AN APPROACH TO A CHILD WITH JUVENILE ARTHRITIS



Introduction
Musculo-skeletal pain is not uncommon in children. Though commonly this is due to a benign process called "growing pains", arthritis can afflict children as well. Arthritis (inflammation in the joints) has been described in children as young as 1 year. For the sake of classification, arthritis occurring in the age groups up-to 16 years is called "Juvenile Arthritis".

Almost all the types of arthritis that have been described in adults have also been described in children. In all there are easily more than 50 different types of arthritis that can develop in children. This communication discusses the presentation and principles of management of the common varieties.

What are the complaints?
In infants and toddlers it may be difficult to elicit symptoms. Inability to use a limb or part of the limb, excessive crying when the affected portion is touched, limp while walking may all be the initial symptoms. Often these are persistent over a period of few weeks. Along with this there could be low grade to high-grade fever, lack of appetite and loss of weight. Arthritis although primarily involves the joints can present with symptoms and signs of other system involvement.

What are the other systems that can be involved?
Almost any other system can be involved. However it is important to note that only a proportion and not all patients with juvenile arthritis get all the various manifestations mentioned. The organ systems that can be involved often depend on the type of arthritis. This will be dealt with when individual disorders are discussed.

What are the common forms of juvenile arthritis?

The most common form of juvenile arthritis is called juvenile idiopathic arthritis. This in-turn is further subdivided into 3 subgroups

  1. Polyarthritis (5 or more joints involved),
  2. Pauci-arthritis (< 5 joints involved) and
  3. Systemic onset disease (also called Still’s disease).

In these forms of arthritis, joint involvement, is the predominant mode of presentation. Low grade fever weight loss and loss of appetite can accompany the first 2 forms of arthritis. Still’s disease often presents with high grade swinging fever, short-lived reddish rash (often with the fever swing) and, in a proportion of patients, other organ involvement. Pleural effusion (fluid in the covering membranes of the lung), pericardial effusion (fluid in the covering membranes of the heart), ascites (fluid in the covering membranes of the intestine), enlargement of the liver/spleen etc are the organ systems that can be involved.

Systemic lupus erythematosus is a form of arthritis characterized by high-grade fever, facial rash, loss of hair, oral ulcers and joint pains. Diagnosing this entity is important, as other system involvement is common and troublesome. Anemia, low platelet count with bleeding, neurological involvement and kidney involvement can occur.

Scleroderma is characterized by tightening of skin (fingers & generalized), Blue fingers especially when exposed to cold (called Raynaud’s), heart burn with or without difficulty in swallowing (due to involvement of the food pipe-esophagus) and joint pains. Lung involvement (called interstitial lung disease), pulmonary hypertension and rapid increase in blood pressure (called accelerated hypertension) are important complications of this condition.

Polymyositis / dermatomyositis are conditions characterized by inflammation in the muscles. Patients present with painful weakness in the muscles. In addition patients with dermatomyositis have a rash on the face, trunk and typically over the knuckles. Lung involvement (interstitial lung disease) can occur in a proportion of patients.

What is rheumatic fever?
This is a form of arthritis, which, occurs after specific streptococcal infection of the throat. It is characterized by fever, flitting and fleeting arthritis (completely improves in one joint before involving the other joint), erythematous rash (in some), subcutaneous nodules (in some) and inflammation of the heart or its covering membrane (pericarditis, myocarditis and endocarditis). It is this cardiac involvement which if not treated adequately leads to valvular involvement later. The short-lived joint involvement, propensity to involve the heart and its association with specific streptococcal throat infection differentiates this disorder from juvenile idiopathic arthritis described earlier. Hence this is an important disease to be excluded when a child presents with joint problems.

What to do when arthritis is suspected?
As and when your general practitioner suspects arthritis he would more often than not send you to a specialist who has experience in treating these disorders.

What would the specialist do?
After confirming the clinical suspicion, the specialist would organize relevant blood tests to diagnose the type of arthritis and the presence and absence of complications. After having assessed the investigations, the specialist would start treatment with appropriate medications.

