COUNSELLING THE PARENTS OF A YOUNG CHILD WHO HAS ARTHRITIS AND HLA B27 POSITIVITY
SLiza (Suri) Rajasekhar, MD *
Department of Pediatrics.*
Counseling in the medical setting is a dynamic process where once a diagnosis is confirmed, one or more aspects of the disease is discussed with the patient or in case of children their care providers. It is dynamic because not only does it happen over a period of time, but the extent of information shared depends upon the level of understanding of the child and his parents. During counseling it is preferable to create an environment where the parents feel comfortable asking question and understanding the answers. Information is then shared in a gradual staggered way, not necessarily in one sitting. Certain issues are common to counseling any child with arthritis. As parents carry the responsibility for health care of the child they must:
  • Make sure that the child sees all the health care team
  • Takes all the prescribed medicines, watch for any side effects the drug may and cause help the child to do exercises to relieve pain and stiffness
  • Provide encouragement to child and other family members
  • Work with the school to make serve the child needs of education and transport are met

Hence, it is important that parents know as much as possible about their child's arthritis as well as the effects it can have on family life

In a child with arthritis and HLA B27 positivity, the following specific issues arise Prognosis..

Arthritis associated with HLA B27 is an 'inflammatory arthritis'. This is a chronic disease and may last several years. There are chances that the child may get better and have no serious permanent handicaps. More often that not the child can keep up with school and other social activities except in times of flare of the arthritis or if a joint has been damaged. Sometimes the arthritis subsides. However when this will happen and how long it will last is unpredictable.

It is important to be aware of possible long-term effects on the joints eyes and heart and work with the health team to look out for these and keep the disease under control.

Symptoms

Usually a child with arthritis and HLA B27 positivity is an older male child around 8 years of age with pauciarticular disease. This usually means that less than 4 joints are involved.

Any joint is lined by a film called synovium. In arthritis this film is swollen up, overgrown and produces too much fluid so that there is pain, swelling, stiffness, warmth and redness of around the joint. The child holds the joint in a bent position due to pain. If this occurs for a long time the muscles around the joint become stiff and weak and the tendon may tighten up and shorten causing deformity called contracture.

To prevent this the child needs an exercise program to keep the full motion of the joints.

Causes

HLA B27 is a protein which can be found on the white blood cells. It is present in 8-10% of the normal population. It is also present in your child and may be a part of the reason your child has developed arthritis. Though 90% of children with juvenile ankylosing spondylitis and 50% of children with juvenile psoriasis will have this HLA B27, it is clear that factors other HLA B27 are involved since only a few HLA B27 individuals develop arthritis.

This protein factor comes from a gene which can be passed down to one's children there is a 50-50 chance of passing it down.

Since this is a genetic factor it is possible that other children or relatives in the family also carry HLA B27 gene but have no symptoms. The risk of developing a spondylo arthropathy is about 10-20% if there is someone in the family with arthritis.

How will the disease evolve
Usually a child with arthritis and HLA B27 may develop into any of the following forms of arthritis:
  1. Undifferentiated spondyloarthropathy
  2. Juvenile ankylosing spondylitis (JAS)
  3. Juvenile psoriatic arthritis
  4. Inflammatory bowel disease (IBD) related arthritis

In the first type of arthritis there are usually four or less joints, usually large joints like the knee, ankle of the lower limbs involved. Children with JAS in addition have inflammatory arthritis and HLA B27 positivity and may develop IBD, through the reverse is more likely.

Should other relatives or siblings be tested?

HLA B27 is not a diagnostic test. Only a proportion of person with HLA B27 positivity develop arthritis. So in an asymptomatic individual there is no role of doing HLA B27 to predict development of arthritis. In a study only 9% of HLA B27 positive relatives of ankylosing spondylitis patients had chronic inflammatory back pain without radiological changes.

However in the following situation it may be of some use. In a relative of a HLA B27 ankylosing spondylitis proband a negative B27 test indicates that any back symptoms are very unlikely to be related to inflammatory arthritis, just as in a positive test in this context, ever with normal radiographs, may suggest a forme fruste of this disease.

HLA B27 has strong links to infection. Individuals with HLA B27 are 100 times more likely to develop arthritis after genitourinary or gastrointestinal infection.

Does presence of HLA B27 predict severity of disease?

80% of children with pauciarticular onset chronic arthritis irrespective of the presence or absence of HLA B27 suffer little or no musculoskeletal disability at 15 years follow-up.

However the presence of HLA B27 is one of the factors which predict sacroiliitis in children with arthritis, the other factors being absence of DP B1*02, hip joint involvement within first six months and disease onset after 8 years of age.

In one study of 130 children with arthritis, 41 had undifferentiated spondyloarthropathy 88% of these were HLA B27 positive 41% of this group had sacroiliitis. 28 patients had one of the three differentiated spondyloarthropathies: reactive arthritis, ankylosing spondylitis and psoriatic arthritis; 97% of this group had HLA B27 positive.

Does the presence of HLA B27 predict extraarticular involvement?

The two most common sites outside the joint affected with inflammation are the eyes and the heart.

Individuals with HLA B27 have more chance of developing inflammation of the eye it occurs in between 10-20% of children. Onset is acute usually unilateral affecting the anterior uveal tract visual prognosis is good but recurrences are common eye examinations are recommended yearly in older children (> 7 years) and every 3 months in younger children.

Inflammation of heart in the setting of arthritis is less common in children compared to adults. However there are scattered reports of clinically significant valvular lesions in older children with HLA B27 related arthritis. Higher frequency of nonspecific inflammation of the gut is seen in patients with HLA B27 related arthropathies.

How does presence of this gene translate into disease?

HLA B27 molecule has a particular fold in it which binds certain peptide fragments. Part of this is similar to the protein structure of a bacteria called Klebsiella. It is possible that in patients who have this gene after a bacterial infection the cartilage or some other part of the own body tissues are recognized as foreign and a reaction mounted against the tissues.

Do I need prophylaxis against infection?

There is no data to suggest that prophylaxis against gut infections will prevent development of the disease or diminish its severity.
Summary
In a child with HLA B27 and arthritis development of sacroiliitis, uveitis and inflammatory bowel disease has to be watched for. Overall prognosis is good. Close relatives are at a higher risk than the general population for developing the disease.
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