Systemic Lupus Erythematosus in Children and Adolescents
Section snippets
Epidemiology
Childhood-onset SLE (cSLE) is a rare disease with an incidence of 0.3 to 0.9 per 100,000 children-years and a prevalence of 3.3 to 8.8 per 100,000 children.1 A higher frequency of cSLE is reported in Asians, African Americans, Hispanics, and Native Americans.2, 3 When compared with 2 more common childhood autoimmune diseases, juvenile idiopathic arthritis (JIA) and type 1 diabetes, cSLE is approximately 10 to 15 times less common in White children.4, 5 However, in Asian children, cSLE is
Classification and diagnosis of cSLE
SLE is called the great mimicker, as the disease shares characteristics with many other (autoimmune) diseases. Especially when the classic malar rash is absent, diagnosing SLE can be a challenge. However, the astute pediatrician who considers SLE when presented with an unusual constellation of symptoms can recognize important patterns of disease manifestations crucial for the diagnosis. Most patients who are diagnosed with cSLE fulfill 4 or more of the American College of Rheumatology
Clinical features
This review does not attempt to describe all possible clinical manifestations but instead focuses on specific features that may be crucial for immediate recognition. Table 2 summarizes the frequencies of the common manifestations of cSLE.7, 12, 13, 14, 15, 16, 17 SLE can affect any organ system, and leads to glomerulonephritis and central nervous system (CNS) involvement arguably more often in cSLE than in adults with SLE.
Is it SLE?
The differential diagnosis of SLE is broad, and includes infection, malignancy, and other inflammatory disorders. The adolescent female patient who presents with a photosensitive malar rash, painless oral ulcer, polyarthritis, Raynaud phenomenon, and pleural effusions is not a diagnostic challenge. However, initial symptoms may be vague, and red flags for the pediatrician include an older child or adolescent with any combination of persistent fever, fatigue, anemia, leukopenia,
Complications
In addition to severe disease flares and complications secondary to medications, 2 further categories warrant mention: infection and MAS.
Treatment
The care of a child or adolescent with SLE requires a multidisciplinary approach, and ideally involves rheumatology, a primary care physician, nephrology (for any patient with renal disease), adolescent medicine, psychiatry and psychology, nursing, social work, and physical and occupational therapy. Pharmacologic treatment is often aggressive, but tailored to the severity and extent of disease manifestations. Although few drugs are actually approved by the Food and Drug Administration (FDA) for
Adolescent issues to consider for optimal disease management
SLE is a lifelong disease, characterized by periods of flare and remission. Because the typical cSLE patient is an adolescent female, addressing the usual challenges of adolescence becomes crucial to ensuring that the adolescents are making informed and correct choices. As the onset of SLE occurs at an already challenging time in life, further stresses and comorbidities of disease should be minimized. At this age, teens are trying to exert their independence, are learning self-sufficiency
Long-term outlook
Studies have shown that patients with SLE do not achieve long-lasting drug-free remission, and many patients have persistently active disease requiring long-term immunosuppressive treatment.61, 62 However, cSLE patients are younger at diagnosis and have a more severe disease course than those with adult-onset SLE, therefore they are prone to develop significant damage caused by the disease or its treatment at a relatively young age.7, 63 Nonwhite ethnicity, especially African American (or
Summary
Childhood-onset SLE is a lifelong autoimmune disease that may be difficult to diagnose because of its multisystem involvement and heterogeneity of clinical manifestations. It follows a more aggressive disease course than adult-onset SLE, with greater disease activity at presentation and over time, and consequently leads to greater morbidity and mortality than adult-onset SLE. Moreover, children with SLE have to deal with this unpredictable, relapsing-remitting disease during adolescence, an
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