What are the common investigations done in children with arthritis?
These may be divided into a few subgroups:

To assess disease activity: CBC, ESR & CRP are common tests done to assess activity.
To assess damage: Radiographs of relevant joints are done for this.
To assess for drug side effects: Routine tests (blood and other) are done on a regular basis to assess for side effects due to drugs.
To assess complication of the disease: Since these arthritis’ can affect other organ system, if a particular organ system is suspected to be affected, relevant investigations to assess that particular system.  

What are the types of medications that are given in arthritis?
There are 3 or 4 types of medications that are given in juvenile arthritis.

Non-Steroidal Anti-Inflammatory Drugs (NSAIDS): These are normally given to reduce the pain, swelling and inflammation in children with arthritis. These act within a few hours. Although in very mild arthritis NSAIDS alone may be enough to control the disease, more often than not other medications (to be discussed) are required. It should be noted that there is no specific NSAID that is better than the others as regards efficacy. Often the NSAID, which, helps the child and at the same time does not cause side effects should be used. Gastrointestinal side effects are the commonest cause why these drugs are either changed or stopped.

Disease Modifying Anti-Rheumatic Drugs (DMARDS): These are slow acting drugs which can control the disease in the long run. These do not control the pain immediately. The commonly used DMARDS are chloroquine, methotrexate, salazopyrine etc. The specialist will explain the beneficial effects and the side effects of these medications and then start it. Regular monitoring of tests is a must on this drugs.

Apart from the above-mentioned 3 DMARDS there are others which are more often than not used when the above 3 fail or there are contraindications to them. These have not been discussed in detail here.

Steroids: Corticosteroids are much-maligned drugs. Although like any other drug they have side effects, when used in an appropriate manner, they are extremely beneficial, especially in patients with severe polyarticular arthritis and systemic onset arthritis.

Calcium & Vitamin D: These supplements are given routinely in children with arthritis to prevent weakness of bones and to help the child achieve optimal bone strength in the future.

Iron supplements: Children with arthritis often have concomitant iron deficiency and anemia. Iron supplements on routine basis take care of this deficiency.

What are the other therapies that children with arthritis would need?
Physiotherapy: Forms an integral part of treatment of children with arthritis. In the acute stages when there is excessive inflammation, the joint is rested. Once the inflammation is reduced, range movement exercises and, strengthening exercises are advised.

Orthotics and appliances: Children with deformities need assessment with a view to providing appliances and splints that would help lessen the disability.

Emotional needs: The amount of impact a chronic disease like arthritis has on a growing child is large. Children may have difficulties in schooling, interaction with other children, interaction with parents and relatives. All these should not be ignored and if needed the help of a child psychologist should be sought.

What role do climate and food have on arthritis?

There is no scientific proof to say that either hot /cold weather is the cause of arthritis. However sudden changes in temperature especially from hot to cold could worsen a pre-existing arthritis. Further since the level of activity goes down during cold weather the joint stiffness increases. When the disease is well controlled with appropriate medications one finds no relationship between the weather and the joint disease.

The kind of food eaten by and large does not affect the joints. Infact when the arthritis is active there is loss of weight and appetite. Hence there is no logic in doing any kind of dieting/food restriction. Less than 5% of children with arthritis have what is termed food allergy. This could be allergy to any kind of food including wheat-based food like chappati/ bread. These patients are told to avoid only that specific food.

What is the prognosis?
Prognosis depends upon the severity of arthritis, the delay in undertaking proper treatment and the efficacy & tolerability of medications.

The most important factor is early diagnosis and proper treatment. The first few years in this disorder is called a window period when, there is inflammation but no damage to the underlying joints. It during this crucial period that treatment is effective in retarding the joint damage.

What is the cause?
The exact cause of these disorders has not yet been clearly defined. However it is thought that in the presence of genetic predisposition an undefined environmental insult perpetuates a chronic inflammation causing the arthritis.

What is the role of other other systems of medicine like Ayurveda/ Homeopathy in these diseases?
Although an allopath is not qualified to comment on other forms of medications the following guidelines may be useful:

Adhere to one form of medicine at a time.
Give a fair trial of at-least 6 months on any system of medication before judging its efficacy or otherwise.

In chronic diseases like arthritis it is quite likely that there are subgroups of patients who find benefit with one specific system of medication.

Last updated on 24-12-2001

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