Acute Rheumatic Fever: Diagnostic and Management SymCARD 4 th 2014 Acute Rheumatic Fever: Diagnostic and Management Didik Hariyanto Indry Putri Festari Pediatric Cardiology Subdivision Division of Cardiology and Vascular Medicine Faculty Medicine Universitas Andalas General Hospital dr. M. Djamil Padang

Introduction Rheumatic fever (RF) is nonsuppurative complications of Group A streptococcal pharyngitis due to a delayed immune response Continues to be problem worldwide Underdiagnosed and undertreated Estimated 30 million people suffer from ongoing heart disease from ARF, 70% dying at average age 35 years old RHD developed in 44% of patients who initially had no clinical evidence of carditis Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013 Lioyd T et all, Pediatrics 2003: 112:1065-68

IS IT ACUTE RHEUMATIC FEVER? Case: A 11 year-old girl, brought to hospital because she has pain in her right knee that is preventing her from walking There’s breathlessness during activity History of sorethroat 2 weeks before ARTRITIS and DISPNEU Diff Dx? Septic arthritis Rheumatic fever Juvenille Rheumatoid Arthritis Congenital Heart DIsease etc IS IT ACUTE RHEUMATIC FEVER?

Arthritis in Acute Rheumatic Fever Most common feature: present in 80% of patients Painful, migratory, short duration, excellent response of salicylates Usually affected and large joints preferred knees, ankles, wrists, elbows, shoulders Small joints and cervical spine less commonly involved Differenciate with athralgia 4 SymCARD 2014 th 1 WHO. Rheumatic Fever and Rheumatic Disease. 2001 2Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013

Carditis 4 SymCARD 2014 Most serious manifestation May lead to death in acute phase or at later stage Any cardiac tissue may be affected Valvular lesion most common: mitral and aortic Clinical manifestations: Breathlessness Tachycardia Murmur (MR and AR) Cardiomegaly Heart failure th 4 SymCARD 2014 1 Park MK. Pediatric Cardiology for Practitioners. 2008 2Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013

Major Manifestation Minor Manifestation WHO Criteria for diagnosis of rheumatic fever (based on revised Jones criteria) Major Manifestation Minor Manifestation Carditis Polyarthritis Chorea Erythema marginatum Subcutaneous nodules Clinical : fever, poliathralgia Laboratory: elevated acute phase reactans (erythrocyte sedimentation rate or leucocyte count) Supporting evidence of a preceding streptococcal infection within the last 45 days Electrocardiogram: Prolonged P-R interval Elevated or rising antistreptolysisn-O or other streptococcal antibody, or A positive throat culture, or Rapid antigen test for group A streptococci, or Recent scarlet fever 1 WHO. Rheumatic Fever and Rheumatic Disease. 2001

Criteria Diagnosis ARF Two mayor manifestation, or Combination 1 mayor and 2 minor manifestations and Supporting evidence of a preceding streptococcal infection 1 WHO. Rheumatic Fever and Rheumatic Disease. 2001 2Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013

2002–2003 WHO criteria for the diagnosis of rheumatic fever and rheumatic heart disease (based on the revised Jones criteria) Diagnostic categories Criteria Primary episode of RF Two major *or one major and two minor** manifestations plus evidence of a preceding group A streptococcal infection***. Recurrent attack of RF in a patient without established rheumatic heart disease Two major or one major and two minor manifestations plus evidence of a preceding group A streptococcal infection. Recurrent attack of RF in a patient with established rheumatic heart disease. Two minor manifestations plus evidence of a preceding group A streptococcal infection Rheumatic chorea. Insidious onset rheumatic carditis Other major manifestations or evidence of group A streptococcal infection not required Chronic valve lesions of RHD (patients presenting for the first time with pure mitral stenosis or mixed mitral valve disease and/or aortic valve disease). Do not require any other criteria to be diagnosed as having rheumatic heart disease

Syndenham’s Chorea Extrapyramidal disorder Fast, clonic, involuntary movements (especially face and limbs) Muscular hypotonus Emotional lability First sign: difficulty walking, talking, writing Usually a late manifestation: months after infection Often the only manifestation of ARF 1 Park MK. Pediatric Cardiology for Practitioners. 2008 2Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013

Subcutaneous Nodules Usually 0.5 - 2 cm long Firm, non-tender, isolated or in clusters Most common: along extensor surfaces of joint knees, elbows, wrists Also: on bony prominences, tendons, dorsi of feet, occiput or cervical spine 1 Park MK. Pediatric Cardiology for Practitioners. 2008 2Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013

Erythema Marginatum Present in 7% of patients Highly specific to ARF Cutaneous lesion: Reddish pink border Pale center Round or irregular shape Often on trunk, abdomen, inner arms, or thighs Highly suggestive of carditis

Arthralgia without objective findings is common in RF Less common Minor Manifestation Supporting evidence Fever Occurs in almost all rheumatic attacks at the onset, usually ranging from 38.4–40.0 °C Diurnal variations are common, but there is no characteristic fever pattern. Athralgia Arthralgia without objective findings is common in RF Less common abdominal pain and epistaxis ECG  Prolong PR interval 1 Park MK. Pediatric Cardiology for Practitioners. 2008 2Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013

Therapy General guideline for bed rest and indoor ambulation Arthritis alone Mild Carditis Moderate Carditis Severe Carditis Bed rest 1-2 week 3-4 week 4-6 week As long as CHF is present Indoor ambulation 2-3 month Recommended anti-inflammatory therapy Arthritis Alone Mild Carditis Moderate Carditis Severe Carditis Prednisone 2-6 week Aspirin 1-2 week 3-4 week 6-8 week 2-4 month Dosages: Prednisone, 2 mg/kg/day, in four divided doses; aspirin, 100 mg/kg/day, in four to six divided doses 1 Park MK. Pediatric Cardiology for Practitioners. 2008

Therapy… Primary prevention of rheumatic fever: recommended treatment for streptococcal pharyngitis 1 WHO. Rheumatic Fever and Rheumatic Disease. 2001

Therapy…. Antibiotics used in secondary prophylaxis of RF 1 WHO. Rheumatic Fever and Rheumatic Disease. 2001

ARF and Heart Failure Management: When and How to Use it? Diuretic ACE-inhibitor Aldosterone antagonist Inotropic When and How to Use it? 1 WHO. Rheumatic Fever and Rheumatic Disease. 2001 2Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013

Monitoring and Evaluation ARF ARF could become Rheumatic Heart Disease Monitoring: Echocardiography Check inflammation marker if needed 1Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013

Complication Rheumatic Heart Disease Heart Failure Other issues: When the patient need to perform surgery? Repair/replacement? 1Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013

Take Home Message Acute Rheumatic Fever leading to Rheumatic Heart Disease is a major problem world wide. Appropriate treatment of group A strep pharyngitis necessary to prevent disease. Preventing recurrences causing chronic heart disease simple, universally available, and costeffective.

terimakasih 4 SymCARD 2014 th