WORKUP	Section 5 of 10
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Lab Studies:

• Initial laboratory evaluation should include CBC count with platelets and reticulocyte count; complete chemistry panel to evaluate electrolytes, liver, and kidney function; urine analysis; and a measure of acute phase reactants (eg, erythrocyte sedimentation rate [ESR] or C-reactive protein [CRP]).

• Diagnostic laboratory studies include antinuclear antibody (ANA), anti–double-stranded DNA, anti-Smith antibody, lupus anticoagulant, and antiphospholipid antibody panel.

• Obtain other autoantibodies, which may be associated with specific disease manifestations, including anti-Ro, anti-La antibodies associated with Sjögren syndrome, and antiribonucleoprotein (anti-RNP) antibodies.

• In addition to anti–double-stranded DNA, complement levels, including total hemolytic complement, C3, and C4, are markers of disease activity and are found to be low in most patients with active disease.

• Quantitative immunoglobulins are useful, because patients with lupus often have hypergammaglobulinemia and have a higher incidence of immunodeficiency.

• Other autoantibodies obtained should be guided by clinical and laboratory manifestations, such as petechiae, anemia, coagulopathy, cerebritis, and thyroid abnormalities.

Imaging Studies:

• Obtain chest radiographs and ECG.

• Other imaging studies should be guided by clinical manifestations and may include the following:

• MRI of the brain

• Renal ultrasonography

• Nuclear medicine evaluation for renal function

• High-resolution CT scan to diagnose and evaluate for pulmonary fibrosis

• Dual-energy x-ray absorptiometry (DEXA) to evaluate bone density

Other Tests:

• Obtain pulmonary function tests, including diffusing capacity of the lung for carbon monoxide (DLCO), to evaluate baseline pulmonary status and to look for subtle disease not seen on chest radiographs.

Procedures:

• The most common procedure in the diagnostic evaluation of SLE is a tissue biopsy to confirm diagnosis and to evaluate disease severity. This is particularly useful in evaluating the severity of renal involvement.

• Skin biopsy is used for diagnostic purposes when the diagnosis is not clear; lesional and sun-exposed skin may show positive immunofluorescence for complement and immune complexes. Skin biopsy is rarely necessary to make the diagnosis of SLE.

• Renal or liver biopsy is obtained more often for evaluation of disease severity and to determine the intensity of the medical regimen required for treatment.

Biopsy findings are classified according to the World Health Organization (WHO) classification and correlate with clinical morbidity and mortality. Class I is defined by normal findings on light microscopy, immunofluorescence, and electron microscopy. Class IIA disease has minimal mesangial deposits and a good prognosis, while class IIB is associated with lymphocytic infiltration and a variable prognosis. Class III disease is characterized by focal segmental proliferative mesangial changes and is associated with chronic renal disease. Class IV disease is defined as diffuse proliferation, with most glomeruli demonstrating cellular proliferation of epithelial, endothelial, and mesangial cells with cellular or fibrous crescent formation. Class IV is associated with an increased risk of end-stage renal disease. Class V disease is defined as a membranous process with significant proteinuria, which is often poorly responsive to treatment.

Staging: Lupus is not generally staged as a disease. However, staging criteria have been proposed to help assess the degree of illness. Determining which set of organs is inflamed is useful to decide treatment options. Kidney disease is classified as described in Histologic Findings. Staging for both WHO histologic class and for acuity and chronicity of renal histologic manifestations is important in determining optimal therapy.

	TREATMENT	Section 6 of 10
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Medical Care: The most important tool in the medical care of the patient with SLE is careful and frequent clinical and laboratory evaluation to tailor the medical regimen and to provide prompt recognition and treatment of disease flare, which is the cornerstone of successful intervention. As lupus is a lifelong illness, patients must be monitored indefinitely. Specific medical interventions are listed below.

Surgical Care: The need for surgical care depends on the severity of organ involvement and the need for tissue diagnosis. Usually, SLE is not a surgical condition. If surgery is necessary, monitor the patient closely for healing and evidence of infection.

