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1ACUTE RHEUMATIC FEVER2Acute Rheumatic FeverDefinitionEtiologyEpidemiologyPathogenesisPathologyClinical ManifestationLaboratory FindingsDiagnosis TreatmentPrognosis

31. DEFINITIONRheumatic Fever: Inflamatory disease, appears as a delayed nonsuppurative sequel to group A streptococcal infection Affect: heart, joint, central nervous system, skin, subcutaneus tissues with varying frequency42. ETIOLOGYUpper respiratory tract with group A streptococcusCutaneous streptococcal infection may lead to acute glomerulonephritis but has never been demonstrated to cause acute rheumatic fever (ARF)

53. EPIDEMIOLOGYMajor health problem in the developing countries in Asia, Africa, Middle East, and Latin America.Hospital statistics from many developing countries reveal that 10 to 35 % of all cardiac admissions are for ARF and rheumatic heart disease.Most common children 5-15 y.o. groupThe twentieth century witnessed a dramatic decline in the incidence of ARF and rheumatic heart disease in industrialized nations 64. PATHOGENESISExact mechanism by group A streptococcus causes unexplainedToxic effects of streptoccal products particularly streptolysin S or OSerum sickness-like reactionAutoimmun phenomena induced by similarity or certain streptoccal antigen7Shared epitops between cardiac myosin and streptococcal M protein that lead to cross reactive humoral and T cell immunity against group A streptococci and the heartARF affects only a small proportion of infected persons possibility of a genetic predisposition to rheumatic attacks.

4. PATHOGENESIS85.PATHOLOGYCharacterized by exudative and proliferative inflammatory lesions of the connective tissue, most notably of heart, joints and cutaneous tissue.When carditis ensues, all layers of the heart are involved.9Myocardium initially there is fragmentation of collagen fibers, lymphocytic infiltration, and fibrinoid degeneration, followed Aschof nodules (pathognomonic of ARF).Aschof nodule consists of an area of central necrosis surounded by lymphocytes, plasma cells, and large mononuclear and giant multinucleate cells.5.PATHOLOGY10Endocardial involvement is responsible for chronic rheumatic valvulitis.Even when no vegetation are present, there is edema and inflammation of the valve leaflets.A thickened and fibrotic patch (MacCllumpatch) may be found in the posterior left atrial wall.Mitral valve is involved most frequently, followed aortic valve5.PATHOLOGY116. CLINICAL MANIFESTATIONSLatent period from the onset of streptococcal sore throat to the onset of initial and recurrent attacts of ARF varies between 1 and 5 weeks Mode of onset is quite variable: an abrupt onset with fever and toxicity, insidious or even subclinical when mild carditis is the initial manifestationArthritis occurs in approximately 75% of first attact, carditis 40-50 %, chorea 15 %, subcutaneous nodules and erytema marginatum < 10%12

13CarditisThe only manifestation of ARF that has the potential to cause long term disability and death. Usually appears within the first 3 weeks of the illness. The diagnosis requires the presence of one of the following manifestations: (1) organic cardiac murmurs not previously present, (2) cardiomegaly, (3) pericarditis,(4) congestive heart failure.14Valvulitis is characterized by characteristic murmurs. Increased flow across the mitral valve in the presence of valvulitis may produce a middiastolic murmur (Carey Coombs murmur) that follows an S3 gallopMitral regurgitation (MR) leads to a blowing holosystolic murmur best heard at the apex and radiating to the axilla and occasionally to the base of the heart or the back.15PolyarthritisThe most frequent major manifestation of ARF. Any joint may be affected, but involvement of larger joints such as knees, ankles, elbows, and wrists is more common.

The synovial fluid contains numerous white blood cells with a marked preponderance of polymorphonuclear leukocytes.

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These nodules are seen in only 1 to 21 percent of patients with ARF.Most often associated with carditis and rarely appear as an isolated manifestation of ARFRound, firm, painless, freely movable subcutaneous lesions varying in size from 0.5 to 2.0 cm.

Subcutaneous Nodules17Erythema MarginatumThis rash is usually found on the trunk and proximal parts of the extremities, with the face being spared. It begins as an erythematous macule or papule that extends outward while skin in the center returns to normal.

18Sydenham's Chorea It is characterized by rapid, purposeless, involuntary movements, most noticeable in the extremities and face. The arms and legs flail about in erratic, jerky, uncoordinated movements. The speech is usually slurred and jerky. Emotional lability is characteristic of Sydenham's chorea and may often precede other neurologic manifestations.

