Rheumatic Fever - Long Case Study

Presenting complains:  18 years old female presents with the complains of:
- Migrating polyarthritis involving bigger joints (knee, ankle, elbow) for … days
-  Fever for … days
- Palpitation, chest pain … for days
- Malaise, weakness, fatigue for … days

History of presenting complains: According to the patient’s statement, she was quite fit and well … days back. Then she suffered from sore throat from which she recovered completely within a few days. After … days, she developed severe joint pain. Initially, it involved the right knee joint, but then sequentially the right ankle, left knee, left ankle and elbow joints were involved. The joints are
swollen, red and very painful, even with mild movement. The smaller joints are not involved. There
was no morning stiffness.
The patient also complains of fever, which is high grade, continued and reduces with antipyretic
drugs. It is not associated with chill and rigor, but there is profuse sweating. She also complains of
palpitation, chest pain, malaise, fatigue, weakness during her disease period. There is no history of
abnormal or involuntary movement (chorea) or skin changes. Her bowel and bladder habits are
normal. She denied any history of diarrhea, sexual exposure, skin rash, mouth ulcer, uveitis or any
urinary complaint.

General Physical Examination: 
- Appearance: Ill looking
-  Built: average
- Nutrition: average
-  Anemia: mildly anemic
- No jaundice, cyanosis, clubbing, leukonychia, koilonychia, edema or dehydration.
-  No lymphadenopathy, thyromegaly, etc

Vitals:
- Pulse: 110/min
-  BP: 130/75 mm Hg
-  Temperature: 39°C
-  Respiratory rate: 24/min

Cardiovascular examination ; Normal with no murmurs audible.

Loco-motor examination: (Knee joint involved)
Swollen and red with increased local temperature. Extremely tender on  palpation and restricted movements due to pain.

Other systemic examination ; Normal

Provisional Diagnosis: Acute Rheumatic Fever

Differential Diagnosis: 

• Infective arthritis (viral or bacterial)
• Juvenile chronic arthritis (juvenile <16 years)
• SLE
• Seronegative arthritis (reactive arthritis, Reiter’s syndrome)
• Rheumatoid arthritis.

Why do you think this is a case of rheumatic fever?
This young patient has a history of sore throat followed by a latent period and then she developed
migrating, inflammatory polyarthritis involving the large joints without any deformity. All these are
in favor of acute rheumatic fever.

Why not this is a case of SLE?
Because , in this case, arthritis is fleeting is nature involving the bigger joints that is not common
in SLE. Also, other criteria of SLE like skin rash, butterfly rash, mouth ulcer, alopecia, menstrual
irregularity, etc. are absent.

What investigations will you do in this patient?
As follows:

• Hb, TC, DC, ESR (high ESR and leukocytosis)
• C-reactive protein (CRP is high)
• Antistreptolysin O (ASO) titre (may be high, in adult > 200, in children > 300)
• Throat swab culture (to find Streptococcus beta hemolyticus)
• Chest X-ray (cardiomegaly, pulmonary edema may be present)
• ECG
• Echocardiography (to see valve abnormality and cardiomegaly)
• Others—RA factor (to exclude rheumatoid arthritis) and ANA (to exclude SLE), if needed.

What is rheumatic fever? What is the mechanism or pathogenesis?

Rheumatic fever is a multisystem disorder, occurring as a sequelae to pharyngitis by group A b

hemolytic Streptococcus. It is due to autoimmune reaction between the antigen (M protein) of

Streptococcus b hemolyticus and cardiac myosin and sarcolemal membrane protein (laminin). As a

result, antibody is produced against streptococcal enzyme, causing inflammation in the endocardium,

myocardium and pericardium as well as joints and skin. There is formation of “Aschoff’s nodule”

in heart, which is pathognomonic of rheumatic fever.

 

What are the usual presenting complaints of a patient with rheumatic fever?

Rheumatic fever (RF) usually occurs in children and young adults. Peak incidence is 5 to 15 years. It usually recurs unless prevented. There is usually a history of sore throat by group A b hemolytic streptococcus 1 to 3 weeks prior to the fever.

Features are:

• Migrating (fleeting), non-deforming polyarthritis involving the large joints (knee, ankle and elbow) and wrists with fever, which may be continuous, high grade is the presenting feature in 75% cases.
• Palpitation and chest pain (due to carditis in 50% cases).
• Skin rash (erythema marginatum), subcutaneous nodules.
• Involuntary movement (chorea in 10 to 30% cases).
• Malaise, weakness and fatigue.

Which joints are commonly involved in acute rheumatic fever?

Commonly large joints like ankle, wrist, knee and elbow (usually does not involve small joints of the hands and feet, rarely involves hip joint).

What are the signs of carditis?

