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Pediatric Arthritis & Other Rheumatic Diseases Rheumatic Fever What is rheumatic fever? Rheumatic fever is a complicated, involved disease that affects the joints, skin, heart, blood vessels, and brain. It is a systemic immune disease that may develop after an infection with streptococcus bacteria, such as strep throat and scarlet fever. What causes rheumatic fever? Rheumatic fever is a delayed, autoimmune reaction to the streptococcus bacteria. It can be prevented with prompt diagnosis of strep throat, and treatment of strep throat with antibiotics. It is uncommon in the US, except in children who have had strep infections that were untreated or inadequately treated. What are the symptoms of rheumatic fever? The symptoms of rheumatic fever usually start about one to five weeks after your child has been infected with streptococcus bacteria. The following are the most common symptoms of rheumatic fever. However, each child may experience symptoms differently. Symptoms may include: • joint inflammation - including swelling, tenderness, and redness over multiple joints. The joints affected are usually the larger joints in the knees or ankles. The inflammation "moves" from one joint to another over several days. • small nodules or hard, round bumps under the skin • a change in your child's neuromuscular movements (this is usually noted by a change in your child's handwriting and may also include jerky movements) • rash - a pink rash with odd edges that is usually seen on the trunk of the body or arms and legs. • fever • weight loss • fatigue • stomach pains Symptoms of rheumatic fever may resemble other medical conditions. Always consult a physician for a diagnosis. Who is at risk for developing rheumatic fever? Children ages 5 to 15, particularly if they experience frequent strep throat infections, are most at risk for developing rheumatic fever. Rheumatic fever is also more common in children who have a family history of the disease. There is an increased prevalence of rheumatic fever in the winter and spring, as strep throats occur more frequently during these seasons. Strep infections are contagious, but rheumatic fever is not. How is rheumatic fever diagnosed? In addition to a complete medical history and physical examination, rheumatic fever is diagnosed by your child's physician based on the presence of criteria found in the revised Jones criteria diagnostic tool (standard guidelines for diagnosis of rheumatic fever): • Major criteria include: o carditis (inflammation of the heart) o polyarthritis (inflammation of more than one joint) o chorea (unusual jerky movements, most often involving the face and hands)

subcutaneous nodules (small, painless bumps under the skin, often over bony areas) o rash (a red, irregular rash on the trunk) • Minor criteria include: o fever o arthralgia (pain in one or more joints) o previous rheumatic carditis (inflammation of the heart) o changes in the electrocardiogram (EKG) pattern o abnormal sedimentation rate or C-reactive protein (laboratory tests performed on blood) The diagnosis of rheumatic fever can be made when two of the major criteria, or one major criterion plus two minor criteria, are present along with evidence of a streptococcal infection. There is no definitive test to diagnose rheumatic fever. Blood work is also usually done to assist in making a diagnosis. Your child's physician may also order an electrocardiogram, or EKG (a test that records the electrical activity of the heart, shows abnormal rhythms arrhythmias or dysrhythmias - and detects heart muscle damage of the heart) as part of the diagnostic process for rheumatic fever. A throat culture may also be done to determine if the child tests positive for streptococcus bacteria, although during the initial phase of rheumatic fever, the throat culture is often negative. Treatment for rheumatic fever: Specific treatment for rheumatic fever will be determined by your child's physician based on: • your child's overall health and medical history • extent of the reaction • your child's tolerance for specific medications, procedures, or therapies • expectations for the course of the reaction • your opinion or preference Children with rheumatic fever are often treated in the hospital, depending upon the severity of the disease. Treatment for rheumatic fever, in most cases, combines the following three approaches: 1. treatment for streptococcus infection The immediate goal is to treat the infection with antibiotics. This is done even if the throat culture is negative. Following the initial treatment for strep infection, your child may continue to receive monthly doses of antibiotics to help prevent further complications. 2. anti-inflammatory medications Based on the severity of your child's condition, your child's physician may prescribe medications to help decrease the swelling that occurs in the heart muscle, as well as to relieve joint pain. 3. bed rest The length of bed rest will be determined by your child's physician, based on the severity of your child's disease and the involvement of the heart and joints. Bed rest may range from two to twelve weeks. Are there any complications from having rheumatic fever? o

Depending on the severity of the initial attack of the disease on the heart, some children may develop heart disease. Physical activity and sports may be restricted in your child, based on your child's physician's findings. Also, if your child had heart involvement during the initial course of rheumatic fever, he/she will need to receive antibiotics before having dental work done. This helps decrease the chance of infection migrating to the heart during the dental procedure. Consult your child's physician for more information. Can my child develop rheumatic fever again? Yes, although the chances are reduced because of the use of antibiotics after the initial disease process. The greatest chance of recurrence is during the first three years. The chance of developing the disease again decreases with age and time since the first attack. After having rheumatic fever, your child will need medications on a monthly basis to help decrease the chance of developing rheumatic fever again. Usually by the time your child is 18 and your child's physician feels he/she is not at risk for developing heart disease, the antibiotic therapy may be stopped. Close follow-up with your child's physician is needed