19Minor ManifestationsMinor manifestations of ARF include fever, arthralgia, and laboratory evidences of inflammation. Fever usually ranges from 38,4 to 40C (101.12F to 104F) and rarely lasts for more than 3 to 4 weeks. Arthralgia is pain in one or more joints without objective evidence for inflammation.20

217. LABORATORY FINDINGSA mild to moderate normochromic normocytic anemia and leukocytosis with an increased proportion of polymorphonuclear leukocytes are common. Elevated serum levels of C-reactive protein and an increased erythrocyte sedimentation rate are almost always present, indicating the presence of acute inflammation. 22Throat cultures are usually negative for group A streptococci by the time ARF appears. Streptococcal antibody tests provide evidence for antecedent Streptococcal infection and include antistreptolysin O (ASO), anti-DNAse B, and antihyaluronidase. The ASO is elevated in 80 percent or more of patients with ARF. ASO liters greater than 200'Todd U/mL in adults and 320 Todd U in children are generally considered elevated. 7. LABORATORY FINDINGS23ElectrocardiogramPersisten sinus tachycardia that does not resolve during sleep is common in the presence of carditis.Sinus bradycardia and sinus arrhythmia may be present in some patients and can be abolished by the administralion of atropine. Prolongation of the PR interval is a common abnormality. 7. LABORATORY FINDINGS24Some investigators have suggested that the AV conduction delay is a manifestation of carditis. Transient complete heart block that causes Stokes-Adams attacks has been described. Atrial flutter and fibrillation have been described in the presence of carditis. 7. LABORATORY FINDINGS25EchocardiogramThe key features of rheumatic mitral valvulitis were annular dilation and elongation of the chordae to the anterior leaflet resulting in MR with a posterolaterally directed jet.TTE studies have shown that patients with ARF and congestive heart failure have preserved left ventricular systolic function and severe MR

26Endomyocardial BiopsyThe presence of Aschoff nodules on histologic specimens obtained at surgery and autopsy can be considered diagnostic of ARF. Aschoff nodules and interstitial mononuclear infiltrates with or without myocyte necrosis have been described in the myocardial biopsy specimens of a few patients with ARF. 278. DIAGNOSISCriteria by Duckett Jones has been modified and updated by AHABased on their diagnostic importance, clinical and laboratory findings are divided into major and minor manifestations. If supported by evidence of a preceding group A streptococcal infection, the presence of two major manifestations, or of one major and two minor manifestations, indicates a high probability of ARF. 28Supporting evidence of a previous group A streptococcal infection is a prerequisite for fulfilling the criteria. In areas of low ARF incidence, the Jones criteria are perhaps most useful in excluding the diagnosis. 8. DIAGNOSIS299. TREATMENTAntibiotics to eradicate group A streptococci remaining in the pharynx and tonsils is usually given. Penicillin G benzathine (1.2 million U intramuscularly as a single injection) is the treatment of choice for patients who are not allergic to penicillin. Erythromycin is prescribed for the penicillin allergic patient. 30Aspirin is very effective in reducing fever, toxicity, and inflammation of the joints, dosage of 90 to 100 mg/kg per day in children and 6 to 8 g/day in adults in divided doses, every 4 h. Adverse effects include salicylism and gastrointestinal bleeding. The precise dose of aspirin is determined by the severity of symptoms, clinical response, salicylate levels, and tolerance to the drug. 9. TREATMENT31Corticosteroids are used in patients with carditis manifested by heart failure and in patients who do not tolerate aspirin or whose symptoms do not respond well to this drug. Prednisone 40 to 60 mg/day in divided doses is given for 2 to 3 weeks, and the dosage is gradually reduced over the following 3 weeks.9. TREATMENT3210. PROGNOSISManifestations of chronic rheumatic heart disease include MR and aortic regurgitation or stenosis, congestive heart failure, and atrial fibrillation.33PreventionThe risk of developing ARF following a symptomatic or asymptomatic streptococcal infection is much higher in patients who have experienced a previous attack than it is in those who are nonrheumatic. In patients with rheumatic heart disease, recurrent attacks lead to progressive damage.

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36Thank You37Bacterial cultures are sterile. Inflammation of any one joint subsides spontaneously within a week, and the entire bout of polyathritis rarely lasts < more than 4 weeks. 38Discomfort in the extremities, borderline temperature elevation, increased intensity of functional murmurs, tachycardia, elevated erythrocyte sedimentation rate, and prolonged PR interval may occur in the absence of major manifestations.

8. DIAGNOSIS39Congestive heart failure is usually caused by left ventricular volume overload associated with severe MR or AR.In the presence of pericarditis, a pericardial friction rub or muffled heart sounds due to a large effusion may be noted. The presence of effusion should be confirmed by echocardiography. 40TTE has some incremental diagnostic value when added to the clinical findings in the diagnosis of Carditis, but management is not altered and prognosis appears to be very good in silent carditis.

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