Barbara E. Ostrov, M.D.Assistant Professor of Pediatrics and Medicine, Pediatric and
Adult Rheumatology Milton S. Hershey Medical Center, Penn State University School of Medicine, Hershey, Pennsylvania
A selection from the Lupus Foundation of America Newsletter Article Library
(originally appeared in Lupus News, Volume 13, Number 3)
Distributed in Canada by Lupus Canada
About 20% of SLE patients are diagnosed before 20 years of age. In the majority of these patients, the illness begins around the time of puberty or 12 to 14 years of age.
The signs and symptoms of SLE in childhood and adolescence are virtually identical to the manifestations described in adult lupus. As with adults, the vast majority of children affected are female -- girls are diagnosed four times as frequently as boys if the disease begins before 10 years and nine times more frequently when the illness starts after puberty. Also like adults, the disease course is very variable, with flare-ups and periods of relative disease inactivity. In young patients, fever, swollen glands (lymphadenopathy), rash, liver and spleen enlargement and weight loss are particularly common findings. Arthritis, anemia, low white blood cell counts (leukopenia) and low platelet counts (thrombocytopenia) are also frequently found in this population. More serious organ involvement, such as of the brain (cerebritis) or heart myocarditis), are usually seen in less than 25% of young patients. However, kidney disease (glomerulonephritis) occurs in 50-75% of children with SLE. The prevalence of glomerulonephritis seems to be slightly higher than that seen in adults.
Some research has suggested that SLE may be a more serious disease in childhood or adolescence. However, detailed analysis of many studies shows that lupus only seems more serious at first glance because young people have their disease for 20 or 30 years longer than their adult counterparts. The outcome and prognosis appear to be very similar no matter what the age of the patient.
The majority of patients do not have major complications due to lupus, although severe fatigue and arthritis can interfere with daily routines at times, particularly when the child or adolescent is attending school. A smaller proportion of patients have difficulties with chronic kidney problems and some may need dialysis or ultimately kidney transplantation. Infections are the most common complaint of SLE, most often due to treatment needed to control the disease (such as steroids - prednisone, medrol, or immunosuppressive drugs - cyclophosphamide or azathioprine). In all populations, the response to and side effects from treatment play a major role in determining the long-term outcome of this disease. Extended use of steroids may prevent normal growth if used before puberty is completed and may damage bones at any age, causing osteoporosis and an increased risk of fractures. It also appears that prolonged use of steroids
In high doses may cause premature hardening of the arteries, particularly if started in adolescence. This complication may lead to difficulties
when young lupus patients age into their thirties or forties. As mentioned above, infections are a common complication of the treatment of lupus, especially if steroids or other immunosuppressive drugs are needed for treatment. This issue becomes particularly important in childhood when the frequency of infections is much greater than in adulthood. Also, some of these treatments influence the use of immunizations, as some vaccines contain live viruses (measles and polio vaccines, for instance) and should not be given immunosuppressed to individuals. Lastly, at all ages, but particularly in young people, the attitude of patients and families and their ability to cope with lupus are extremely important in determining outcome.
Usually, adolescence is a time of dramatic changes for a young person. It is the period of transition from childhood to adulthood. Physical changes appear; mature thinking abilities develop; and social and psychologic independence from one's family occurs. In the child with a chronic disease such as lupus, many or all of these normal developmental changes may be delayed or interrupted. For example, lupus itself or treatments required to control disease activity may interfere with the physical process of growth and puberty (lack of a growth spurt or delayed onset of menstruation). Normally an adolescent would participate in decision making at this age. However, when serious medical conditions are present, often parents and physicians make the decisions, keeping the patient dependent and unable to develop self-reliance. In addition, young people normally have concerns about sexuality, education and career development. These issues are often neglected because concerns about medical conditions predominate.
These subjects may be even more important to adolescents with SLE who wonder how this disease or its treatment will affect all aspects of their future.
With all these changes, it is easy to see how a young person with lupus may have trouble adjusting to their disease and its effects. These adolescents frequently have a negative body image (due to, for example, facial rash or hair loss from the disease or swelling from steroid use) and often exhibit poor compliance with treatment or diet because of this. Excessive absenteeism from school is also common in this situation and may be a good reflection not only of the disease activity but of coping as well. These patients are often angry/or depressed and may have inappropriate behaviors, such as drug abuse or intentional medication misuse. They want very much to maintain some degree of independence and control, and may alienate their friends and family by their actions - just when they need these support people the most. These behaviors may cause social isolation and produce even more psychologic dysfunction.
Clearly, it is important for physicians caring for young lupus patients to keep all these issues in mind. Knowledge of the disease and its medical treatment alone are not adequate when caring for a child with lupus. It is also necessary for the doctors to be able to listen to the child's and family's concerns and to be aware of the special needs of the adolescent/ pediatric patient. The long-term physical, psychological and emotional impact of all aspects of treatment and of the physician's approach to treatment must be considered every step of the way. Only by allowing the child to participate in his/her own care and decision-making (whenever feasible) and by seeing that pubertal issues are addressed whenever possible, can we minimize the complications of this disease, both medical and psychological.