Consultations: A rheumatologist should be an integral part of the medical care team supporting the lupus patient. Other consultants depend on the type of organ involvement. Consider consultation with a nephrologist for severe end-organ disease.

Diet: Diet restrictions are driven by the medical therapy. Most patients require a course of corticosteroids and should be on a no added salt, low-fat, and calcium-sufficient diet. Recognize that patients frequently try nontraditional medical remedies and food supplements. These remedies should be met with an open and supportive response. Monitoring nontraditional remedies and food supplements is important because they may alter metabolism of more traditional medications, such as warfarin sodium, or they may have a negative effect. Of note, L-canavanine in alfalfa sprouts has been implicated in causing lupus, and excess use should be avoided.

Activity: Encourage patients with SLE to maintain a normal lifestyle. Exercise is important in maintaining bone density and an appropriate weight. Caution patients that fatigue and stress have been associated with disease flares. Caution patients to avoid sunlight and to use waterproof sunblock liberally every 2 hours when exposed to the sun. Fluorescent lights may also cause increased rash in patients with SLE.

	MEDICATION	Section 7 of 10
Author Information Introduction Clinical Differentials Workup Treatment Medication Follow-up Miscellaneous Bibliography

Therapeutic interventions for pediatric lupus should occur under the direction or with the advice of an experienced physician. Many medications are used to treat lupus and are chosen depending on disease manifestations. The goal of therapy is to control disease manifestations, allowing the child to have a good quality of life without major disease exacerbations, as well as preventing serious organ damage that adversely affects function or life span. At the same time, the physician is challenged to prevent intolerable adverse effects from the therapeutic regimen.

Before treatment, identify organ system involvement and exclude other possible diagnoses. Many of the therapeutic options have serious adverse effects, contraindications, and drug interactions. A high risk for infection, infertility, and future cardiovascular disease exists. Most medications are considered a high risk during pregnancy. Patients with lupus who are pregnant should seek the expertise of an obstetrician and rheumatologist with experience in treating other patients with similar conditions.

The most important management tool in the treatment of SLE is meticulous and frequent re-evaluation of patients. Re-evaluation includes clinical and laboratory evaluation, allowing prompt recognition and treatment of disease flare that is essential to patient outcome.

Patients with hypertension should be treated aggressively. If hypertension is a consequence of corticosteroid therapy, consider immunomodulating medications as steroid-sparing agents to help control hypertension. For more information, see eMedicine's topic on pediatric Hypertension.

Drug Category: Antimalarial agents -- Rash and other minor symptoms including musculoskeletal symptoms can be treated with hydroxychloroquine 3-7 mg/kg/d, usually no more than 400 mg/d PO. Evidence indicates that long-term use of antimalarial drugs is steroid sparing. Hydroxychloroquine may also decrease risk of thrombotic events. Long-term use of this medication requires monitoring for retinal pigment changes every 6 months. Adverse effects are infrequent and include eye changes, GI symptoms (of which diarrhea is most prominent), and CNS changes.

Drug Name	Hydroxychloroquine (Plaquenil) -- Antimalarial drugs inhibit synthesis of DNA, RNA, and proteins by interacting with nucleic acids. Antimalarial drugs have a variety of immunosuppressive effects, can act as antioxidants, and interfere with prostaglandins. 200 mg of the sulfate salt = 155 mg of the base.
Adult Dose	200-400 mg (as sulfate salt)/d PO (3-7 mg/kg/d)
Pediatric Dose	3-7 mg (as sulfate salt)/kg/d PO; not to exceed 400 mg/d
Contraindications	Documented hypersensitivity; G-6-PD deficiency; retinal or visual field changes; porphyria; psoriasis
Interactions	Few reported; chloroquine may potentiate possible ocular toxicity of other drugs (eg, cisplatin); serum levels increase with cimetidine; magnesium trisilicate may decrease absorption
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Caution in hepatic disease, G-6-PD deficiency, psoriasis, and porphyria; not recommended for long-term use in children; perform periodic (6 mo) ophthalmologic examinations; test periodically for muscle weakness; adverse effects are infrequent and include eye changes, GI symptoms (of which diarrhea is most prominent), and CNS changes

Drug Category: Corticosteroids -- These agents elicit anti-inflammatory and immunosuppressive properties, cause profound and varied metabolic effects, and modify the body's immune response to diverse stimuli.

