RHEUMATIC FEVER

RHEUMATIC FEVER

DEFINITION

RHEUMATIC FEVER……. It is an acute, subacute and chronic systemic disease involving the connective tissue throughout the body characterized by fleeting polyarthritis and great tendency to carditis which is usually a sequel to tonsillo-pharyngeal infection by streptococcus haemolyticus. The streptococci belong to group A of lancefield (GAS). Through the incidence of the RF has sharply declined in many developed countries it still remains a major problem in many developing countries. RHEUMATIC FEVER

AETIOLOGY

  1. SEASON: commonly seen during autumn and winter months.
  2. CLIMATE: It chiefly affects people in the temperate climates, comparatively less common in tropics. But this is not the position now.
  3. FAMILY HISTORY: the disease may run in families which is often due to poor hygienic conditions.
  4. SOCIAL HISTORY: It is a disease of poverty and usually affects persons of the lower income group.
  5. SANITARY CONDITION: The disease is often seen amongst people living in the bustee area where there is overcrowding, low sanitary condition and dampness.
  6. UPPER RESPIRATORY INFECTION: The disease is usually preceded by upper respiratory infection commonly tonsillitis or pharyngitis 1-3 weeks before the attack.
  7. AGE: Common in lower age group (5 years to 15 years); rare before 4 years and after 50 years.
  8. SEX: It is equal in both sexes but when chorea is present it is common in females.
  9. AUTO-IMMUNE THEORY: Antimyocardial antibodies may react with the sarcolemma and sub-sarcolemmal myofibril particularly at the intercalated discs and may have an antigen-antibody reaction with the antigen on the streptococci. Through anti-myocardial antibody have been demonstrated in about seventy per cent cases of acute rheumatic fever this theory has not been widely accepted.
  10. Deficiency of blood group substance in the saliva may predispose to rheumatic fever.
  11. SUSCEPTIBILITY/IDIOSYNCRASY: Only 3% of people develop rheumatic fever in streptococcal epidemics. Higher number of patients are non-secretors of ABO blood groups.

The main characteristic lesion consists of perivascular granulomatous lesion associsted with vasculitis. RHEUMATIC FEVER

PATHOLOGY

Through widespread involvement of connective tissue id seen in the disease, cardiovascular system is mainly affected usually in the form of pancarditis, i,e., endocarditis, myocarditis and pericarditis. There are two types of reactions—proliferative and exudative. Proliferative reaction consists of formation of Aschoff;s body which is most characteristically seen in myocardium and is called myocardial Aschoff body. These are fusifrom lesions with central area of fibrinoid degeneration consisting of swelling, fragmentation, hyalinization and acidophilic staining. This is surrounded by histiocytes mainly multinucleated, called Aschoff’s cell, inflammatory cells like lymphocutes, monocytes and polymorphonuclear leucocytes. These rheumatic granulomata are found throughout the body such as heart, blood vessels, trachea, pharynx, oesophagus, joint capsule, bursea, tendon sheath, etc. These Aschoff bodies are converted to spindle shaped or triangular scars in course of time. Rheumatic endocarditis produces lesions along the line of closure of heart valves with the formation of rheumatic verrucous vegetation which leads to fibrosis and different types of valvular defects. The mitral valve in 75%-80% cases, aortic valve in 30% (but rarely alone), tricuspid and pulmonary valves in less than 5% of the cases are affected. Rheumatic pericarditis is usually a serofibrinous inflammation which may lead to calcification but no pericardial constriction. Extracardiac lesions involve the joints, subcutaneous tissue, lungs, pleura and brain. The joint lesion is mainly exudative, that is why there is no scarring. The extracardiac lesions are not definite and characteristic. RHEUMATIC FEVER

CLINICAL FEATURES:

Onset is usually acute in about 70% of the cases but may be insidious in the remaining cases.

SYMPTOMS:

  1. FEVER

Temperature remains usually high up to 39’C which is usually of remittent or intermittent in character. Some degree of fever is always present so long as the rheumatic activity persists. The fever is not associated with chill and rigour. Occasionally hyperpyrexia may occur as a complication. RHEUMATIC FEVER

  1. ARTHRITIS:

It is seen in 70% of the cases and is characteristically fleeting or migratory polyarthritis. Big joints are commonly affected and kness, ankles, elbows, wrists, shoulder and hip joints are affected in this order. Small joints may rarely be affected last of all. Temporomandibular and sternoclavicular joints are usually not involved. The affected joint becomes red, hot, painful, swollen and tender, so that all movements of joints are restricted. This persists for a day or two when another joint is involved. Very rarely in adults the disease may be limited to a single joint. The affected joint improves after 1-5 weeks without any residual deformity except rarely in Jaccoud’s arthritis, where there may be minor deformities of the hand and feet. The joint swelling is mainly periarticular. Though the joint fluid is very much viscid with high protein content and increased cellularity (mainly polymorphonuclear) it does not suppurate and the culture is sterile. A characteristic feature is promt improvement of arthritis with therapeutic trial of salicylates or non-steroidal agents. RHEUMATIC FEVER

SWEATING:

Sweating is moderate in amount, acrid or offensive in odour

OTHER SYPMTOMS:

These include sore throat, epistaxis, abdominal pain, skin rashes. constitutional symptoms like anorexia, constipation, weakness, vague aches in different parts of the body.

