinoid necrosis ( destroyed fragmented collagen)Fibrinoid necrosis ( destroyed fragmented collagen) Surrounded by lymphocytes and histiocytes &Surrounded by lymphocytes and histiocytes & Aschoff cells (large mononuclear or multinuclear Aschoff cells (large mononuclear or multinuclear

macrophages)macrophages)

Aschoff’s bodyAschoff’s body

Blood vessel fibrinoid degeneration

Aschoff cells

Chronic phaseChronic phase:: Over years or decades the Aschoff Over years or decades the Aschoff

bodies undergo fibrous scarringbodies undergo fibrous scarring

22 - -Rheumatic PericarditisRheumatic Pericarditis : :

Acute phaseAcute phase: Aschoff bodies are formed : Aschoff bodies are formed accompanied by serofibrinous accompanied by serofibrinous inflammation.inflammation.

Chronic phaseChronic phase: Fibrosis and adhesions : Fibrosis and adhesions may occur between the visceral and the may occur between the visceral and the parietal layers of the pericardiumparietal layers of the pericardium

33 - -Rheumatic EndocarditisRheumatic Endocarditis::

It affects both mural and valvular It affects both mural and valvular endocardiumendocardium

1.1. Mural EndocardiumMural Endocardium: : i- i- Acute phaseAcute phase: Aschoff bodies develop in : Aschoff bodies develop in

the endocardiumthe endocardium ii- ii- Chronic phaseChronic phase: healing results in a : healing results in a

white patch white patch

Valvular EndocardiumValvular Endocardium

Vegetations (thrombi) develop at the Vegetations (thrombi) develop at the lines of contact of the cusps causing lines of contact of the cusps causing friction of the swollen cusps.friction of the swollen cusps.

Rheumatic Mitral ValveRheumatic Mitral Valve

Small vegetations are formed at injured parts

CHRONIC RHEUMATIC CHRONIC RHEUMATIC VALVULAR DISEASEVALVULAR DISEASE

Mitral & Aortic Valves PathologyMitral & Aortic Valves Pathology:: Thickening of valve leaflet, especially Thickening of valve leaflet, especially

along the lines of closure along the lines of closure Fusion of commissures Fusion of commissures Result is mitral or aortic stenosis, Result is mitral or aortic stenosis,

insufficiency, or bothinsufficiency, or both

Rheumatic Mitral StenosisRheumatic Mitral Stenosis

Thick valve leaflet

Fusion of commisures

EXTRACARDIAC LESIONS OF RHEUMATIC FEVEREXTRACARDIAC LESIONS OF RHEUMATIC FEVER

JointsJoints:: Rheumatic arthritis affect the large joints in a Rheumatic arthritis affect the large joints in a fleeting way i.e joint inflammation is followed by joint fleeting way i.e joint inflammation is followed by joint resolution, then another joint become inflamed followed resolution, then another joint become inflamed followed by resolution and so on. The affected joint is painful, by resolution and so on. The affected joint is painful, tender, hot & swollen.tender, hot & swollen.

BrainBrain:: Rheumatic chorea (rapid involuntary purposeless Rheumatic chorea (rapid involuntary purposeless movements); it is due to inflammation of the basal movements); it is due to inflammation of the basal ganglia. The condition is reversibleganglia. The condition is reversible

SkinSkin:: Rheumatic subcutaneous nodules occur over bony Rheumatic subcutaneous nodules occur over bony prominences and their structure is similar to the Aschoff prominences and their structure is similar to the Aschoff bodies.bodies.

Rheumatic arteritisRheumatic arteritis: affecting the coronaries, renal, : affecting the coronaries, renal, mesenteric and cerebral arteriesmesenteric and cerebral arteries

Pleurisy and peritonitisPleurisy and peritonitis: serofibrinous type: serofibrinous type

PERICARDIAL DISEASESPERICARDIAL DISEASES

I. PERICARDITISI. PERICARDITIS

Inflammation of the pericardiumInflammation of the pericardium CausesCauses

MI, Staphylococcus, tumor, TB, uremia MI, Staphylococcus, tumor, TB, uremia

II. II. PERICARDIAL EFFUSIONPERICARDIAL EFFUSION Serous fluid in pericardial sacSerous fluid in pericardial sac Usual cause: Chronic Heart Failure Usual cause: Chronic Heart Failure

III. HEMOPERICARDIUMIII. HEMOPERICARDIUM Myocardial rupture from MIMyocardial rupture from MI TraumaTrauma Bleeding from infection, tumor, etc.Bleeding from infection, tumor, etc. Haemorrhage from aortaHaemorrhage from aorta

Hemopericardium