Treat children who have evidence of severe renal, CNS, or hematologic diseases with corticosteroids. The dose varies with intensity of the organ system involved and in select individuals with serologic disease activity. Consider initiating therapy with daily prednisone (1 mg/kg/d) or higher-dose alternate-day prednisone (5 mg/kg/d, not to exceed 150-250 mg depending on the size of the patient). Alternatively, lower-dose daily prednisone (0.5 mg/kg) may be used in conjunction with intermittent high-dose IV methylprednisolone (30 mg/kg/dose, not to exceed 1 g) on a weekly basis.

Children who are systemically ill with renal, neurologic, severe hematologic, cardiac, or pulmonary disease are begun on high-dose daily prednisone 2 mg/kg/d (not to exceed 80 mg/d) in divided doses, which are consolidated after serologic disease activity is controlled and finally switched to alternate-day prednisone.

Alternatively, the patient may be treated with IV pulse methylprednisolone therapy (3 d of high-dose IV corticosteroids) and then switched to intermittent high-dose IV corticosteroids with lower daily prednisone doses depending on disease severity. Obtain PPD and Candida testing before commencement of medical therapy in patients who require steroids. Consider further evaluation for mycobacterial disease in patients who are anergic to both tests.

Drug Name	Prednisone (Deltasone, Orasone, Sterapred) -- Decreases inflammation by suppression of the immune system: (1) decreased lymphocyte volume and activity, (2) decreased PMN migration, (3) decreased or reversal of capillary permeability. High doses, especially over periods >2-3 wk, suppress adrenal function.
Adult Dose	1-2 mg/kg/d PO
Pediatric Dose	1-2 mg/kg/d PO initially in divided doses up to qid, then consolidated to a daily dose before tapering the total mg/d Severe disease: 30 mg (as methylprednisolone)/kg IV infused over 1 h initially; not to exceed 1 g; may be administered as a 3-d pulse regimen or as part of a steroid regimen under the guidance of a rheumatologist
Contraindications	Documented hypersensitivity; serious infection (eg, systemic fungal infection, varicella), except septic shock or tuberculous meningitis; GI bleeding or ulceration
Interactions	Coadministration with estrogens may decrease prednisone clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics
Pregnancy	B - Usually safe but benefits must outweigh the risks.
Precautions	Carefully monitor patients receiving corticosteroids for infection and carefully evaluate in the setting of fever with no obvious source; monitor patients for diabetes mellitus, osteoporosis, osteonecrosis, hypertension, glaucoma, cataract, altered mood, and gastritis; evaluate patients for occult infection, including TB and HIV, before starting corticosteroids; avoid discontinuing suddenly in patients receiving long-term steroids, even in active infection; infection can cause disease flare, and sudden discontinuation of steroids may cause an Addisonian crisis (carefully consider steroids in active infection and discuss with experienced physicians); consider alternate immunosuppression in patients who develop diabetes mellitus while on corticosteroids and taper steroids carefully; in the interim, the use of insulin may be required

Drug Category: Immunosuppressive agents -- Evaluate children with signs of active nephritis to determine the WHO classification category of their nephritis. Patients with class IV nephritis and some patients with class III nephritis should be treated with corticosteroids and cyclophosphamide. Mycophenolate mofetil has become an alternative therapy for lupus nephritis. Azathioprine is used for more mild nephritis. Consider cyclophosphamide for severe systemic involvement of other vital organs, especially the brain. Other agents (eg, mycophenolate mofetil, cyclosporine, methotrexate) are considered when standard therapies have failed.

