causes, symptoms, diagnosis, treatment, complications

The disease usually develops two to four weeks after a strep throat infection

The heart is involved in about half of the cases. Damage to the heart valves, known as rheumatic heart disease (hence the acronym “RHD”), usually occurs after repeated attacks, but can sometimes occur after just one.

Damaged valves can cause heart failure, atrial fibrillation and valve infection.

Acute rheumatic fever can occur as a result of infection of the throat with the bacterium streptococcus pyogenes (“group A β-hemolytic streptococcus”)

If the infection is not treated, rheumatic fever affects up to 3% of people.

The underlying mechanism is thought to involve the production of ‘self’ antibodies, ie antibodies mistakenly directed against certain tissues in the body (autoimmune disease).

Diagnosis of RF is often based on the presence of signs and symptoms associated with evidence of recent streptococcal infection.

Treating people with streptococcus with antibiotics, such as penicillin, reduces the risk of developing rheumatic fever.

To avoid misuse of antibiotics, it is important to be certain of the bacteria present in the respiratory tract.

Other preventive measures include improving hygienic conditions.

In people with rheumatic fever and rheumatic heart disease, prolonged periods of antibiotics are sometimes recommended.

After an attack, there may be a gradual return to normal activities.

Once rheumatic heart disease develops, treatment becomes more difficult.

Sometimes valve replacement surgery or valve repair is needed.

Rheumatic fever is so called because its symptoms are similar to those of certain rheumatic disorders

The first descriptions of a disease similar to rheumatic fever are thought to date back to at least the 5th century BC in the writings of Hippocrates.

Acute articular rheumatism was certainly the most widespread rheumatic disease until the end of the Second World War.

Later, thanks to the spread of antibiotics and the improvement of social and economic conditions in Western countries, its occurrence decreased considerably.

In the second half of the 20th century, the incidence was one case per 1000 inhabitants per year.

Rheumatic fever affects approximately 325,000 children each year and approximately 33.4 million people currently suffer from rheumatic heart disease.

People who develop rheumatic fever are most often between 5 and 14 years old, with 20% of the first attacks occurring in adults.

It affects both sexes equally.

The disease is more common in developing countries and among indigenous populations in the developed world, where it remains a public health problem and incidence reaches 100 cases per 100,000, while in places like Australia or the Eastern European states, it is generally over 10 cases per 100,000.

In 2015, it caused 319,400 deaths compared to 374,000 in 1990.

Most deaths occur in developing countries, where up to 12.5% ​​of those affected may die each year.

Currently in Italy, thanks to the increase in socio-economic well-being, the occurrence of this disease has decreased considerably to 1 case per 100,000 people.

Causes of rheumatic fever

The origin of the disease lies in a pathogen localized in the throat which causes pharyngo-tonsillitis: group A β-hemolytic streptococcus.

If this is not treated adequately, the risk of contracting the disease increases.

There is also a greater familial predisposition in some individuals.

Manifestations of the disease are due to inflammation of the valve tissue, which leads to a decrease in the capacity of the heart valve (valvular insufficiency) and an increase in the chemotaxis of lymphocytes to it.

The causes are to be sought in an autoimmune-type condition: recurrent and frequent states of angina-pharyngitis lead to sensitization of the subject against antigenic molecules carried by Streptococcus, which leads to a cross-reaction against common epitopes, from the self , at the cardiac level , location of joints and blood vessels.

That is, antibodies, especially IgGs produced against antigens carried by the bacteria, also interact with molecules structurally similar to bacterial antigens, even causing irreversible damage.

Risk factors

Due to their genetics, some people are more likely than others to contract the disease when exposed to the bacteria.

Besides familiarity, other risk factors include:

  • low socioeconomic status,
  • malnutrition by default,
  • poor hygiene,
  • poverty,
  • frequent respiratory infections.

Symptoms and signs

Signs and symptoms include fever, multiple joint pains, involuntary muscle movements, and sometimes a characteristic non-itchy skin rash known as “erythema marginatum”.

Other symptoms include drowsiness, fatigue, abdominal pain, anorexia and epistaxis (“nose bleeds” seen in 4% of children).

