From the National Magazine

Lupus in Childhood

by David S. Matheson, M.D. F.R.C.P. (C)
British Columbia Childrens Hospital, Vancouver. British Columbia, Canada.

Systemic lupus erythematosus (SLE) in childhood differs very little from the adult form of the disease. In adults, female to male ratio is approximately 8:1, whereas in children the ratio is 2:1. In childhood, the commonest age for onset of the disease is 11 to 15 years with cases exceedingly rare less than 5 years of age. Approximately 20% of all SLE begins during childhood.

Clinically, there is considerable similarity between adult and childhood forms of the disease. Affected children tend to have fatigue, fevers (80%) joint involvement (80%) and skin rashes (70%). Less commonly, the body systems are involved: kidneys (60%), lungs (20%), intestinal tract (20%) and central nervous system (15%). It is likely that involvement of lungs and brain is less common than in the adult form of the disease.

As in the case in the adult form of SLE, the cause is unknown. It appears that there is a genetic predisposition, that environmental factors such as drugs or viruses may initiate the disease, and that immune responses are involved in maintaining the disease. Patients develop antibodies directed against their own tissue (auto-antibodies) and have circulating immune complexes. The mechanisms which prevent excessive immune responses are deficient thereby allowing elevated levels of auto-antibodies. The inflammation that is induced in the skin, joints or other organs leads to the signs and symptoms suffered by the patient.

Therapy is directed at stopping that inflammation and ultimately decreasing the abnormal immune response. In milder forms, salicylates or nonsteroidal anti-inflammatory agents can control the symptoms. In more severe cases, particularly if there is kidney involvement, steroids and other immunosuppressive agents such as cyclophosphamide or azathioprine are necessary. Herein lies one of the major differences between the adult and childhood forms of the disease. Not only do children suffer from the same side effects of the steroids as do adults, they also suffer from impairment of growth and a much higher incidence of breakdown of the bones in the hip. Hence efforts should be directed at proceeding to alternate-day drug therapy as soon as is feasible so that the bodys own hormonal function is not so altered and growth can begin again. Morbidity associated with disease may in fact be higher for children than for adults but no good studies are available to assess this.

The prognosis in childhood lupus is related to the systemic involvement and the drug therapy. When death occurs, the cause is usually nephritis, central nervous system disease or infection. Some argue that if SLE presents to 12 years if age, then the outlook is more guarded. Estimates of mortality are usually around 10% for five years. Most authors state that with modern therapy, children do almost as well as adults in terms of mortality and slightly worse in terms of morbidity. No recent reports on the survival and complication rate is available for childhood lupus, however.

A description of lupus in childhood would not be complete without mentioning that newborn babies of mothers with lupus occasionally have symptomatic involvement. Something, presumably auto-antibody from the mother, crosses the placenta and may cause rashes, anemia, decreased white cells or platelets. Most babies are not symptomatic and those that are usually improve over several weeks or months. If the mother has one type of auto-antibody termed anti SSB the baby is at risk for developing heart block.

Hence, lupus in childhood is not very different from that in adults. Affected children have the same symptoms, the same laboratory findings, require the same treatment and are prone to the same recurrences. They, too, must learn to cope with the fatigue, the flareups and the side effects, all in the context of learning, growing and interacting with family and peers. Life styles change and acceptance is sometimes difficult. They know they are different at a stage in life when differences are not as acceptable. They want to be treated as any other child with the same rules and regulations, the same rights and responsibilities. Exceptions bring out denial and sometimes aggressive responses. Children actually cope very well, but the added strain of an uncertain disease with a terrible therapy causes considerable stress which requires extra coping skills from patient, family and the medical team involved.

We are obliged to Dr Matheson and the Lupus Foundation of America for allowing use of this article.

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