eMedicine Specialties > Pediatrics: General Medicine > Rheumatology
Systemic Lupus Erythematosus: Treatment & Medication
Updated: Dec 8, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Treatment
Medical Care
The most important tool in the medical care of the patient with systemic lupus erythematosus (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. Because lupus is a lifelong illness, patients must be indefinitely monitored. Specific medical interventions are listed below. New studies bring attention to the need for preservation of gonadal function when gonadotoxic therapies are used to treat severe disease. Consideration should be given to prevention of atherosclerosis and osteoporosis because these are long-term consequences of the disease and its treatment.
Surgical Care
The need for surgical care depends on the severity of organ involvement and the need for tissue diagnosis. Usually, systemic lupus erythematosus is not a surgical condition. If surgery is necessary, closely monitor the patient 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 systemic lupus erythematosus 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 liberally apply waterproof sunblock every 2 hours when exposed to the sun. Fluorescent lights may also cause increased rash in patients with systemic lupus erythematosus.
Medication
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 is noted. 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 systemic lupus erythematosus (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 aggressively treated. 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.
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 orally. 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.
Hydroxychloroquine (Plaquenil)
Antimalarial drugs inhibit synthesis of DNA, RNA, and proteins by interacting with nucleic acids. Antimalarial drugs have various immunosuppressive effects, can act as antioxidants, and interfere with prostaglandins.
200 mg of the sulfate salt = 155 mg of the base.
Adult
200-400 mg (as sulfate salt)/d PO (3-7 mg/kg/d)
Pediatric
3-7 mg (as sulfate salt)/kg/d PO; not to exceed 400 mg/d
Few reported; chloroquine may potentiate possible ocular toxicity of other drugs (eg, cisplatin); serum levels increase with cimetidine; magnesium trisilicate may decrease absorption
Documented hypersensitivity; G-6-PD deficiency;
retinal or visual field changes; porphyria; psoriasis
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
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
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 intravenous 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 intravenous pulse methylprednisolone therapy (3 d of high-dose intravenous corticosteroids) and then switched to intermittent high-dose intravenous 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.
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
1-2 mg/kg/d PO
Pediatric
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
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
Documented hypersensitivity; serious infection (eg, systemic fungal infection, varicella), except septic shock or tuberculous meningitis; GI bleeding or ulceration
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
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
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.
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
500-1000 mg/m2 IV q3-4wk
Pediatric
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/μL)
Concomitant use of mesna to reduce toxicity is strongly recommended; antiemetics are often necessary adjuncts
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
Documented hypersensitivity; severely depressed bone marrow function; infection
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
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
Mycophenolate (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-cell 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
CellCept: 1-1.5 g PO bid
0.5 g/dose may be considered for nonrenal disease manifestations
Myfortic: 720 mg PO bid
Pediatric
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
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%
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
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
Azathioprine (Imuran)
Antagonizes purine metabolism and may inhibit synthesis of proteins, RNA, and DNA. May interfere with mitosis and cellular metabolism.
Adult
1-2.5 mg/kg/d PO qd
Pediatric
1-3 mg/kg/d PO qd
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
Documented hypersensitivity
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Monitor carefully for renal toxicity and hepatotoxicity; use with care in patients with liver or renal disease
Calcium and vitamin D supplements
All patients with systemic lupus erythematosus 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.
Calcium carbonate (Oystercal, Caltrate)
Used as an antacid and for the prevention of calcium depletion. Calcium carbonate 1 g = 400 mg elemental calcium.
Adult
800-1200 mg (as elemental Ca)/d PO
Pediatric
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
Use with caution in patients using digitalis; may antagonize effects of calcium channel blockers; decreases bioavailability of tetracyclines, fluoroquinolones, iron salts, salicylates
Hypercalcemia; renal calculi; ventricular fibrillation; risk of digitalis toxicity; renal or cardiac disease
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Use with caution in patients with renal disease, cardiac disease, or sarcoidosis
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
20-100 mcg/d PO; titrate to obtain reference range serum calcium and phosphorus levels
Pediatric
Not established, limited data suggest:
<30 kilograms: 20 mcg PO 3 times/wk
>30 kilograms: 50 mcg PO 3 times/wk
Effects enhanced by thiazide diuretics and reduced by cholestyramine and colestipol; may precipitate arrhythmia in conjunction with digitalis
Documented hypersensitivity; hypercalcemia
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Calcium-phosphorus product (serum calcium X serum phosphorus) must not exceed 70; avoid hypercalcemia
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 various adverse effects that should be monitored, including gastritis, bone marrow suppression, hepatitis, interstitial nephritis, and CNS changes. Occasionally, a patient with systemic lupus erythematosus has a hypersensitivity reaction to NSAIDs, most often characterized as hepatotoxicity, but the reaction can include other symptoms and must be kept in mind.