Data on symptoms and clinical signs were first compiled in 1944 by Jones TD, and subsequently reviewed by other groups.

Thus, major and minor criteria necessary for the establishment of diagnoses have been developed, which must support either 2 major criteria, or a major criterion and 2 minor criteria to be valid; in the latter possibility, these criteria must be accompanied by the demonstration either of a recent streptococcal infection, which can also be demonstrated by a throat swab, or of a positive antistreptolysin titer.

Major criteria

  • Rheumatic heart disease (endocarditis, myocarditis, pericarditis, identifiable in 50% of cases) very often associated with the appearance of heart murmurs (by aortic insufficiency and mitral insufficiency), with various forms, the most severe of which can lead to the death of the patient .
  • Sydenham’s chorea (10% in children), formerly known as Saint Vitus’ dance, occurs late, even months after the onset of the disease, and leads the patient to make involuntary movements.
  • Marginal erythema, which rarely occurs on the trunk and does not itch.
  • Polyarthritis, a migratory arthritis that mainly affects the large joints (knees, ankles, shoulders, etc.), which is the most common manifestation (70%). It responds well to salicylates but prolongs its duration by a few weeks if left untreated.
  • The subcutaneous nodules (or rheumatic nodules of Meynet), of small size (varying in volume from a lentil to a hazelnut), which are localized on the extensor surfaces of the joints, are mobile, not painful and transient.
  • The acronym JONES is used to recall these criteria and derives from the initials of the English words: Joints (joints, polyarthritis), O (where the ‘O’ represents the heart, thus indicating carditis), Nodules (cutaneous nodules), Erythema marginatum (erythema marginatum), Sydenham’s chorea (Sydenham’s chorea).

Minor criteria

  • fever,
  • arthralgia,
  • high ESR,
  • PCR positivity,
  • lengthening of the PR path on ECG,
  • previous episode of rheumatic fever.

Rheumatic fever, diagnosis

Jones’ main criteria still remain valid, although they are changed and updated very often.

Tests useful for diagnosis are:

  • blood tests, where the ESR appears elevated;
  • electrocardiogram, where certain arrhythmias or conduction blocks are found (first degree atrioventricular block);
  • pulmonary radiography;
  • echocardiography with colordoppler, showing insufficiency of one of the heart valves, in particular the mitral and the aortic;
  • endomyocardial biopsy;
  • throat swab (showing infection with group A beta-hemolytic streptococcus);
  • search for antibodies directed against streptococcal antigens (antistreptolysin titer).

The differential diagnosis arises in relation to:

  • infective endocarditis;
  • rheumatoid arthritis;
  • septic arthritis;
  • Hepatitis B;
  • rubella.


The management of rheumatic fever aims to reduce inflammation with anti-inflammatories such as aspirin or corticosteroids.

In addition to forced rest and a controlled diet in case of carditis, drug treatment and, in severe cases, surgical treatment are also provided.

To combat the infection, penicillin V or G is used, which must be taken for at least 10 days.

Primary prophylaxis serves to avoid complications of pharyngo-tonsillitis and to prevent rheumatic diseases.

Other medications:

  • erythromycin, 250 mg, every 6 hours, always for 10 days
  • prednisone, 40-60 mg (for carditis)
  • Penicillin: phenoxymethylpenicillin 250 mg (to be administered every 6 hours).

It is only in certain cases, the most serious, that surgical valve replacement is necessary, whereas corrective surgery does not always give positive results.


No vaccine is currently available to protect against S. pyogenes infection, although research is underway to develop one.

Difficulties in vaccine development include the wide variety of S. pyogenes strains present in the environment and the large amount of time and people that will be required to adequately test the safety and efficacy of the vaccine.


Some patients develop significant carditis that manifests as congestive heart failure.

This requires the usual treatment for heart failure: ACE inhibitors, diuretics, beta-blockers and digoxin. Unlike typical heart failure, rheumatic heart failure responds well to corticosteroids.


The prognosis is positive in almost all cases within two months of its onset. The symptoms regress until they disappear.

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