Rheumatic fever can cause carditis involving all the layers of the heart (endocardium, myocardium

and pericardium), called pancarditis.

Signs of endocarditis:

• Soft heart sounds
• Pansystolic murmur (due to MR)
• Mid diastolic murmur (Carey Coombs murmur)
• Early diastolic murmur (due to AR which is due to valvulitis with nodules on the valve).

Signs of myocarditis:

• Tachycardia
• Soft heart sounds, S3 gallop
• Cardiomegaly
• Features of heart failure.

Signs of pericarditis:

• Pericardial rub (patient usually complains of chest pain)
• Pericardial effusion may be present

What is ‘Aschoff’s nodule’?

It is a granulomatous nodule composed of central fibrinoid necrosis and multinucleated gaint cells, with surrounding macrophages and T-lymphocytes. It occurs throughout the heart and is common in the interstitial tissue close to the small blood vessels situated beneath the endocardium of the left ventricle. It is pathognomonic of RF.

What is erythema marginatum?

It is a transient, geographical type rash with pink or red raised edges, round margin and clear center. It may coalesce into crescent or ring shaped patches. The rash blanches on pressure. It is found mostly on the trunk and proximal limbs (not in face). It occurs in about 10% cases.

What is subcutaneous nodule?

These are small, mobile, firm, painless, pea-shaped nodules, felt over bony prominences, tendons or joints on the extensor surface. Occurs in 10 to 15% cases.

What is Sydenham’s chorea (St Vitus’ dance)?

It is a neurological manifestation of acute RF, which usually occurs after 3 months of an acute attack, when almost all other signs have disappeared.

• It occurs in one-third of cases, common in children and adolescents, especially in female of 5 to 15 years of age.
• Usually associated with emotional instability, irritability, inattentiveness and confusion.
• It may occur without any feature of acute RF. Carditis is common and may be the first manifestation. 
• Speech may be explosive and halting.
• ESR, ASO titre and CRP are usually normal.
• Rheumatic chorea is usually self-limiting, and recovers within few months.
•  Relapse may occur only in few cases, occasionally during pregnancy (called chorea gravidarum) or in those who use oral contraceptive pill.
• Treatment—sedation (haloperidol) along with other treatment and prophylaxis of rheumatic fever.
• 25% of cases develop chronic rheumatic heart disease in course of time

How to treat acute RF?

As follows:

1. Complete bed rest (until disease activity resolves).

2. Oral phenoxymethylpenicillin 250 mg 6 hourly for 10 days or single injection of benzathine penicillin 1.2 million units, deep IM in the buttock (to eliminate the streptococcal infection).

Erythromycin may be given if allergic to penicillin.

3. Analgesic (to relieve pain). Aspirin 60 mg/kg per day in divided doses. Higher dose may be required.

4. Other treatment:

- If there is carditis or severe arthritis, corticosteroid should be given (prednisolone 1 to 2 mg/ kg daily).

- If there is chorea, diazepam (for mild case) or haloperidol (in severe case) should be given.

-  If there is erythema marginatum or subcutaneous nodules, no treatment is necessary.

5. Treatment of complications like cardiac failure, valvular lesion, heart block, arrhythmia, etc. if needed.

What is the prophylactic treatment of RF? How long should it be continued?

Recurrence is common in patient who had carditis during initial episode. In children, 20% recurrence occurs within 5 years. Recurrence is uncommon after 5 years and in patient over 25 years of age.

To prevent recurrence, oral phenoxymethylpenicillin 250 mg 12 hourly or injection benzathine penicillin 1.2 million units deep IM in the buttock every 4 weeks should be given. In penicillin sensitive patient, erythromycin (250 mg 12 hourly) or sulfadiazine (1 g daily) may be used.

Prophylactic drug should be continued up to 21 years of age or 5 years after the last attack (recurrence after 5 years is rare), whichever comes last. After this, antibiotic prophylaxis should be

given for dental or surgical procedure. However, in high-risk streptococcal infection or if the attack occurs in the 5 years or patient lives in high area of prevalence, treatment may need to be extended.

If there is documented recurrence or documented rheumatic valvular heart disease, life-long prophylaxis should be considered.

What is the prognosis?

Acute attack may last up to 3 months, but recurrence may be precipitated by streptococcal infections, pregnancy, use of oral contraceptive pill, etc. 60% patients with carditis develop chronic rheumatic heat disease. Mitral valve is most commonly involved followed by aortic valve. Tricuspid and pulmonary valves are rarely involved. Usually, regurgitation of cardiac valves develops during acute attacks, while stenosis develops years later.

What are the causes of migrating polyarthritis?

As follows:

• Rheumatic fever
• Septicemia
• Gonococcal arthritis
• Syphilitic arthritis
• Lyme arthritis
• Hyperlipidemia (type 2)
• SLE.