Understanding Juvenile Rheumatoid Arthritis

Published in City Times



Understanding Juvenile Rheumatoid Arthritis

It may begin as an unexplainable rash, a swollen knuckle or a spiking fever in a child. Most likely, the condition is one of several types of Juvenile Arthritis. Juvenile Rheumatoid Arthritis is one of the most prevalent forms of juvenile arthritis, affecting 1 in every 1000 children, reveals Dr.Bassel F.Darwish, Specialist Rheumatologist, Welcare Hospital, Dubai. The key point is: JRA is not very common, but it is not very rare, either. So the key to treatment is going to the right specialist. Usually, a good pediatrician will be right away able to suspect the disease. Diagnosis should not be delayed and referral to a rheumatologist should be made early.

What is JRA and what happens in the condition?
JRA is a condition that causes inflammation in one or more joints and typically begins between the ages of 6 months to 16 years. JRA is an autoimmune disease in which the immune system loses its ability to tell the difference between the body's own healthy cells and tissues and harmful bacteria and viruses that invade the body. The immune system, releases chemicals that damage the healthy tissues, causing inflammation and pain.

What are the factors that contribute to JRA?
The causes of the disease are not known for certain but there appears to be a genetic predisposition to the condition in some types of childhood arthritis. Some features of the disease suggest that there may be infectious triggers in a genetically predisposed child, but no specific infectious cause has been identified.

How does JRA manifest and what are the early symptoms of the condition?
There are several patterns of JRA and though all have joint inflammation, they behave very differently, present in different ways with different symptoms, require different treatment and even have different outcome. The type of JRA a child has is generally determined by the pattern of symptoms that occurs within the first 6 months after symptoms appear. Though there are several forms of the disease, oligoarticular or pauciarticular, polyarticular and systemic arthritis are the three main categories of JRA, into which most cases fall. While inflammation and pain in the joints are common to all forms of JRA, children with JRA may not really complain of pain as adults do, for reasons unexplained.

Oligoarticular or Pauciarticular tends to affect more girls than boys, especially younger girls between the ages of 1 - 7 years. This form of JRA affects fewer than four joints within the first six months of the disease, whenever it appears. While any joint can be affected with pain and inflammation, the wrist and knee joints are the most commonly affected. In a good percentage of children, this form of JRA may affect the eye, causing damage to it and even lead to blindness on very rare occasions. Older children with pauciarticular JRA may develop "extended" arthritis, involving multiple joints, lasting into adulthood.

Polyarticular arthritis can occur at any age and it affects more girls than boys, particularly, older girls. It affects five or more joints within six months of the disease first appearing. When it affects the older girls, it resembles the adult form of rheumatoid arthritis. The small joints of the hands are affected as well as the weight-bearing joints such as the knees, hips, ankles, feet, and neck. Pain and inflammation may be accompanied by low grade fever and the appearance of bumps or nodules on the body on areas subjected to pressure from sitting or leaning. Those children with a positive blood test for rheumatoid factor, are more likely to develop joint damage and may require more aggressive treatment.

Systemic JRA is the worst form of the disease since it tends to attack other areas and organs of the body. Symptoms include unexplained high fevers, unexplained skin rash, elevated white blood count, unexplained anaemia, enlarged spleen and lymph nodes and pain, swelling and stiffness of several joints in the body. Arthritis may occur after several weeks or months of fever.

Fortunately, only 10% of all JRA will take the systemic form; 40% cases are affected by polyarticular and approximately 50% of children will suffer from pauciarticular JRA. A very small minority of children may be affected by other kinds of JRA.

How is JRA diagnosed?
JRA may be difficult to diagnose because some children may not complain of pain at the start and joint swelling may not be obvious. Observations of limping, stiffness when awakening, reluctance to use a limb or reduced activity level may be clues, indicative of the problem. Persistent fever, joint swelling, night awakening or weight loss may be other signs. Tests which are commonly positive in adult arthritis (rheumatoid factor in the blood or changes on X Rays), are usually negative in childhood types.

Also, a number of other conditions can mimic JRA and further evaluation to exclude these conditions may be necessary before diagnosis is confirmed.

Would this involve testing the synovial fluid at any point of time in the disease?
Yes, that's an important question. We do not test the synovial fluid unless we suspect infection of the joints. However, when a child or adult gets the very first attack of the condition and upon clinical examination we don't find any clear explanation for the episode, we draw the synovial fluid to do a culture test. But once the patient gets another attack in a different joint, we know that it is not infected and it is most likely autoimmune arthritis.

How is JRA treated?
I would like to emphasize that sometimes, children having JRA are erroneously diagnosed as having rheumatic fever. In the advanced countries, including the Gulf region, rheumatic fever, though it does occur, it is rare and a disease of the past unlike in the developing world where sanitation and housing are of low standards. So it is imperative that children manifesting symptoms of JRA which mimics symptoms of rheumatic fever, should be seen by a specialist rheumatologist so that appropriate treatment can be given. Treatment is aimed at modifying the action of the immune system, to relieve pain and inflammation, slow down or prevent the damage to joints, and restore use and function of affected joints in order to promote optimal growth, physical activity, and social and emotional development of the children, allowing them to lead normal life to the extent possible.

JRA is treated with a combination of medication, physiotherapy and exercise. When the condition is mild, it is enough to treat with NSAIDs (Non-steroidal anti inflammatory drugs) which reduce the inflammation and pain but don't change the activity of the disease. Depending on the severity of the condition, more aggressive treatment form need to be adopted. In specific situations, we may use cortisone orally or as injections in inflammed joints and that could be very useful in treating the condition. However we always look at cortisone as a temporary measure till other more specific medicines show their effects. Finally and most importantly are the specific disease modifying medicines including the old like methotrexate, sulfasalazine, and hydroxychloroquine and the newer drugs, the anti TNF agents, which are biologic agents. Though very expensive, these new drugs are very effective in completely controlling the disease and with less side effects.

What is the role of Physiotherapy in the disease?
Physiotherapy is particularly very important in kids to keep their limbs mobile. Muscle strength needs to be built up and limbs kept mobile by regular exercise and physiotherapy. In the unfortunate cases of advanced deformities, some splints may be supplied for the fingers or hands to help movement.

Can children on treatment for JRA participate in normal sporting activities?
By and large, these children can participate in normal school and routine activities but in some instances, we restrict certain activities like high impact sports which may aggravate the disease.

Does diet have a role to play in preventing or treating the disease?
No. We have no data to support any role for diet. However, it is important that children affected with the condition should be careful not to become over-weight or obese, since this will result in aggravation of the problem, involving joints of the lower limbs.

What is the role of surgery in JRA?
If the disease is not treated properly, it may destroy the joints. It is only in such situations that surgery is required. Treatment with medication, physiotherapy, if properly done, should produce very good results in most patients.



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