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

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

Disclosures

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

In This Article

Abstract and Introduction

Abstract

Systemic lupus erythematosus (SLE) is a multifactorial autoimmune rheumatic disease. Although its highest prevalence is among women of childbearing age, the disease is not confined within this population. A total of 15–20% of cases of SLE are diagnosed in children younger than 16 years (childhood-onset lupus). Although there have been few studies directly comparing childhood- to adult-onset lupus, there is substantial evidence to suggest that pediatric lupus patients display some differences in their disease profile compared with adult-onset populations. Overall, an increased male-to-female ratio, a higher prevalence of nephritis and CNS involvement necessitating a more sustained need for steroids and immnosuppressive drugs, and a higher prevalence of progression to end-stage renal disease are distinguishing features of childhood-onset lupus. In contrast, a higher prevalence of pulmonary involvement, arthritis and discoid lupus are reported in adult-onset SLE patients. Furthermore, childhood-onset lupus patients may experience a serious negative impact on their psychosocial and physical development, issues that pose extra challenges to healthcare providers. Growth delay, osteoporosis, the psychological effect of steroid-induced alterations of the physical image, and often poor treatment compliance are the issues that need to be addressed in pediatric lupus populations. In this review, we compare the epidemiological, clinical and laboratory features, and treatment options of childhood- and adult-onset lupus, and comment on the applicability of the instruments that measure activity, severity and cumulative disease damage in childhood-onset disease. In addition, we highlight special issues of concern for pediatric lupus patients, discussing the significance in the transition from pediatric to adult rheumatology care.

Introduction

Systemic lupus erythematosus (SLE) is an autoimmune rheumatic disease that results from the interaction of multiple environmental, immunological and genetic factors, causing inflammation and eventually damage in a wide range of organs and systems. Its prevalence ranges from approximately 40 cases per 100,000 individuals in Caucasians to more than 200 cases per 100,000 individuals among black people. In common with other autoimmune diseases, it is more prevalent among women, in adults by a factor of 9.[1,2] Although mainly a disease of women of childbearing age, its prevalence is not confined within this population. A total of 15–20% of cases present in children under 16 years of age. Although there have been limited studies directly comparing adult- and childhood-onset SLE (cSLE) (Table 1), it has been suggested that pediatric lupus patients have a more aggressive disease course and an increased rate of more unusual initial clinical presentations compared with their adult counterparts.[3–10] Differences in the serological and autoantibody profiles of children and adults with SLE have also been described. Lupus may be life-threatening when major organs are affected; however, in the majority of cases, it results in chronic debilitating ill health. Thus, the impact of the disease on growth and development, and its effects on the psychosocial adjustment of children, are important issues that need to be addressed by healthcare providers.

In this review, we compare the clinical and serological profiles of pediatric and adult-onset SLE patients, also commenting on possible discrepancies in the underlying immunological abnormalities. We also focus attention on the validity of established classification, disease assessment, and outcome criteria in pediatric lupus patients. Finally, we discuss different therapeutic options that apply to the two populations, highlighting any special considerations that are specifically applicable to children and adolescents with lupus.

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