초록 |
Systemic lupus erythematosus (SLE) is a multisystem, autoimmune, connective tissue disorder with a wide range of clinical presentations. In fact, 20% of all cases of SLE are diagnosed during the first 2 decades of life. Although the presentation, clinical symptoms and immunological findings of pediatric SLE are similar to those of adult SLE patients, children usually have a more severe disease at onset. The natural history of SLE is unpredictable; patients may present with a history of many years of symptoms or with acute, life-threatening disease. Intermittent or sustained fever, fatigue, weight loss, and anorexia are manifestations of active disease in most children or may occur as the presenting symptoms. Isolated discoid lesions are even less common in children. Arthritis which occurs in up to 90% of pediatric patients generally presents as a symmetric polyarthritis involving both large and small joints. Because of its possible manifestations, SLE must be considered in the differential diagnosis of many problems, ranging from fevers of unknown origin to arthralgia, anemia, and nephritis. The initial presentation may be atypical such as parotitis, abdominal pain, transverse myelitis, or dizziness. SLE should be considered in patients with multiorgan symptoms, usually with an abnormal complete blood count or urinalysis. Early diagnosis and treatment tailored to the particular problems of an individual patient can greatly improve the prognosis for what used to be a fatal disease. |