Acute Rheumatic Fever


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Acute Rheumatic Fever

  1. 1. ACUTE RHEUMATIC FEVER Definition: Acute rheumatic fever is defined as a systemic illness characterized by fever polyarthritis and carditis. The importance of the disease lies in the fact that after an interval of several years damage to the heart valves may become manifest in the patient leading to chronic disability and even fatal outcome arising out of one of the complications. It is mainly an infection prevalent in the young age. It is still very common in the developing countries where as it has been controlled in developed countries. Etiology: It is caused by a delayed antigen antibody reaction to infection with group A B hemolytic streptococcal infection of the throat. The patient develops the throat infection first in the form of a pharyngitis or tonsillitis by streptococci, which might subside within a week or so with or without treatment. These forms of upper respiratory infections are very common in young children living in circumstances of poor hygiene and over-crowding. But only a small proportion of these children develop acute rheumatic fever 2-4 weeks afterwards. It is important to note that similar infection caused to the skin in the form of infected scabies or pyoderma often leads to acute glomerulonephritis and not acute rheumatic fever. Symptoms: The child usually presents with sudden onset of high grade and continuous fever and painful swelling of one or more major joints of the body. The usual joints that are affected are the elbow, wrist, knee and ankle. The involvement is symmetrical and usually spares the smaller joints unlike in rheumatoid arthritis, which is the immediate differential diagnosis. The characteristic pattern of involvement is called flitting or migratory. This means that as one joint involvement is decreasing another joint gets affected. At any point of time two or three joint will be affected. This is also unlike in rheumatoid arthritis where the involvement is uniformly symmetrical and simultaneous. There is painful restriction of mobility and the patient may remain bedridden for weeks together. After one or two weeks the patient may develop another major manifestation termed as carditis. This is a pancarditis meaning inflammation of all the layers of the heart namely the pericardium, myocardium and the endocardium. The patient will then complain of chest pain, palpitation and dyspnoea along with fever. Some patient may develop pitting pedal edema of both legs due to development of cardiac failure The next major manifestation is rheumatic chorea, otherwise called as Sydenham’s chorea. This is usually seen in young children with rheumatic fever. It manifest in the form of repeated but unintentional movements of the upper limbs producing bizarre jerky movements, which are also done often without any purpose. The child also is irritable and restless and fidgety and always moving his torso except during sleep. This form of involuntary movements are very important because the chance of developing a carditis soon and valvular heart
  2. 2. disease later is much more in these patients than in rest of the patients without chorea. The fourth major manifestation of acute rheumatic fever is erythema marginatum. These are usually seen only in fair skinned people. It is in the form of reddish macular lesions with raised edges and blanching center. They may later coalesce and become bigger. They are mainly seen over the ankle joints and over the shin. The fifth major manifestation is sub-cutaneous nodules. These are seen mainly over the extensor aspects of the limbs and over the tendons. These are small round firm and non-tender nodules. They are smaller than the nodules seen in rheumatoid arthritis. The minor clinical manifestations of importance are fever, polyarthralgia in the absence of polyarthritis and the previous history of streptococcal infection of the throat. Signs: The child is usually sick, febrile and bed-ridden. There will be fever of high grade. Tachycardia, which is sometimes out of proportion to the fever and attributed to the carditis, is usually prominent. Normally the heart rate increases by eighteen beats per minute for every degree rise in Celsius temperature. Here it may be much more than that. Blood pressure is maintained normally in most of the cases and the jugular venous pressure may also be normal, unless there is cardiac failure. The heart size is usually enlarged as evidenced by a downward and outward displacement of the apex. The appearance of a new murmur or the change in characteristics of an already existing murmur is very suggestive of rheumatic valvulitis. The usual murmurs are those due to mitral regurgitation and aortic regurgitation. The former is a pan-systolic murmur and the latter an early diastolic murmur. Some times there can be amid-diastolic rumble heard due to valvulitis of the mitral valve producing obstruction to blood flow across the mitral valve. This murmur, which might disappear later is called Carey Coombs murmur Investigations: The investigations to be done in a suspected case of acute rheumatic fever are important because the diagnosis is also based on them. These are the routine blood examination, which may reveal leucocytosis, raised erythrocyte sedimentation rate and an increase in the C reactive protein levels all of which indicate acute inflammation. The electrocardiogram will reveal tachycardia, evidence of cardiac chamber enlargement and evidence of first degree hear block, which manifests as a prolongation of the PR interval or second-degree heart block, which manifests as periodically dropped beats. The X-Ray of chest will also show features of cardiomegaly and pulmonary venous congestion. Diagnosis: The diagnosis of acute rheumatic fever is made depending on a mixture of clinical signs and investigations. This is now based on the revised Jones criteria. Two or more major manifestation or one major and two or more minor
  3. 3. manifestations are essential for the diagnosis. The minor manifestations included in the list among investigations are leucocytosis, high ESR, increased C Reactive Protein and the presence of first degree or second degree heart block in the electrocardiogram, In addition to these an evidence of previous streptococcal infection of the throat in the form of raised ASO titre or positive throat swab culture must be there to arrive at a diagnosis. Treatment: Bed Rest: The child is given complete bed rest during the acute phase of rheumatic fever. This is important in not only preventing relapses, but also in reducing the incidence of carditis and permanent valvular damage. The bed rest must be continued for 2 to 4 weeks only after the clinical and investigation parameters like the ESR and C reactive protein have fallen to normal ranges. Aspirin: Children and young adults, when given in large doses best tolerate acetyl salicylic acid in enteric-coated forms. Two to three tablets of 325mg are given 4 to 5 times a day after food and along with antacids and H2 receptor blockers like ranitidine in order to minimize the gastric irritation. This dose is continued till the ESR falls to normal and then slowly tapered off. Corticosteroids: Corticosteroids are indicated in presence of severe arthritis and carditis. Prednisolone is the drug of choice and it is given in the dose of 1mg per kg body weight per day in two or three divided doses along with food. Penicillin: Treatment to eradicate infection of the throat with streptococci is important. This can be achieved by a course of crystalline penicillin 10lakh units given IM or IV 6 hrly for 7 days followed by oral administration of phenoxymethyl penicillin 500mg twice daily for ten days. There after for prevention of recurrence it is advised to continue the oral penicillin on a daily basis till the patient reaches the age of twenty-five years or in case of carditis thirty years or at least five years after the last attack of acute rheumatic fever