Health Related Quality of Life of Pediatric Patients with Systemic Lupus Erythematosus (SLE)

Abstract

Summary S ystemic Lupus Erythematosus (SLE) is an autoimmune rheumatic disease that is characterized by the production of autoantibodies which leads to immune complex deposition, inflammation and permanent organ damage. Hormonal, environmental and genetic factors play a contributory role in the development of SLE. Environmental factors including drugs, ultra violet light exposure, pregnancy and response to infectious stimuli such as viruses, also play a role. Pediatric SLE is more severe and with higher rates of organ involvement and more aggressive course than adult SLE.The most common presenting complaints of children with SLE include fever, fatigue, hematologic abnormalities, arthralgia, and arthritis. Renal disease in SLE is often asymptomatic, underscoring the need for careful monitoring of blood pressure and urine analysis; in adolescents, SLE often presents with nephrotic syndrome and/or renal failure with the predominant symptoms being edema, fatigue, changes in urine color, and nausea/vomiting. CNS involvement occurs in more than half of the children and adolescents who have SLE, usually early in the course of the disease, and typically includes both neurologic and psychiatric symptoms. Psychiatric symptoms may be a reflection of an autoimmune process or related to the underlying vasculitis, or the psychiatric disorder may be unrelated and coincidental. Psychosis, depression, anxiety, cognitive deficits, and emotional distress are seen frequently in patients who have SLE. Depression and other neuropsychiatric symptoms in patients who have SLE are associated with increased risk for suicidal behavior. The presence of delirium, psychosis, confusion, depression, or mania in a patient who has SLE suggests primary CNS involvement. The diagnosis of NPSLE should be suspected when neuropsychiatric symptoms occur in patients known to have SLE and should be investigated in children and adolescents who have acute onset of delirium or profound psychiatric symptoms, with or without neurologic symptoms. SLE is associated with both primary and secondary effects on psychosocial functioning. Because SLE symptoms and findings may develop serially over several years and not be present at one time, the diagnosis may require longitudinal follow up. SLE is often characterized by periods of flare and disease quiescence. A combination of clinical features and laboratory markers is used to diagnose SLE. Classification criteria developed by the American College of Rheumatology (ACR) are generally used in forming the diagnosis. Four out of the 11 stated criteria are highly suggestive of SLE. The multi-systemic nature of the condition means that pSLE may present to different specialists causing a delay in the initial diagnosis. Clinical features may take some time to evolve, and children may not initially meet all of the features to fulfil a formal diagnosis. A complete blood count and differential, ESR and urine analysis, studies performed on most unwell children, may provide results that strongly suggest lupus. Most newly diagnosed children will have a normochromic normocytic anaemia, a lymphopaenia and a low or low–normal platelet count in the face of a high ESR. Autoantibodies are the hallmark of SLE and a positive ANA is found in virtually all of the patients, but is not specific or necessarily associated with disease manifestations. Anti- dsDNA antibodies are the most commonly seen and are associated with disease activity, especially with active renal disease. Anti-Ro and La antibodies are seen less frequently, but are associated with skin disease and neonatal lupus. Anticardiolipin antibodies and the LAC make up the aPL and are associated with an increased risk of thrombosis. And one of the most helpful tests to determine disease activity is the measurement of C3 and C4 complements. Appropriate imaging studies need to be considered carefully, and may include: ECG, chest X-ray, echocardiography, bone marrow aspiration, lumbar puncture, magnetic resonance imaging (MRI), and renal biopsy.