Other treatments under study include hormonal therapy and biologic agents that target cytokine production and anti-DNA antibodies. Clinical trials using autologous and stem cell transplantation are in progress for severe persistent disease. Most recently, anti-CD19 monoclonal antibodies (ie, rituximab) initially developed for treatment of B cell malignancies have shown promise in the treatment of lupus, in particular cytopenias and kidney disease resistant to other forms of therapy.

Drug Name	Cyclophosphamide (Cytoxan, Neosar) -- Interferes with normal function of DNA by alkylation and cross-linking the strands of DNA and by possible protein modification. Chemically related to nitrogen mustards. As an alkylating agent, mechanism of action of active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells.
Adult Dose	500-1000 mg/m2 IV q3-4wk
Pediatric Dose	500-750 mg/m2 IV q3-4wk; not to exceed 1 g/m2 Note: Should be administered IV with continuous hydration and monitoring; monitor WBCs at 8-14 d following each dose (adjust dose to maintain WBCs >2000-3000/mm3) Concomitant use of mesna to reduce toxicity is strongly recommended; antiemetics are often necessary adjuncts
Contraindications	Documented hypersensitivity; severely depressed bone marrow function; infection
Interactions	Allopurinol, may increase risk of bleeding or infection and enhance myelosuppressive effects; may potentiate doxorubicin-induced cardiotoxicity; may reduce digoxin serum levels and antimicrobial effects of quinolones; chloramphenicol may increase half-life while decreasing metabolite concentrations; may increase effect of anticoagulants; coadministration with high doses of phenobarbital may increase rate of metabolism and leukopenic activity; thiazide diuretics may prolong cyclophosphamide-induced leukopenia and neuromuscular blockade by inhibiting cholinesterase activity
Pregnancy	D - Unsafe in pregnancy
Precautions	Regularly examine hematologic profile (particularly neutrophils and platelets) to monitor for hematopoietic suppression; regularly examining urine for RBCs is not useful because most patients already have RBCs in their urine secondary to lupus nephritis; patients have WBC nadir 8-11 d after therapy, monitor closely for infection; pregnancy test required for females with child-bearing potential prior to each infusion

Drug Name	Mycophenolate mofetil (CellCept, Myfortic) -- Immunosuppressant agent used in conjunction with other immunosuppressive therapies (eg, corticosteroids) to treat lupus kidney disease. May be considered as steroid-sparing agent for other organ-specific disease manifestations. Inhibits inosine monophosphate dehydrogenase (IMPDH) and suppresses de novo purine synthesis by lymphocytes, inhibiting their proliferation. Inhibits antibody production by inhibiting T- and B-cell proliferation, cytotoxic T-cell generation, and antibody secretion. Two formulations are available and are not interchangeable. The original formulation, mycophenolate mofetil (MMF, CellCept), is a prodrug that, once hydrolyzed in vivo, releases active moiety mycophenolic acid. A newer formulation, mycophenolic acid (MPA, Myfortic), is an enteric-coated product that delivers the active moiety.
Adult Dose	CellCept: 1-1.5 g PO bid 0.5 g/dose may be considered for nonrenal disease manifestations Myfortic: 720 mg PO bid
Pediatric Dose	CellCept: 600 mg/m2/dose PO bid; not to exceed 1 g bid Alternatively, may dose according to BSA: BSA 1.25-1.5 m2: 750 mg cap PO bid BSA >1.5 m2: 1 g cap or tab PO bid Myfortic: BSA <1.19 m2: Unable to accurately administer Myfortic tab BSA 1.19-1.58 m2: 400 mg/m2 PO bid; not to exceed 1080 mg/d BSA >1.58 m2: 400 mg/m2 PO bid; not to exceed 1440 mg/d
Contraindications	Documented hypersensitivity
Interactions	Drugs that alter GI flora and antacids decrease MPA absorption; acyclovir and ganciclovir increase plasma concentrations; cholestyramine decreases mycophenolate concentration by 40%; probenecid increases mycophenolate concentration; salicylates increase free fraction of MPA; phenytoin decreases protein binding of phenytoin from 90% to 87%; theophylline decreases protein binding of theophylline from 53% to 45%
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Dosage has to be adjusted in patients with chronic renal impairment, hepatic impairment or neutropenia; immunosuppression with mycophenolate may result in an increased susceptibility to infection and an increased risk of developing lymphomas and other malignancies, particularly of the skin; effective contraception should be initiated prior to use and continued for 6 wk after discontinuation; caution in active peptic ulcer disease; commonly causes constipation, nausea, diarrhea, urinary tract infection, and nasopharyngitis; rare reports include interstitial lung disorders, colitis, pancreatitis, intestinal perforation, GI hemorrhage, gastric ulcers, duodenal ulcers, ileus; do not chew, crush, or cut Myfortic tab