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
500-1000 mg/d PO divided bid
Pediatric
7-20 mg/kg/d PO divided bid/tid; not to exceed adult dose
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
Documented hypersensitivity; gastritis; hepatic or renal insufficiency; coagulopathy; other conditions in which changes in platelet function could be harmful
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
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
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
1200-1800 mg/d PO divided tid
Pediatric
15-30 mg/kg/d PO divided tid/qid; not to exceed adult dose
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; closely monitor PT (instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; phenytoin levels may be increased when administered concurrently
Documented hypersensitivity; gastritis; hepatic or renal insufficiency; coagulopathy; other conditions in which changes in platelet function could be harmful
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
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
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
100-200 mg/d PO divided bid
Pediatric
<12 years: Not recommended
>12 years: 2-3 mg/kg/d PO divided bid; not to exceed adult dose
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
Documented hypersensitivity; gastritis; hepatic or renal insufficiency; coagulopathy; other conditions in which changes in platelet function could be harmful
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
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 occurs
Monoclonal Antibody
This agent is used investigationally for systemic lupus erythematosus.
Rituxamab (Rituxan)
An anti-CD20 monoclonal antibody. Originally used to treat B-cell lymphoma, the monoclonal antibody is now used to treat persisting immune thrombocytopenia in children and rheumatoid arthritis. Use in SLE is investigational.
Adult
375 mg/m2 IV qwk for 3-8 wk; infuse at initial rate of 50 mg/h, if tolerated may escalate by 50 mg/h q30min, not to exceed 400 mg/h
Premedication with IV corticosteroids is recommended
Pediatric
Not established, limited data available; off-label use, administer as in adults
Coadministration with cisplatin is known to cause severe renal toxicity including acute renal failure; may interfere with immune response to live virus vaccine (MMR) and reduce efficacy (do not administer within 3 months of vaccine)
Documented hypersensitivity; IgE-mediated reaction to murine proteins; breastfeeding
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution with dormant infections such as hepatitis B, hepatitis C, or CMV due to risk of reactivation; caution with abnormal cardiac, pulmonary, or renal function; hypotension, bronchospasm, and angioedema may occur, premedication with acetaminophen and diphenhydramine may decrease incidence; discontinue treatment if life-threatening cardiac arrhythmias occur; must administer by slow IV infusion, do not administer IV push or bolus
More on Systemic Lupus Erythematosus |
| Overview: Systemic Lupus Erythematosus |
| Differential Diagnoses & Workup: Systemic Lupus Erythematosus |
 Treatment & Medication: Systemic Lupus Erythematosus |
| Follow-up: Systemic Lupus Erythematosus |
| References |
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References
Osler W. On the visceral manifestations of the erythema group of skin diseases. Am J Med Sci. 1904;27:1.
Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. Sep 1997;40(9):1725. [Medline].
Yurasov S, Wardemann H, Hammersen J, et al. Defective B cell tolerance checkpoints in systemic lupus erythematosus. Journal of Experimental Medicine. 2005;201:703-711. [Medline].
Weening JJ, D'Agati VD, Schwartz MM, Seshan SV, Alpers CE, Appel GB. The classification of glomerulonephritis in systemic lupus erythematosus revisited. Kidney Int. Feb 2004;65(2):521-30. [Medline].
Agnello V. Complement deficiency states. Medicine (Baltimore). Jan 1978;57(1):1-23. [Medline].
Arbuckle MR, McClain MT, Rubertone MV, et al. Development of autoantibodies before the clinical onset of systemic lupus erythematosus. New England Journal of Medicine. 2003;349:1526-1533. [Medline].
Aringer M, Wintersberger W, Steiner CW, et al. High levels of bcl-2 protein in circulating T lymphocytes, but not B lymphocytes, of patients with systemic lupus erythematosus. Arthritis Rheum. Oct 1994;37(10):1423-30. [Medline].
Athreya BH, Rafferty JH, Sehgal GS, Lahita RG. Adenohypophyseal and sex hormones in pediatric rheumatic diseases. J Rheumatol. Apr 1993;20(4):725-30. [Medline].
Balow JE, Austin HA, Tsokos GC, et al. NIH conference. Lupus nephritis. Ann Intern Med. Jan 1987;106(1):79-94. [Medline].
