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SYSTEMIC LUPUS ERYTHEMATOSUS

ETIOLOGY:
Systemic lupus erythematosus (SLE) is a multisystem disorder of unknown
etiology characterized by a production of large amounts of circulating
autoantibodies. This antibody production may be due to loss of Tlymphocyte control on B-lymphocyte activity, leading to hyperactivity of B
lymphocytes, which leads to nonspecific and specific antibody and
autoantibody production. These antibodies form immune complexes that
become trapped in the microvasculature, leading to inflammation and
ischemia.

EPIDEMIOLOGY:
Although SLE affects primarily women of childbearing age, approximately 5%
of cases present in childhood, mainly around puberty. SLE is rare in children
younger than 9 years of age. Although there is a female predominance of
this disease in adolescence and adulthood, there is an equal gender
distribution in children. The overall prevalence of SLE in the pediatric
population is 10 to 25 cases per 100,000 children.

Patients with SLE can present either in an abrupt fashion with fulminant
disease or in an indolent manner. Nonspecific symptoms are common but
can be quite profound and may include significant fatigue and malaise, Lowgrade fever and weight loss. Skin disease can be a prominent finding,
occurring in up to 95% of patients. A raised, erythematous rash on the
cheeks, called a malar butterfly rash, is common .This rash also can occur
across the bridge of the nose, on the forehead, and on the chin.
Photosensitivity can be problematic, particularly during the summer months.
Both of these rashes improve with appropriate therapy. The rash of discoid
lupus, by contrast,
is an inflammatory process that leads to disruption of the dermal-epidermal
junction, resulting in permanent scarring and loss of pigmentation in the
affected area. If discoid lupus occurs in the scalp, permanent alopecia ensues
because of loss of hair follicles. Raynaud phenomenon, although not specific
for SLE, and livedo reticularis also can occur.
Mouth and nasal sores resulting from mucosal ulceration are a common
complaint in patients with SLE and can lead to ulceration and perforation of
the nasal septum. Because of reticuloendothelial system stimulation,
lymphadenopathy and splenomegaly are common findings in SLE. In

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CLINICAL MANIFESTATIONS:

predisposes to SLE. usually at high titers. SLE can affect the central nervous system (CNS). primarily manifest as lymphopenia. a false-positive Venereal Disease Research Laboratory (VDRL) test. with low levels of C3 and C4 and decreased complement function as measured by CH50. a negative antinuclear antibody has a high negative predictive value for SLE. Page5 LABORATORY AND IMAGING STUDIES: . itself. Thrombocytopenia and anemia of chronic disease may be found. which also can be seen in other rheumatologic diseases and in primary antiphospholipid syndrome. determine prognosis. Excessive antibody production can lead to polyclonal hypergammopathy with an elevated globulin fraction in the serum. occurring in 50% to 70% of children. is common. with muscle weakness and muscle fatigability. nephrotic syndrome.any joint may be involved. and monitor response to therapy. These antibodies lead to an increased risk of arterial and venous thrombosis and can be detected by the presence of anticardiolipin antibodies. Renal involvement is one of the most serious manifestations of SLE and is common in pediatric SLE. Hematologic abnormalities also are prevalent in patients with SLE. which. with chest pain and pleural or pericardial friction rubs or frank effusion. Excessive circulating antibodies and immune complexes also lead to the consumption of complement proteins. DIAGNOSIS: Testing for SLE is performed to establish the diagnosis. Likewise patients with SLE can have antibodies directed against phospholipids. Effective therapy returns the low complement levels to normal.particular. Myalgias or frank myositis. Antibodies to Ro (SSA) and La (SSB) also can be found in patients with SLE. axillary lymphadenopathy can be a sensitive indicator of disease activity. Antibodies directed against Sm (Smith) are specific to SLE but are found in only approximately 30% of persons with SLE. but they also occur in patients with Sjögren syndrome. or a prolonged activated partial thromboplastin time. Hypertension or the presence ofedema suggests lupus renal disease. This is one way to monitor therapy except in patients with familial deficiency in complement components. and renal failure. Serositis can be seen. to seizures. Rarely patients with SLE develop Coombs-positive autoimmune hemolytic anemia. Arthralgias and arthritis are common. The presence of antibodies to doublestranded DNA should raise suspicion for SLE because these antibodies are present in most patients with SLE and are found almost exclusively in the disease. Because of its high sensitivity. may occur. The arthritis is rarely deforming and typically involves the small joints of the hands. Leukopenia. and stroke. leading to a myriad of symptoms ranging from poor school performance and difficulty concentrating. Renal disease may range from microscopic proteinuria or hematuria to gross hematuria. Titers of anti-double-stranded DNA antibodies are quantifiable and vary with disease activity. Although nonspecific. limiting its clinical utility. psychosis. a positive antinuclear antibody is found in more than 97% of patients with SLE.