Drug Name	Azathioprine (Imuran) -- Antagonizes purine metabolism and may inhibit synthesis of proteins, RNA, and DNA. May interfere with mitosis and cellular metabolism.
Adult Dose	1-2.5 mg/kg/d PO qd
Pediatric Dose	1-3 mg/kg/d PO qd
Contraindications	Documented hypersensitivity
Interactions	Toxicity increases with allopurinol (decrease azathioprine dose by 25-33%); concurrent use with ACE inhibitors may induce severe leukopenia; may increase levels of methotrexate metabolites and decrease effects of anticoagulants, neuromuscular blockers, and cyclosporine
Pregnancy	D - Unsafe in pregnancy
Precautions	Monitor carefully for renal toxicity and hepatotoxicity; use with care in patients with liver or renal disease

Drug Category: Calcium and vitamin D supplements -- All patients with SLE who are on corticosteroids or who have arthritis are at increased risk for osteopenia and its complications. Diet and appropriate supplementation with vitamin D and calcium are important tools for bone health in these patients.

Drug Name	Calcium carbonate (Oystercal, Caltrate) -- Used as an antacid and for the prevention of calcium depletion. Calcium carbonate 1 g = 400 mg elemental calcium.
Adult Dose	800-1200 mg (as elemental Ca)/d PO
Pediatric Dose	Doses are expressed as elemental calcium <6 months: 360 mg/d PO 6-12 months: 540 mg/d PO 1-10 years: 800 mg/d PO 11-18 years: 1200 mg/d PO
Contraindications	Hypercalcemia; renal calculi; ventricular fibrillation; risk of digitalis toxicity; renal or cardiac disease
Interactions	Use with caution in patients using digitalis; may antagonize effects of calcium channel blockers; decreases bioavailability of tetracyclines, fluoroquinolones, iron salts, salicylates
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Use with caution in patients with renal disease, cardiac disease, or sarcoidosis

Drug Name	Calcifediol (Calderol) -- 25-Hydroxycholecalciferol. Vitamin D regulates calcium homeostasis, promoting absorption of calcium by the gut, resorption of calcium by the kidney, and increasing bone mineral metabolism.
Adult Dose	20-100 mcg/d PO; titrate to obtain reference range serum calcium and phosphorus levels
Pediatric Dose	Not established, limited data suggest: <30 kilograms: 20 mcg PO 3 times/wk >30 kilograms: 50 mcg PO 3 times/wk
Contraindications	Documented hypersensitivity; hypercalcemia
Interactions	Effects enhanced by thiazide diuretics and reduced by cholestyramine and colestipol; may precipitate arrhythmia in conjunction with digitalis
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Calcium-phosphorus product (serum calcium X serum phosphorus) must not exceed 70; avoid hypercalcemia

Drug Category: Nonsteroidal anti-inflammatory drugs (NSAIDs) -- A child who presents with mild disease with no evidence of nephritis, hypocomplementemia, and elevated anti–double-stranded DNA antibodies is treated symptomatically and is monitored closely for signs of disease progression. Arthritis is treated with NSAIDs. Select a specific agent based on patient response to medication, history of previous drug allergy or reaction, and ease of use.
Administer NSAIDs with caution in any patient with renal or liver disease and avoid administering NSAIDs during pregnancy. NSAIDs have a variety of adverse effects that should be monitored, including gastritis, bone marrow suppression, hepatitis, interstitial nephritis, and CNS changes. Occasionally, a patient with SLE has a hypersensitivity reaction to NSAIDs, most often characterized as hepatotoxicity, but the reaction can include other symptoms and must be kept in mind.

