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Rheumatic Fever / Rheumatic Heart Disease

Definition

Rheumatic fever is an auto-immune inflammatory process that occurs as a result of infection with streptococcus bacteria ("strep infection"). The inflammation typically occurs following throat infection ("strep throat"), though fortunately only occurs in less than 3% the population. The inflammation in rheumatic fever may involve the brain ("nervous system"), joints, skin, and heart. When the inflammation affects the heart, rheumatic heart disease results. Not all cases of rheumatic fever result in rheumatic heart disease. Not all types of streptococcal bacteria that cause strep throat can cause rheumatic fever. Rheumatic fever appears more prevalent in areas of extreme crowding and low socioeconomic status.   

 

Signs/Symptoms

The presentation of rheumatic fever is variable. The constellation of signs and symptoms required to make the diagnosis include arthritis, carditis, subcutaneous nodules, rash, and Sydenham chorea. 

- arthritis: the arthritis is typically symmetrical and involves large joints such as the knees, ankles, elbows, and wrists. Multiple joints can be involved simultaneously. The arthritis includes swelling, tenderness, warmth, and redness overlying the joint. The arthritis typically resolves in 4 weeks with no permanent damage.

- carditis:inflammation of the heart has been estimated to occur in approximately 30-60% of cases of rheumatic fever. symptoms included shortness of breath, particularly with activity or while going to sleep. Chest pain may also occur.

- subcutaneous nodules: "cutaneous" means skin. Therefore, these nodules are small bumps that tend to appear over bony surfaces, such as the elbows, knees, wrists, ankles, Achilles tendon, and spine. They typically appear a few weeks after the initial infection and persist for 1-2 weeks after treatment. These nodules are not specific to rheumatic fever and may be seen in other medical conditions too. 

- rash:the skin rash in rheumatic fever is called "erythema marginatum". This is an "serpiginous, evanescent rash", meaning that it spreads from one area of the skin to another, much like a snake slithers across the ground in a tortuous fashion. This rash typically travels fast, sometimes completely disappearing within ten minutes. 

- Sydenham chorea: this neurologic condition consists of a constellation of signs that results from inflammation of the nervous system. It may include emotional lability, personality changes, muscular weakness, and uncoordinated, involuntary, purposeless movements. The latter can involve the muscles of the face, hands, and feet, manifesting itself as difficulty with speech, with walking, or with grasping objects (particularly holding onto objects for an extended period of time). These movements do not typically occur while the patient is sedated, or asleep.  

Diagnosis

The diagnosis of rheumatic fever is based on the patient's history of illness, findings on physical examination, and results of certain laboratory tests. This constellation of historical and physical data have been classified into major and minor criteria.

"Major" criteria include those listed above (arthritis, carditis, subcutaneous nodules, rash, Sydenham chorea).

"Minor" criteria include arthralgia (joint pain - not arthritis, which implies inflammation of the joint), fever, bloodwork suggestive of increased inflammation (elevated ESR or CRP - "inflammatory markers"), and abnormalities on ECG.

Confirmation of a recent infection with streptococcus bacteria is required, and typically involves a throat swab ("rapid strep test", throat culture) and/or bloodwork (ASLO titers - these represent antibodies that our body produces specifically in response to the streptococcus bacteria). In addition, the patient must have either two Major criteria or one major and two minor criteria to fulfill the diagnostic criteria. One exception to this rule includes the presence of Sydenham's chorea alone.

Treatment

Treatment of rheumatic fever includes antibiotics in addition to supportive measures including anti-inflammatory medications such as acetaminophen or ibuprofen. The antibiotic of choice is penicillin, although other agents can be used in patients who are allergic to penicillin. Anti-inflammatory agents are used to control fever and the underlying inflammatory process. Rarely, specific heart medications may be needed if the  carditis is severe enough, causing congestive heart failure; this includes medications that remove excess fluid from the lungs that result in congestive symptoms, and possibly medications to control changes in heart rhythms.

 

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