Childhood- and adult-onset lupus: an update of similarities and differences

Eva D Papadimitraki, MD, PhD; David A Isenberg, MD, FRCP, FACS


Expert Rev Clin Immunol. 2009;5(4):391-403. 

In This Article

Specific Issues for Childhood-onset SLE

Childhood- and adolescent-onset SLE remains a serious and devastating disease, as indicated by the high SLE-related damage, which reaches 40% as measured using the SDI and Child Health Related Quality of Life (CHQ) indices. Physicians involved in the care of these age groups have to meet with multiple challenges, ranging from the severity of the illness per se, to the cumulative treatment toxicity, and the psychosocial consequences of a major chronic disease on emotionally immature individuals who are just beginning to formulate their concept of self. Recognition of the special considerations that relate to ongoing physical and emotional growth directly influences the choice of treatment and the likelihood of success.

Some adolescents experience long periods of illness before they receive appropriate treatment. Inappropriate referrals to psychiatrists (due to mood swings, weight loss, weakness and constitutional symptoms that are the most common manifestations of the disease in this age group), unnecessary diagnostic procedures, prolonged difficulties in learning activities and a significantly increased risk of organ involvement and damage all represent deleterious consequences of long diagnostic delays.[95,96]

The consequences of both short-term (e.g., alterations in appearance) and long-term corticosteroid treatment, which is often needed in childhood-onset SLE, may have negative impacts on adolescent physical and psychosocial development. Long-term physical changes may include include obesity, stunted growth, delayed puberty, striae and hirsuitism. These problems have been shown to negatively affect adherence to treatment in up to 50% of patients.[97]

Delayed puberty has been documented in 11% of patients with childhood-onset SLE and may be attributed either to the effects of treatment or to hypothalamic dysregulation due to chronic illness.[72] Osteoporosis in cSLE stems from chronic inflammation, pubertal delay, sustained steroid administration and voluntary decreased sun exposure. The consequences on bone mass index (BMI) are considerable in this age group, since in healthy children the BMI increases by 14% during puberty, reaching its peak at 18 years of age. Importantly, a study of 70 juvenile SLE patients with a disease duration of 10.8 ± 8.3 years, revealed a significant decrease of bone mass density, which correlated with male sex and corticosteroid use.[98] A more recent study by Silverman and colleagues reported a 37.5% incidence of osteopenia and a 20% incidence of osteoprorosis, which is associated with immunosuppressive treatment, corticosteroid use and nephritis in 64 patients with juvenile lupus.[99] The use of biphosphonates for optimal bone loss prevention in children remains controversial owing to concerns for their sustained fixation on the growing bone and their presumed extra-skeletal deposition (e.g., in the coronary arteries), which may aggravate atherosclerosis, a major concern in SLE patients.

Both the disease per se and the use of immunosuppressive drugs have been related to premature gonadal failure. A 3-year cohort study showed that while premature ovarian failure was rare, 30% of the young females, especially those having received a cumulative dose of 21 g of CYC, had a severe decrease of their ovarian reserve.[100] The risk for premature gonadal failure and the fear for sterility may lead young patients to delay treatment with CYC; however, CYC seems to have less gonadal toxicity when administered to younger patients. The Lupus in Minority populations: Nature via Nurture (LUMINA) study identified disease activity and Texan–Hispanic ethnicity as predictors of a shorter time to premature gonadal failure and confirmed the association between CYC-related gonadal toxicity and older age..[101] Male adolescents with SLE have an even higher risk of infertility than females; however, storing of male gametes prior to chemotherapy is a widely accepted and used method to preserve fertility in this population.[102]

The health-related quality of life (HRQL) score represents a major concern in patients with juvenile SLE since it is directly related to organ damage. A study investigating the CHQ in 297 juvenile SLE patients has shown that they had poorer HRQL as compared with healthy controls in both physical and psychosocial domains, with renal and musculoskeletal damage being the parameters that most prominently influenced their physical health.[72] Table 4 summarizes the incidence of the most important lupus complications affecting physical and psychosocial health in juvenile patients.

The overall prognosis of childhood-onset SLE has markedly improved over the last decades. Ten-year survival of children with SLE now exceeds 90% and is comparable to adult SLE patients. The main disease-related causes of death are acute cardiovascular and hematological events, mostly occurring in patients with chronic renal failure. Infections are also important causes of both morbidity and mortality in pediatric as well as adult lupus patients. However, secondary neoplastic diseases such as non-Hodgkin's lymphoma and lung cancer, which are common causes of death in adults, are not commonly reported in pediatric lupus populations.[96]

The transition from pediatric, where priorities are focused on growth, development and family problems, to adult-orientated healthcare, where independence, professional tasks and reproduction issues are the major concerns, requires a suitable period of preparation, which may include shared/altered consultations by adult/pediatric rheumatologists in the pediatric and adult clinics and a carefully coordinated multidisciplinary approach that integrates a complex medical story and the emotional expectations of the adolescent and their family.


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