Belmont HM, Storch M, Buyon J, Abramson S. New York University/Hospital for Joint Diseases experience with intravenous cyclophosphamide treatment: efficacy in steroid unresponsive lupus nephritis. Lupus. Apr 1995;4(2):104-8. [Medline].
Benseler SM, Silverman ED. Systemic Lupus Erythematosus. Pediatric Clinics of North America. 2005;52:443-467. [Medline].
Bonfa E, Golombek SJ, Kaufman LD, et al. Association between lupus psychosis and anti-ribosomal P protein antibodies. N Engl J Med. Jul 30 1987;317(5):265-71. [Medline].
Boumpas DT, Austin HA, Vaughn EM, et al. Controlled trial of pulse methylprednisolone versus two regimens of pulse cyclophosphamide in severe lupus nephritis. Lancet. Sep 26 1992;340(8822):741-5. [Medline].
Bowness P, Davies KA, Norsworthy PJ, et al. Hereditary C1q deficiency and systemic lupus erythematosus. QJM. Aug 1994;87(8):455-64. [Medline].
Brinciotti M, Ferrucci G, Trasatti G, et al. Reflex seizures as initial manifestations of systemic lupus erythematosus in childhood. Lupus. Aug 1993;2(4):281-3. [Medline].
Brown DA, Rose JK. Sorting of GPI-anchored proteins to glycolipid-enriched membrane subdomainsduring transport to the apical cell surface. Cell. Feb 7 1992;68(3):533-44. [Medline].
Brunner HI, Ruth NM, German A, et al. Initial validation of the pediatric automated neuropsychological assessment metrics for Childhood-Onset systemic lupus erythematosus. Arthritis Rheum. Sept 2007;57(7):1174-1182. [Medline].
Cassidy JT, Petty RE. Textbook of Pediatric Rheumatology. Philadelphia, Pa: WB Saunders; 2005.
Cunningham S, Conway EE Jr. Systemic lupus erythematosus presenting as an intracranial bleed. Ann Emerg Med. Jul 1991;20(7):810-2. [Medline].
DelGiudice GC, Scher CA, Athreya BH, Diamond GR. Pseudotumor cerebri and childhood systemic lupus erythematosus. J Rheumatol. Aug 1986;13(4):748-52. [Medline].
Esdaile JM. The efficacy of antimalarials in systemic lupus erythematosus. Lupus. Feb 1993;2 Suppl 1:S3-8. [Medline].
Fauci AS, Steinberg AD, Haynes BF, Whalen G. Immunoregulatory aberrations in systemic lupus erythematosus. J Immunol. Oct 1978;121(4):1473-9. [Medline].
Ferraz MB, Goldenberg J, Hilario MO, et al. Evaluation of the 1982 ARA lupus criteria data set in pediatric patients. Committees of Pediatric Rheumatology of the Brazilian Society of Pediatrics and the Brazilian Society of Rheumatology. Clin Exp Rheumatol. Jan-Feb 1994;12(1):83-7. [Medline].
Feutren G, Querin S, Noel LH, et al. Effects of cyclosporine in severe systemic lupus erythematosus. J Pediatr. Dec 1987;111(6 Pt 2):1063-8. [Medline].
Frank MM, Hamburger MI, Lawley TJ, et al. Defective reticuloendothelial system Fc-receptor function in systemic lupus erythematosus. N Engl J Med. Mar 8 1979;300(10):518-23. [Medline].
Fukuda M, Kamiyama Y, Kawahara K, et al. The favourable effect of cyclophosphamide pulse therapy in the treatment of massive pulmonary haemorrhage in systemic lupus erythematosus. Eur J Pediatr. Mar 1994;153(3):167-70. [Medline].
Gelfand MC, Steinberg AD, Nagle R, Knepshield JH. Therapeutic studies in NZB-W mice. I. Synergy of azathioprine, cyclophosphamide and methylprednisolone in combination. Arthritis Rheum. May-Jun 1972;15(3):239-46. [Medline].
Gieron MA, Khoromi S, Campos A. MRI changes in the central nervous system in a child with lupus erythematosus. Pediatr Radiol. 1995;25(3):184-5. [Medline].
Golombek SJ, Graus F, Elkon KB. Autoantibodies in the cerebrospinal fluid of patients with systemic lupus erythematosus. Arthritis Rheum. Sep 1986;29(9):1090-7. [Medline].
Hargraves MM, Richmond H, Morton R. Presentation of two bone marrow elements: the "tart" cell and the "L.E." cell. Proc Staff Meet Mayo Clin. 1948;23:25.
