ACUTE RHEUMATIC FEVER. Definition Current Diagnosis 07 An acute systemic immune disease that may develop after an infection with Group A beta- hemolytic.

ACUTE RHEUMATIC FEVER

Definition Current Diagnosis 07

• An acute systemic immune disease that may develop after an infection with Group A beta- hemolytic Streptococcal infection of the pharynx.

• This disease can affect the HEART, JOINTS, SKIN, SUBCUTANEOUS TISSUE, BRAIN, RESPIRATORY SYSTEM, VESSELS, SEROSAL MEMBRANES, TENDONS AND FASCIAL SHEATHS

GENERAL CONSIDERATIONS

• Usually preceded – 2-3 weeks (1-5 weeks) by sore throat.

• Peak incidence 5- 15 years.

Rare in <4 year olds and > 40 years

3% of pt dev ARF

PATHOLOGYThe Aschoff bodies comprises a localised

area of inflammation having a central deposit of amorphous fibrinoid material surrounded by an inflammatory infiltrate of mesenchymal cells known as Anitschkow giant cells or “caterpillar cells” (because the chromatin is distributed in the centre of the nucleus in the forrm of a slender wavy ribbon that resemles the attenuated body with innumerable fine leg like projections )

and an occasional multinucleated Aschoff giant cell with”owl eyed” nucleoli

Fully developed Aschoff bodies are pathognomonic of RF

Aschoff bodies proceed thru 3 phases- exudative, proliferative and healed

The heart has been sectioned to reveal the mitral valve as seen from above in the left atrium. The mitral valve demonstrates the typical "fish mouth" shape with chronic rheumatic scarring. Mitral valve is most often affected with rheumatic heart disease, followed by mitral and aortic together, then aortic alone, then mitral, aortic, and tricuspid together.

Microscopically, acute rheumatic carditis is marked by a peculiar form of granulomatous inflammation with so-called "Aschoff nodules" seen best in myocardium. These are centered in interstitium around vessels as shown here. The myocarditis may be severe enough to cause congestive heart failure.

Here is an Aschoff nodule at high magnification. The most characteristic component is the Aschoff giant cell. Several appear here as large cells with two or more nuclei that have prominent nucleoli. Scattered inflammatory cells accompany them and can be mononuclears or occasionally neutrophils.

Another peculiar cell seen with acute rheumatic carditis is the Anitschkow myocyte. This is a long, thin cell with an elongated nucleus.

MODIFIED JONES’ CRITERIA

MAJOR:

• Polyarthritis

• Carditis

• Chorea

• Subcutaneous nodules

• Erythema marginatum

MINOR CRITERIA

Clinical• Fever• Polyarthralgia• h/o previous ARF or Rheum. heart diseaseLab• Reversible prolongation of PR interval• Inc ESR• Inc C Reactive Protein• + throat culture Or rapid streptococcal antigen

test• Inc ASO titre

POLYARTHRITIS

• Migratory – flitting and fleeting• Involves large joints sequentially• Polyarthritis- in adults only a single joint may be

affected• Lasts 1-5 weeks • Occurs in 75% or patients• Subsides without residual deformity• Dramatic response of arthritis to therapeutic

doses of aspirin or NSAIDs

CARDITIS• Most likely in children and adolescents• Occurs in 1/3 of cases• Any of the following signs suggest the presence of carditis1. Endocardial- - MR or AR murmurs indicative of dilatation of valve ring with or without associated valvulitis -Short mid-diastolic murmur (Carey-Coombs) may be present - Changing quality of heart sounds2. Myocardial - Tachycardia even at rest. Arrhythmias or ectopic beats - Cardiomegaly- on physical exam, CXR or ECHO - Congestive cardiac failure – right or left sided3. Pericardial - Pericarditis - Pericardial effusionECG Changes - Changing contour of P waves - Inversion of T waves - Prolongation of PR interval

Maybe self limiting or may lead to slowly progressive valvular deformityMitral valve attacked in 75% cases, aortic in 30% ( but rarely as the sole valve), tricuspid and

pulmonary in < 5% cases

SYDENHAM’S CHOREA

• Involuntary choreo- athetoid movements primarily of the face, tongue, and upper extremities

• Maybe sole manifestation- in 50% of cases no other signs of RF

• Girls more frequenty affected• Rare in adults• Lease common(<3%) but most diagnostic

of the manifestations of RF

Erythema Marginatum

• Rapidly enlarging macules that assume the shape of rings or crescents with clear centres

• They may be raised, confluent and either transient or persistent.

Subcutaneous Nodule

• Uncommon except in children

• Small (<2cm in diameter) firm & nontender

• Attached to fascia, or tendon sheaths over bony prominences

• Persist for days or weeks• Are recurrent• Indistinguishable from

rheumatoid nodules

• “Also there” features:• Pneumonia• Epistaxis• Erythema nodosum• Abdominal pain

REQUIRED FOR DIAGNOSIS

• Two major criteria OR

• One major and two minor criteria

DIFFERENTIAL DIAGNOSIS

• Rheumatoid arthritis• Osteomyelitis• Endocarditis• Chronic meningiococcemia• SLE• Lyme disease• Sickle cell disease• Surgical abdomen

TREATMENTPHARYNGITISBenzathene penicillin 1.2 million units ( 50,000 units/kg to a max of 1.2 million units) is

injected IM once orInj Procaine penicillin 600,000 units once daily for 10 daysErythromycin can be substituted ( 40mg/kg/day)CARDITIS• Bed rest – until temp, ESR, resting pulse rate and ECG have all returned to normal• Prednisone if there is CCF or cardiomegalyPOLYARTHRITIS• Anti inflammatory agent - Aspirin markedly reduces fever, joint pain and swelling• No effect on the natural course of the disease• 100mg / kg/day in 4-6 divided doses. Can be reduced to 75mg/Kg/day once there is a

response . Given for 4-6 weeks• Toxicity includes- tinnitus, vomiting and GI bleeding.• When response to aspirin is inadequate a short course of prednisone (1 mg/kg/day)

orally daily usually causes rapid improvement of joint symptoms. It is tapered over 2 weeks. Add aspirin when tapering begins.

PREVENON OF ARF-PRIMARY

• Early and adequate treatment of

Strep. throat infections with a penicillin or Azithromycin will prevent Rheumatic Fever

• Avoidance of overcrowding & improved hygiene will decrease the incidence of pharyngitis

PREVENTION -SECONDARYThose who have had RF can have recurrencesRecurrences are most common in children and in those patients who have had

carditis during their initial episode of RF Recurrences are prevented by giving Benzathine penicillin 1.2million units IM

every 4 week OROral penicillin 250 mg bid Erythromycin 250 mg bidAzithromycinDuration controversial:5 years after last attack or at 25 years, whichever is later(earlier recommendation: life-long)Those with cardiac involvement and in high risk group- military personnel,

health staff, school teachers, parents of young children- life long prophylaxis

IMPORTANT!!

The complication of untreated, or

inadequately treated Acute rheumatic

fever is

RHEUMATIC HEART DISEASE

RHEUMATIC HEART DISEASE

• Results from single or repeated attacks of RF• Rigidity and deformity of valves resulting in

stenosis or incompetence or both • Mitral valve alone in 50%• Mitral + Aortic in 25%• Pure aortic uncommon• History of RF obtained in 60%• Should receive prophylatic penicillin monthlyand

preceding dental extractions,urologic and surgical procedures to prevent endocarditis