Rheumatic fever

Definition

Epidemiology

Commonest in 5-15y

Rare in developed world, but important
cause of heart disease in children worldwide

Reducing incidence due to imropoved sanitation, living conditions and prompt tx of strep pharyngitis

Autoimmune destruction of heart valves
due to cross-reaction of antibodies
to GAS B-haemolytic strep

Pathophysiology

Agent
Group A B-haemolytic strep colonises
and infects the pharynx (pharyngitis)

Mechanism
Abs to strep made during pharyngitic or skin infection
2-6 weeks after infection
Antigens on heart valves similar to those on strep (molecular mimicry) - Abs recognise valves as foreign
Immune destruction of heart valves
Leads to scarring and fibrosis of valves (MS)

Clinical
presentation

Diagnosis

Management

Complications

Prevention

Chronic rheumatic heart disease
80% of cases
Mitral stenosis
Recurrent RF

Examination

Investigations

History

PMH
Growth and development
Vaccinations
Known medical conditions

DH
Meds (recent abx), allergies

POH
Scans, bloods, growth, gestation,
delivery, weight, complications

FH
Heart conditions
Recently unwell

PC/HPC
Fever, malaise, polyarthritis
Preceeding sore throat (abx?)

Malaise

Polyarthritis
(flitting lasts <1w, migrating to other joints)

Fever

Chest pain

Sydenham chorea
10% of cases
2-6m after infection
Involuntary movements, emotional lability for 3-6m

Diagnostic criteria
(Jones criteria)

2 major or 1 major+ 2 minor
PLUS microbio GAS evidence

SH
Living arrangements,
school/nursery

Cardio
Murmur (diastolic if MS/AR, systolic if MR)
Pericardial rub (if pericarditis)

Heart failure
Due to myocarditis

Presentation
Carditis, involving endocardium, myocardium and pericardium (effusion, tamponade)
Migratory arthritis affecting multiple joints
Syndenhams chorea
Skin lesions (erythema marginatum, SC nodules) - rare

Musculoskeletal
Tender, erythematous, swollen joints

Bedside
Obs (fever)
ECG (prolonged PR)

Bloods
FBC (high WCC), CRP/ESR (high),
U+E, LFT (baseline)
Rapid strep antigen test (+ve)
Strep Ab titre (high/rising)

Swabs
Throat culture (GAS +ve)

Major
Significant murmur/evidence valve dysf on ECHO
Pericardial rub/effusion/tamponade
Migratory arthritis
Sydenham's chorea
Erythema marginatum
SC nodules

Minor
Fever
Polyarthralgia
Raised CRP, ESR, WCC
Prolonged P-R on ECG

Medical

Surgical

Conservative
Information, advice, support
Self care (bed rest, fluids, limit exercise if ECHO changes)

Imaging
CXR: nil (unless HF)
ECHO: may see valve changes

Anti-inflammatories
Indication: all patients
E.g. aspirin, ibuprofen
MOA: inhibits inflammation of
heart valves and joints, reduces pain
NB, monitor salicylate levels

Steroids
Indication: anti-inflammatories ineffective
MOA: reduce inflammation of heart valves and joints

Pericardiocentesis
Indication: pericardial effusions
MOA: drain fluid from around the heart

Primary
Rapid abx treatment of GAS pharyngitis

Secondary
Benzylpenicillin IM monthly for 10y to lifelong
(or PO erythromycin if pen allergic)

Valve surgery
Indication: significant valve disease
MOA: repair or replacement

Abx
Indication: all patents
E.g. benzylpenicillin PO 10d
MOA: destroy remaining GAS

Rash
(red raised edges, clear centre)

Skin nodules
(extensor surfaces and spine)

Derm
Skin nodules on spine/extensors
Erythema marginatum rash

Neuro
Choreas

Sedatives
Indication: chorea
E.g. haloperidol, diazepam