Rheumatic fever, pericarditis

Rheumatic fever, pericarditis
Szántó Antónia
3rd Dept. Of Medicine
Rheumatic fever
• First description in 17th century
• Autoimmune postsuppurative
streptococcal pharyngitis cascade,
leading to various manifestations
• In industrialized countries, its
significance is decreasing, in developing
nations, it remains common
• Incidence /industrialized nations: 1/100 000
/developing nations: 100/100 000
Decline in mortality from rheumatic fever in the United States during the
20th century.
Decline began well before the availability of penicillin.
Dashed arrows mark the multiple modifications of the Jones-criteria
• Group A beta-hemolytic Streptococcus (GABHS)
• More than 100 subtypes are defined by M
protein surface molecules
• Antiphagocytic properties of M protein allow
persistence of bacteria in tissues up to 2
weeks, until specific antibodies are created.
• M protein and several other epitopes can mimic
– Myocardium (myosin, tropomyosin)
Heart valves (laminin)
Synovia (vimentin)
skin (keratin)
Subthalamic and caudate nuclei (lysogangliosids)
• Superantigenic activity triggered by M proteinfragments as well as streptococcus toxins have been
implicated in B- and T-cell mediated autoimmune
• T-cells activated against myosine and bacterial
epitopes react to valve tissue with host factors that
may enhance inflammatory response in heart valves
Disease course
• Acute rheumatic fever (RF) occurs after
GABHS pharyngitis, and probably not after
other infections caused by S. pyogenes.
• Phases:
Pharyngitis -> (in 2-3 weeks) -> migratory
polyarthritis, erythema marginatum -> carditis
->(within 1-6 months)-> Sydenham chorea,
subcutaneous nodules
Symptoms - Carditis
40 to 60% of rheumatic fever episodes result in rheumatic
heart disease
Carditis tipically manifests as valvulitis detected by the
presence of mitral regurgitation (MR) or (less commonly)
aortic regurgitation (AR)
Physical examination:
– Sinustachycardia
– First heart sound varies from normal to diminished
– Second heart sound is normally or variably split
– MR: soft, blowing, pansystolic murmur of the apex
conducted to the axilla and back
– AR: early diastolic murmur best heard along the base and
left sternal border
– Sometimes even other murmurs can be heard (later,
because of secondary valve-deformities)
– Secondary pericarditis can be present
• Echocardiography: not necessary to the diagnosis,
rheumatic fever can be overdiagnosed – controversal
Rheumatic mitral regurgitation
Symptoms - Arthritis
• Most frequent manifestation of RF, in up to 75% of
patients with acute symptoms
• Painful, migratory, limited to the major joints
• Earliest manifestation after streptococcal pahryngitis,
within 2-3 weeks after onset of RF, may be the only
clinically apparent manifestation in 33-50% of patients
• Self-limited with varying symptoms from minor arthralgia
to severe arthritis (erythema, warmth, swelling)
• Joint aspiration may reveal moderate leukocytosis
• Individual arthritis last 1 to 2 weeks, polyarthritis
resolves in 1 month or less
• Arthritis phase frequently overlaps with the onset of
carditis, and the two manifestations appear to be
inversely related in severity
Monoarthritis in RF
Symptoms - Chorea
• Involuntary, irregular movements,
fibrillatory muscle movements of the
tongue, external rotation of the hands,
abolition of the movements with sleep
• Latency period can last for 1-7 months
• Other neuropsychiatric manifestations:
emotional lability, obsessive-compulsive
behavior, seizures and chronic migraine
Symptoms – skin symptoms
Subcutaneous nodules (in patients with
moderate-to severe carditis)
• They occur several weeks after the
onset of cardiac findings
• Firm, asymptomatic nodules over major
joints and bony prominences, resolving
within few weeks
• Can be seen with other autoimmune
disorders, too
Subcutaneous nodules in RF
Symptoms – skin symptoms
Erythema marginatum
• Occurs in milder carditis
• May last for months or years
• Tend to occur over the trunk or
proximal extremities
• Occurs with sepsis and drug
reactions, too
Erythema marginatum
Laboratory findings
• CRP, ESR are significantly elevated (their time
course correlates with the disease activity)
• Throat culture is positive for GABHS, but many
careers exist and after beginning of treatment
it can also be negative
• Rising streptococcal antibody titers:
– Anti-streptolysin-O
– Anti-DNAse B (more frequent in glomerulonephritis)
– Anti-hyaluronidase
Diagnosis: Jones-criteria
Major criteria
• Carditis
• Polyarthritis
• Chorea
• Erythema
Minor criteria
• Arthralgia
• Fever
• Elevated CRP, ESR
• Elevated PQ/PR
• Subcutaneous nodules
Rheumatic fever = 2 major or 1 major+2
minor criteria and proved previous S.
