Acute Rheumatic Fever: Diagnostic and Management

1. 4 th 2014 SymCARD Acute Rheumatic Fever: Diagnostic and Management DidikHariyanto IndryPutriFestari Pediatric Cardiology Subdivision Division of Cardiology and Vascular Medicine Faculty Medicine UniversitasAndalas General Hospital dr. M. DjamilPadang

2. Introduction Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013 Lioyd T et all, Pediatrics 2003: 112:1065-68 Rheumatic fever (RF) is nonsuppurativecomplications 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

3. 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?

4. 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 th 4 SymCARD 2014 1 WHO. Rheumatic Fever and Rheumatic Disease. 2001 2Hampole CV. Rheumatic Fever. Manual of Cardiovasc Med. 2013

5. Carditis • Most serious manifestation • May lead to death in acute phase or at later stage • Any cardiac tissue may be affected • Valvularlesion 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

6. WHO Criteria for diagnosis of rheumatic fever (based on revised Jones criteria) 1 WHO. Rheumatic Fever and Rheumatic Disease. 2001

7. 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

8. 2002–2003 WHO criteria for the diagnosis of rheumatic fever and rheumatic heart disease (based on the revised Jones criteria)

9. 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

10. 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

11. 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

12. 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

13. Therapy General guideline for bed rest and indoor ambulation Recommended anti-inflammatory therapy 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

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

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

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

17. 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

18. 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

19. 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.

20. terimakasih th 4 SymCARD 2014