SINGS

. GENERAL:

Age: 5-15 years. Sex: both. Temperature is raised. Pulse shows tachycardia even during sleep. Patient is reluctant to change the posture for fear of joint pain. Tonsils are enlarged, congested with presence of pus points. Tonsillar lymph nodes are enlarged and tender. RHEUMATIC FEVER

. HEART

Cardiac involvement occurs even upto 75% of the cases in the form of carditis particularly seen in children and adolescents. Due to carditis the following features are seen:

SPECIFIC SINGS OF CARDITIS:

  1. a) Apex beat may be slightly shifted outside due to cardiac dilatation which may be confirmed by serial x-rays.
  2. b) Pericarditis: It is seen in 10% of the cases and is usually dry but rarely there may be effusion. In latter cases mortality is very high. Pericarditis does not indicate severe myocarditis. ECG may not be helpful in its diagnosis in many cases and echocardiogram may be helpful. Typical pericardial friction sound is the hallmark of diagnosis of acute pericarditis. It is uncommon in adults. Serial X-rays may show progressive enlargement of cardiac shadow.
  3. c) Mitral and Aortic diastolic murmur: Short apical mid- diastolic murmur (36%), called Carey-Coomb’s murmur and an early aortic diastolie murmur (23%) at the base of the heart may be heard, indicative of dilatation of valve ring or the myocardium with or without concomitant valvitis. Carditis is almost always associated with murmur.
  4. d) Frank congestive cardiac failure: Though rare, left or right ventricular failure may develop but dyspnoea or orthopnoea are not so striking as in adults and enlargement of liver is a valuahle sign.

. LESS SPECIFIC SINGS OF CARDITIS:

In absence of above findings of carditis the following less specific signs should also be considered valuable.

  1. a) ECG changes include prolonged PR interval more than 0.04 second above the patient’s normal expectation and is the most important abnormality. QRS and QT duration may also be prolonged. Changing contour of the P waves and inversion of T waves are also seen.
  2. b) Changing quality of heart sound: First heart sound is usually accentuated due to tachycardia but it may be soft if there is first degree heart block. Tic-tac rhythm, gallop thythm may also be present. Physiological third heart sound in children must not be confused with gallop.
  3. c) Pulse shows sinus tachycardia which is usually disproportionate to the rise of temperature and persists even during sleep and abruptly rises after slight exercise.
  4. d) A loud pansystolic apical murmur (60%) may be heard. However, soft pulmonary systolic murmur is normally present in children and not indicative of abnormality.
  5. e) Cardiac irregularities including ectopic beats and wandering pacemaker may also be present. Subjects having carditis at times may not have any symptom referable to heart.

LUNGS:

Lungs may show evidences of pleurisy in 10% and pneumonia in 2% of cases.

. Nervous System

In 3% of the cases, rheumatic chorea develops which is due to involvement of the basal ganglia and mild encephalitis. In this condition peculiarly arthritis, high ESR or leucocytosis are not seen. It is common in female in the ratio of 3: 1 and rare in adults. This is a very important diagnostic feature.

. Abdomen

Diffuse abdominal pain may be complained of in absence of definite tenderness anywhere which may be attributed to rheumatic peritonitis.

Skin Changes

  1. a) Rheumatic nodules are seen in children and they indicate activity of the rheumatic process. These are non-tender, firm small (S2 cm) nodules found over the bony prominences or around the affected joints. They are usually seen over the knuckles, suboccipital region, scapular margins, joints and shin bones. They persist for days or weeks and are usually relapsing in nature. Pathologically these are aggregation of Aschoff s nodules. These are indistinguishable from Rheumatoid nodules.
  2. b) Erythema nodosum: These are rarer than rheumaticnodules. They are tender nodules chiefly found over the shin bones and thigh. The superlying skin always shows some colour changes day-to-day.
  3. c) Erythema annulare and marginatum: Erythema annulare is specific for rheumatic fever which appears on the trunk and on the proximal parts of limbs. These are erythematous or pinkish rashes with circular, crescentic, or zig-zag margin which persist for a short period. When the margin is raised with clear centre it is called erythema marginatum which occurs in 8% of the cases. Few such patches may conglomerate together forming big irregular rashes. These are either transient or persistent.
  4. d) Other skin rashes: These include maculopapular rashes, urticarial, purpuric spots, sudaminal rashes or miliaria.

JOINTS:

The affected joints are swollen and show all features of inflammation and when they subside no residual deformity is seen. Clinical evidence of fluid in the joint is usually lacking though it is present. Surrounding muscles never show wasting.

Modified Ducket Jones criteria for the diagnosis o Rheumatic fever:

The diagnosis of Rheumatic fever is almost certain when at least one major and two minor or two major and one minor criteria are present.

RHEUMATIC FEVER

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