Drug Name	Naproxen (Aleve, Naprelan, Naprosyn) -- Used for analgesic and anti-inflammatory properties to treat arthralgia and arthritis. Available with slightly different safety and efficacy profiles. Inhibits inflammatory reactions and pain by decreasing activity of cyclo-oxygenase, which is responsible for prostaglandin synthesis. Available in SR formulation (Naprelan) for once daily dosing.
Adult Dose	500-1000 mg/d PO divided bid
Pediatric Dose	7-20 mg/kg/d PO divided bid/tid; not to exceed adult dose
Contraindications	Documented hypersensitivity; gastritis; hepatic or renal insufficiency; coagulopathy; other conditions in which changes in platelet function could be harmful
Interactions	Coadministration with aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity of NSAIDs; may decrease effect of hydralazine, captopril, beta-blockers; may decrease diuretic effects of furosemide and thiazides; monitor PT closely (instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; phenytoin levels may be increased when administered concurrently
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Pregnancy category D in third trimester; acute renal insufficiency, interstitial nephritis, hyperkalemia, hyponatremia, and renal papillary necrosis may occur; patients with preexisting renal disease or compromised renal perfusion risk acute renal failure; leukopenia occurs rarely, is transient, and usually returns to normal during therapy; persistent leukopenia, granulocytopenia, or thrombocytopenia warrants further evaluation and may require discontinuation of drug

Drug Name	Tolmetin (Tolectin) -- Used for their analgesic and anti-inflammatory properties treating arthralgia and arthritis. Available with slightly different safety and efficacy profiles. Inhibits inflammatory reactions and pain by decreasing activity of cyclo-oxygenase, which is responsible for prostaglandin synthesis.
Adult Dose	1200-1800 mg/d PO divided tid
Pediatric Dose	15-30 mg/kg/d PO divided tid/qid; not to exceed adult dose
Contraindications	Documented hypersensitivity; gastritis; hepatic or renal insufficiency; coagulopathy; other conditions in which changes in platelet function could be harmful
Interactions	Coadministration with aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity of NSAIDs; may decrease effect of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; monitor PT closely (instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; phenytoin levels may be increased when administered concurrently
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Pregnancy category D in third trimester; acute renal insufficiency, interstitial nephritis, hyperkalemia, hyponatremia, and renal papillary necrosis may occur; patients with preexisting renal disease or compromised renal perfusion risk acute renal failure; leukopenia occurs rarely, is transient, and usually returns to normal during therapy; persistent leukopenia, granulocytopenia, or thrombocytopenia warrants further evaluation and may require discontinuation of drug

Drug Name	Diclofenac (Voltaren, Cataflam) -- Inhibits prostaglandin synthesis by decreasing activity of enzyme cyclo-oxygenase, which, in turn, decreases formation of prostaglandin precursors. Also available in SR formulation (Voltaren-XR [100 mg]) that allows once or twice daily dosing.
Adult Dose	100-200 mg/d PO divided bid
Pediatric Dose	<12 years: Not recommended >12 years: 2-3 mg/kg/d PO divided bid; not to exceed adult dose
Contraindications	Documented hypersensitivity; gastritis; hepatic or renal insufficiency; coagulopathy; other conditions in which changes in platelet function could be harmful
Interactions	Coadministration with aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity of NSAIDs; may decrease effect of hydralazine, captopril, beta-blockers; may decrease diuretic effects of furosemide and thiazides; monitor PT closely (instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; phenytoin levels may be increased when administered concurrently
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Pregnancy category D in third trimester; acute renal insufficiency, hyperkalemia, hyponatremia, interstitial nephritis, and renal papillary necrosis may occur; increases risk of acute renal failure in patients with preexisting renal disease or compromised renal perfusion; low WBC counts occur rarely and usually return to normal in ongoing therapy; discontinuation of therapy may be necessary if persistent leukopenia, granulocytopenia, or thrombocytopenia exists

