Issuu on Google+

Rheumatic Fever (A disease of poverty) Susana LEITU Tuisese ILAITIA Trainee Interns FSM 2008


Acute rheumatic fever is a multi-system auto-immune disease that follows group A streptococcal infection (GAS)


Background Information • ARF & RHD – most common heart disease in children & young adults • Major health problem in developing countries – Pacific Islands • ≈ 15.6 million ppl are affected worldwide – 2.4 million – are children (5-14yrs) in developing countries

• Almost ≈ 0.5 million new cases declared every year


Group A Streptococcal • Beta hemolytic • Common culprit of pharyngitis, impetigo and scarlet fever • Virulent due to – M protein – F protein – Acid (lipoprotein & hyaluronic) – cell and capsule – Produces toxin - streptolysin O


Pathogenesis • Abnormal humoral and cellular immune response occurs –Antigenic mimicry btw antibodies to Strept M protein and human tissues • Valves, myosin, tropomysin of heart • Brain protein • Synovial and cartilages

–Causing inflammation and tissue damage


www.health.gov.mt


Manifestations Resolved sore throat – 1-2wks b4 symptoms

• Arthritis – Pain & swelling in more then one large jnt (ankle, knees, wrist) – Usually ‘migratory’

• Fever


• Carditis – inflammation of heart – Commonly present as a heart murmur – Chest pain +/- difficult breathing

• Chorea (Sydenham’s) – Twitching, jerking movement and muscle weakness (most obvious in the face, hands and feet) – Usually resolves within 6 weeks (may last 6 months or more)


Less common

• Subcutaneous Nodules

– Painless lumps – outside surface of elbow, wrist, knees, ankle in grps of 3-4-12) – Skin not red/inflamed – Last 1-2 weeks

• Erythema Marginatum – Painless, flat pink patches on skin – Usually on the trunk – Hard to see on dark skin ppl

• Cough and Abdominal pain


Diagnosis - Modified JONES

MAJOR CRITERIA I. Carditis II. Polyarthritis (migratory) III.Chorea IV.Subcutaneous Nodules V. Erythema Marginatum

MINOR CRITERIA I. Fever II. Arthralgia III.Prolonged PR interval IV.Elevated ESR, CRP


1st episode of ARF (WHO) <

2 MAJOR + Evidence of preceding Group A Streptococcal Infection OR

<

1 MAJOR + 2 MINOR + Evidence of preceding Group A Streptococcal Infection


• Untreated Grp A strept infxn (eg pharyngitis) Æ lead to ARF • Repeated / recurrent infxns lead to chronic heart valve damage (RHD)


Differential diagnosis • Systemic lupus eruthromatosus • Infetive endocarditis • Reactive arthritis • Drug reaction • Other connective tissue disease


Baseline assessment • ARF criteria • ECG • Swab throat and any infected skin sores – usually negative • FBC, ESR, ASOT, Blood culture • Echocardiography – cardiac functions and monitor progression


Management (no RHD) 1.

Admit for clinical care & education for prevention further episodes 2. Benzathine Penicillin G (single dose) OR Oral Penicillin V x 10 days 3. Paracetamol / asprin â&#x20AC;&#x201C; fever and arthritis 4. Severe Chorea â&#x20AC;&#x201C; Carbamazepine or Valporic acid


5. Carditis • •

bed rest Anti failure medication – diuretics, ACEI, digoxin (anticoagulants if AF)

When stabilized • Give 1st dose of 2° prophylaxis • Educate pt & family - preventions – Recurrent episodes, endocarditis, & compliance – Occasional follow ups


Long-term Management •

Regular prophylaxis 1.

Benzathine Penicillin G 600,000 – 1,200,000 units IM 3 weekly – Penicillin V 250mg oral BD (if 1 is contraindicated/not tolerated) – Erythromycin 250mg oral BD (if penicillin alergic)


Rheumatic Heart Disease


Rheumatic heart disease is the chronic damage to heart valves that follows acute rheumatic fever


1. Mitral valve is affected in >90% cases 2. Aortic valve 3. Tricuspid & pulmonary â&#x20AC;&#x201C; rarely (only in severe RHD when all valves are affected)


1.