pyogenes infection
• Acute antimicrobal therapy to remove GABHS from the
pharynx (antibiotics started less than 10 days after the
onset of pharyngitis almost completely eliminates the
risk of the disease)
• Bed rest: for patients with carditis
Primary prevention (at S. pyogenes infection, to
prevent rheumatic fever):
• Penicillin per os for 10 days
• Benzathine penicillin G intramuscular
• In case of allergy to penicillin: erythromycin
Secondary prevention (preventing recurrence):
• benzathine penicillin every 4 weeks
– For 5 years or until age 18 in the abscence of carditis
– For 10 years or until age 25 for patients with mild
Treatment of carditis
• Salicylates, NSAID: only if concomitant
pericarditis is present
• Acute carditis: corticosteroids
• Adequate treatment of heart failure
and valvular regurgitation
• In acute rheumatic fever, surgery must
be avoided because of high mortality
Treatment of arthritis
• Salicylate (100 mg/kg/day in 4 divided
both therapeutic and diagnostic: failure
of the pain to resolve within 24 hours
suggests alternative causes of arthritis
Treatment of chorea
• Sedation
• Antiseizure
• Antipsychotic medications
• Sac created from a duplicated connective tissue
(mesothel) membrane
• Contains an external (fibrous) and an internal
• Involves the heart, the first part of the aorta,
the pulmonary trunk and the final part of the
pulmonary veins
• Contains cca. 15-50 ml pericardial fluid
• Functions: to stabilize the position of the
heart, to inhibit the extension of the
inflammatory and malignant processes of the
sorrounding tissues
Categories of pericardial disease
*= etiologies that can present as acute pericarditis
Primary (mesothelioma, fibrosarcoma, lipoma)
Secondary (breast and lung carcinoma, lymphomas, Kaposi-sarcoma)
Radiation induced*
Early postcardiac surgery
Autoimmune diseases* (SLE, RA, scleroderma)
Vasculitis (polyarteritis nodosa, arteritis temporalis)
Inflammatory bowel diseases
Early post-myocardial infarction
Late post- myocardial infarction (Dressler-syndrome)*
Drug induced* (procainamid, hydralazine, INH, cyclosporin-A)
Neoplastic diseases
Viral* (coxsackie-, echo-, adeno-, cytomegalovirus, HIV, HBV)
Bacterial* (Pneumococcus, Staphylococcus, Streptococcus, Meningococcus, Mycoplasma, Lyme, H. influenzae)
Mycobacteria* (Tb, avium-intracellulare)
Fungal (Histoplasmosis, Coccidiomycosis)
Post-myocardial infarction – free wall rupture
Device and procedure related
Aortic dissection
Miscellaneous (chronic renal failure, chylopericardium, hypo- and
hyperthyreoidism, amyloidosis)
Acute pericarditis
• Symptoms resulting from pericardial
inflammation of no more than 1-2 weeks’
• Almost always presents with chest pain caused
by epicardial and pleural involvement
• Inflammation of the visceral membrane leads to
pericardial effusion
Symptoms, physical examination
• Pericardial pain, chest pain (substernal, left anterior
chest, soometimes epigastrium)
• Radiation to the trapezius ridge, to the left side of the
• Pain is relieved by sitting forward and worsened by lying
• Dyspnea, coughs, low-grade fever
• Tachycardia
• Friction rub caused by contact between visceral and
parietal pericardium
• Classic rub is distinctive, easily recognized, consists of
three components corresponding to ventricular systole,
early diastolic filling and atrial contraction (similar to the
sound made when walking on crunchy snow)
• Rub is loudest at the lower left sternal border, extends
to the cardiac apex, best heard with patient leaning
ECG signs of acute pericarditis
Phase 1 (initial): PQ depression, ST elevation
Phase 2 (1-2 weeks): isoelectric ST, flat T waves
Phase 3: negative (inverted) T waves
Phase 4 (weeks-months): isoelectric T waves
ECG in acut pericarditis. Diffuse ST elevation and PQ depression
Chest X-Ray, echocardiography
Changes present only when effusion is present
Treatment of acut pericarditis
• Usually self-limited without significant
• Bed rest, analgetics, lowering fever
• NSAIDs, high-dose salicylate
• If presence of fever after 48 hours:
• If concomittant autoimmune disease is present:
immunosuppressive drugs
• If bacterial origin is proved: antibiotics
• If caused by known malignant or infectious
disease: treating of the primary disease
• Relapse in cca. 25% of cases
Pericardial effusion
Everything that can cause pericarditis can cause pericardial
Can be asymptomatic as well
Significant symptoms occur when the effusion reduces the
volume of the cardiac chambers such that cardiac output begins
to decline
Determinants of the hemodynamic consequences are
– Level of pericardial pressure
– Ability of the heart to compensate for elevated pressure
Pressure depends on:
– Amount of the effusion
– Accumulating velocity of the exsudate (200-2000 ml!)
– Physical and biochemistry features of the pericardium
In extreme cases: circulatory collapse (pericardial tamponade)
Pericardial tamponade
• Elevation of intrapericardial volume and pressure leading
to a circulatory collapse
• Clinical presentation:
Dyspnea, sinus tachycardia, muffled heart sounds
Wider relative dullnes
Friction rub may be present
Elevated jugular venous pressure
Paradoxical pulse (inspiratory decrease in arterial pressure is
• Hypotension, shock
• ECG: low voltage, electrical alternans
• Even the suspicion requires echocardiography!
• Echocardiography signs: right atrial and ventricular
diastolic collapse, increased inferior caval vein filling,
„swinging” heart
• Treatment: pericardiocentesis
- subxiphoid needle insertion, best performed under
echocardiographic guidance
Constrictive pericarditis
End stage of an inflammatory process involving the pericardium
Pericardial scarring leads to restricted filling of the heart
Clinical signs are symptoms of right heart failure
Diastolic pressure is increased and causes atrial, pulmonary and
systemic venous pressure elevation
Low cardiac output is compensated by tachycardia
Peripheral pulse is weak
Prominent jugular veins
Liver congestion sometimes leading to cardiac cirrhosis
Chest radiograph: cardiac silhouette is enlarged secondary to a
coexisting pericardial effusion
Pericardial calcification often can be seen
Treatment: pericardiectomy
Chest radiographshowing marked pericardial calcifications
in constrictive pericarditis