Urinalysis may show hematuria and proteinuria. identifying patients with lupus nephritis. Serum blood urea nitrogen and creatinine evaluate renal function. CNS lupus has a specific pattern on gadolinium-enhanced magnetic resonance imaging. Elevation of muscle enzymes may be a clue for the presence of myositis. Elevated cerebrospinal fluid (CSF) protein and an elevated IgG-to-albumin ratio when comparing CSF with serum (IgG index) can indicate antibody production in the CSF and help diagnose SLE affecting the CNS. myalgia Arthralgia CUTANEOUS Raynaud phenomenon Alopecia Urticaria-angioedema Panniculitis Livedo reticularis NEUROPSYCHIATRIC Personality disorders Stroke Peripheral neuropathy Chorea Transverse myelitis Migraine headaches Depression CARDIOPULMONARY Endocarditis Myocarditis Pneumonitis OCULAR Episcleritis Sicca syndrome Retinal cytoid bodies GASTROINTESTINAL Pancreatitis Mesenteric arteritis Serositis Hepatomegaly Hepatitis (chronic lupoid) Splenomegaly RENAL Nephritis Nephrosis Uremia Page5 Additional Manifestations of Systemic Lupus Erythematosus . Hypoalbuminemia and hypoproteinemia may be present. SYSTEMIC Fever Malaise Weight loss Fatigue MUSCULOSKELETAL Myositis.

antimalarials (hydroxychloroquine). TREATMENT: • • • MILD DISEASE: Rashes. Anticoagulation where appropriate • • • • Life-long burden of renal failure and (multiple) renal transplant(s) Steroid toxicity Immunosuppressive toxicity Infection risk different in children: – CMV. arthritis. pneumonitis. MMF SEVERE DISEASE: Severe. thrombocytopenia. page). it can be difficult to diagnosis early in the disease course.Hypertension REPRODUCTIVE Repeat spontaneous abortions Neonatal lupus erythematosus Congenital heart block DIFFERENTIAL DIAGNOSIS: Because SLE is a multisystem disease. leukopenia. low dose methotrexate MODERATE DISEASE: Mild disease + mild organ system involvement such as: mild pericarditis. Azathioprine. fever. fatigue – Treatment: NSAIDs. Criteria have been developed for the diagnosis of SLE (see Table prev. low dose corticosteroids (<60 mg/day). life-threatening organ system involvement – Treatment: High dose corticosteroids (2-3 mg/kg/day or pulse). Immunosuppressives (IV pulse Cyclophosphamide). mild CNS disease – Treatment: Prednisone 1-2 mg/kg/day. Low dose methotrexate. Plasmapheresis. anemia. arthralgias. Suspicion must be high in patients who present with diffuse symptoms. Many of the clinical manifestations of SLE are found in other inflammatory illnesses and during acute or chronic infection. EBV – Bacterial infections. particularly adolescent girls. Antimalarials. The presence of 4 of 11 of these criteria has 98% sensitivity and 97% specificity for SLE. esp. NSAIDS. hemolytic anemia. mild renal disease. strep – Fungal infections Page5 SPECIAL CONSIDERATIONS IN CHILDREN AND ADOLESCENTS: .

com/contents/systemic-lupus-erythematosus-slein-children-treatment-complications-and-prognosis? source=search_result&search=lupus+in+childhood&selectedTitle=1~1 50 3.uptodate. Copyright © 2015.com/article/332244-overview 1. Worse prognoses are seen in patients with severe lupus nephritis or cerebritis. Hudeify Ahmed Ali 3. References. Adult patients with SLE develop accelerated atherosclerosis. with risk of chronic disability or progression to renal failure. Muhammad Omar Warsame 2. most patients live well into adulthood. not only because of prolonged corticosteroid use but also due to the underlying disease.medscape. infections. however. 2.uptodate.• Developmental age and psychosocial issues COMPLICATIONS: Long-term complications include avascular necrosis secondary to corticosteroid use. 1. PROGNOSIS: Outcomes for SLE have improved significantly over the past several decades and depend largely on the organ systems that are involved.com/contents/systemic-lupus-erythematosus-slein-children-clinical-manifestations-and-diagnosis? source=search_result&search=lupus+in+childhood&selectedTitle=2~1 50 4. Awale Ahmed Ibrahim Page5 GROUP 4: . http://www. http://emedicine. SEVENTH EDITION. With current therapy for the disease and the success of renal transplantation. All patients with SLE should be counseled regarding their weight and maintaining an active lifestyle to reduce other cardiac risk factors. http://www. and myocardial Infarction. NELSON ESSENTIALS OF PEDIATRICS.

4. . Mustafe Jama Barkhadle 5. Ahmed Moalimow Page5 End.