Valvular lesions Mitral Regurgitation 1. Most common lesion 2. Find commonly in children & adolescent

2. Mitral Stenosis 1. Longer chronic damage to mitral valve 2. Commonly seen in adults 3. Complication â&#x20AC;&#x201C; Atrial fibrillation

3. Aortic Stenosis 1. Rarely seen as an isolated lesion 2. Usually develop as a long-term complication of AR


Symptoms – depends on valve lesion & severity • May not show for years until disease become severe – SOBOE – Feeling tired – General weakness

• As Lt ventricular failure advances (later) – Orthopnea (SOB on lying down) – Paroxysmal Noctural Dyspnoea (PND) – waking up at night with SOB


• Rt ventrical may also fail – Peripheral oedema

• If Atrial fibrillation (in MS) – Palpitation • Risk of thromboembolic strokes

• Patients with Aortic valve lesion may experience (in addition to SOB) – Angina – Syncope


Examination & Investigations Clinical assessment should be conducted CAREFULLY - early detection makes a big different in life saving • Careful auscultation to pick out murmurs – refer for echocardiography • Thorough clinical examination to assess severity and complication – Ventricular failure – AF or stroke – IE


♥ECG - Determines sinus rhythm & ventricular failure ♥Chest X-ray - Aids in assessing chamber size & detecting signs of failure – pulmonary congestion ♥Echocardiography - Detects valve damage, assessing the severity & Lt ventricular function


Management (RHD) GOAL – prevent disease progression & to avoid, or delay valve surgery Mx depends on severity of disease 1.

2° prophylaxis (paeds 3wkly, adults 4wkly) 2. Regular clinical review (3wkly – yearly) 3. Follow up echo – follow progression


Complications of RHD • Congestive cardiac failure • Infective endocarditis • Atrial fibrillation – mostly in MS • Stroke – 2˚ to AF, LA dilatation


Case 13 yo Fijian male referred from Sigatoka hospital for: • Cough & fever – 2/7 • SOBOE & on rest – 1/52


• HPC: – Previously well until 1/52 (developed SOB on exertion and on rest) – 2 days ago developed productive cough and fever. – Uses to play rugby before • No chest-pain • No PND – uses 1 pillow at night • Gets tired easily • No history of sore throat

• ROS: 9PU 9BO • No vomiting, no dizziness and no fitting


• PMH

–Currently the 1st admission • SH

–3rd eldest of 5 children –Parents separated – children living with mothers family –Pt lives with maternal grandmothers sister


Clinical Findings • O/E: Well built and in dyspnoeic – SaO2: 91%RA – HR: 126 – BP: 104/88 – T: 36.7° C – MAP: 95


Chest • • • •

Hyperdynamic precordium Apex beat @ 6th ICS/MCL Parasternal heave S1S2 – grade 4 Pansystolic murmur radiating up to axilla • Soft diastolic murmur • Coarse crepitation bibasally


Abdomen: • Soft, not distended • Liver – 5-6cm BRCM • Spleen not palpable Extremities: • Mild bipedal pitting oedema Skin: • Generalized tinea versicolour lesions


Investigations • • • • • • • • • • • •

Urea Creatinine Sodium Potassium Chloride Glucose/RBS Total Bilirubin ALP ALT AST Total Protein Albumin

2.9 47 137 4.9 107 3.9 19 151 33 22 74 31

(1.7-8.3) (62-106) (135-148) (3.5-5.3)

(90-110) (3.9-9.9) (0-17) (40-129) (10-41) (10-37) (66-87) (34-48)

mmol/l L µmol/l mmol/l Hmmol/l mmol/l mmoll µmol/l 1H U/I U/I U/I H g/l g/l


ÓChest X-Ray ÓBlood culture – R/o IE – 3 sets of blood ÓNo growth 24hrs, 48hr and 72hrs

ÓEchocardiography


Assessment 1. 2. 3. 4. 5.

Rheumatic Heart Disease Mitral regurgitation ?? AR Heart failure DDx Infective Endocarditis


Management • Supportive – O2 etc • Anti-failure – Frusemide 67mg IV 6hourly – Spironolactone 25mg orally 8hourly – El Enalapril 1mg orally BD – Digoxin 250mcg orally BD

• Crytalline Penicillin


Follow up ☺For 3 weekly prophylaxis ☺Follow up - SOPD ☺Follow up echocardiography ☺IE prophylaxis



Rheumatic ARF/RHD + case