	FOLLOW-UP	Section 8 of 10
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Further Inpatient Care:

• Further inpatient care is required for severe hematologic, nephrologic, neurologic, or psychiatric disease or for complications from these (eg, severe anemia, renal failure, stroke, seizure), including the use of intravenous high-dose corticosteroids or chemotherapy as required.

• Hospitalization may also be required for severe hypertension.

• Inpatient care is appropriate for the patient with unexplained fever to provide sepsis evaluation and treatment, as well as to evaluate the patient for disease flare and to treat accordingly.

Further Outpatient Care:

• Patients with SLE have a chronic, often persistent, disease and require constant monitoring of disease activity and modification of medical regimen according to fluctuations in disease status.

In/Out Patient Meds:

• See Medication.

Transfer:

• Consider transfer to a tertiary care facility for all children with SLE.

Deterrence/Prevention:

• Disease flares lead to poor outcome because of re-injury to vital organs. A poor outcome can be prevented with meticulous medical surveillance and attention to the chronic nature of the disease. Patient and family education is extremely important in this regard.

• Some flares are the result of excessive sun exposure. These can be avoided using sun protection.

Complications:

• SLE is a high-risk disease with the possibility of end-organ damage to any vital or nonvital organ. This damage can severely affect organ function and can lead to decreased quality of life.

• Treatment of SLE is also fraught with potential complications from steroid adverse effects, infection from immunosuppression, and cardiovascular disease leading to early myocardial infarction.

• Pregnancy can also complicate SLE. Pregnancy increases the risk of renal disease, thrombophlebitis, and disease flare. The infant is at risk for being small for gestational age (SGA) and for neonatal lupus.

Prognosis:

• Current mortality figures suggest that patients have a 95% rate of survival at 5 years. Some clinicians report a 98-100% survival rate at 5 years. These figures depend on disease severity and compliance with therapy.

• Mortality rates rise over time, with the major causes of death being infection, nephritis, CNS disease, pulmonary hemorrhage, and myocardial infarction. One indicator of morbidity and mortality risk is frequency of emergency department visits.

Patient Education:

• The patient and family must have a thorough understanding of the disease, potential severity, and complications of the disease and therapy.

• Treatment is difficult, especially for adolescent patients. The physician and parents should expect issues, including depression and noncompliance, to arise. The best method for deterrence is to thoroughly educate the patient and family through discussion, support groups, and literature.

• For excellent patient education resources, visit eMedicine's Blood and Lymphatic System Center and Muscle Disorders Center. Also, see eMedicine's patient education articles, Lupus (Systemic Lupus Erythematosus), Chronic Fatigue Syndrome, and Chronic Pain.

	MISCELLANEOUS	Section 9 of 10
Author Information Introduction Clinical Differentials Workup Treatment Medication Follow-up Miscellaneous Bibliography

Medical/Legal Pitfalls:

• Review disease and medication risks carefully with the patient and family prior to treatment with any new medication.

• Review the risks of discontinuing therapy and noncompliance with the patient and family.

• Review diagnosis and prognosis of any new organ manifestations with patient and family.

Special Concerns:

• Educate all patients with SLE with regard to the serious complications possible from unplanned pregnancy, poor compliance, recreational drug use, and infection, including sexually transmitted diseases (STDs). Poor compliance, in particular, is a significant prognostic factor.

	BIBLIOGRAPHY	Section 10 of 10
Author Information Introduction Clinical Differentials Workup Treatment Medication Follow-up Miscellaneous Bibliography

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