Harter JG, Reddy WJ, Thorn GW. Studies on an intermittent corticosteroid dosage regimen. N Engl J Med. Sep 19 1963;269:591-6. [Medline].
Helyer BJ, Howie JB. Renal disease associated with positive lupus erythematosus tests in a cross-bred strain of mice. Nature. Jan 12 1963;197:197. [Medline].
Jacobs JC. Pediatric Rheumatology for the Practitioner. New York, NY: Springer-Verlag; 1992.
Klein-Gitelman M, Reiff A, Silverman E. Systemic Lupus Erythematosus in Childhood. Rheumatic Disease Clinics of North America. 2002;28:561-577. [Medline].
Lahat E, Eshel G, Azizi E, et al. Chorea associated with systemic lupus erythematosus in children. A case report. Isr J Med Sci. Oct 1989;25(10):568-70. [Medline].
Lahita RG. Systemic Lupus Erythematosus. 3rd ed. San Diego, Calif: Academic Press; 1999.
Lehman TJ. A practical guide to systemic lupus erythematosus. Pediatr Clin North Am. Oct 1995;42(5):1223-38. [Medline].
Malaviya AN, Singh RR, Sindhwani R, et al. Intermittent intravenous pulse cyclophosphamide treatment in systemic lupus erythematosus. Indian J Med Res. Apr 1992;96:101-8. [Medline].
Male C, Foulon D, Hoogendoorn H, et al. Predictive value of persistent versus transient antiphospholipid antibody subtypes for the risk of thrombotic events in pediatric patients with systemic lupus erythematosus. Blood. Dec 15 2005;106(13):4152-8. [Medline].
Montanaro A, Bardana EJ Jr. Dietary amino acid-induced systemic lupus erythematosus. Rheum Dis Clin North Am. May 1991;17(2):323-32. [Medline].
Phillips R, Lomnitzer R, Wadee AA, Rabson AR. Defective monocyte function in patients with systemic lupus erythematosus. Clin Immunol Immunopathol. Jan 1985;34(1):69-76. [Medline].
Roholt NS, Lapiere JC, Wang JI, et al. Localized linear bullous eruption of systemic lupus erythematosus in a child. Pediatr Dermatol. Jun 1995;12(2):138-44. [Medline].
Schaff Z, Barry DW, Grimley PM. Cytochemistry of tubuloreticular structures in lymphocytes from patients with systemic lupus erythematosus and in cultured human lymphoid cells: comparison to a paramyxovirus. Lab Invest. Dec 1973;29(6):577-86. [Medline].
Schanberg LE, Sanborg C. Dyslipoproteinemia and premature atherosclerosis in pediatric systemic lupus erythematosus. J. Rheumatol. Dec, 2004;6(6):425-433. [Medline].
Stichweh D, Arce E, Pascual V. Update on pediatric systemic lupus erythematosus. Current Opinion in Rheumatology. 2004;16:577-587. [Medline].
Suzuki M, Ross GF, Wiers K, et al. Identification of a urinary proteomic signature for lupus nephritis in children. Pediatr Nephrol. Sep 2007;ePub. [Medline].
Tolaymat A, Leventhal B, Sakarcan A, et al. Systemic lupus erythematosus in a child receiving long-term interferon therapy. J Pediatr. Mar 1992;120(3):429-32. [Medline].
Tsai YT, Chiang BL, Kao YF, Hsieh KH. Detection of Epstein-Barr virus and cytomegalovirus genome in white blood cells from patients with juvenile rheumatoid arthritis and childhood systemic lupus erythematosus. Int Arch Allergy Immunol. Mar 1995;106(3):235-40. [Medline].
Uziel Y, Laxer RM, Blaser S, et al. Cerebral vein thrombosis in childhood systemic lupus erythematosus. J Pediatr. May 1995;126(5 Pt 1):722-7. [Medline].
Wallace DJ, Hahn BH. Dubois' Systemic Lupus Erythematosus. 5th ed. Baltimore, MD: Williams and Wilkins; 1997.
Yancey CL, Doughty RA, Athreya BH. Central nervous system involvement in childhood systemic lupus erythematosus. Arthritis Rheum. Nov 1981;24(11):1389-95. [Medline].
Further Reading
Keywords
systemic lupus erythematosus, lupus, SLE, lupus erythematosus, LE, fevers of unknown origin, arthralgia, anemia, nephritis, psychosis, fatigue, rheumatic disease, atherosclerosis, hemolytic anemia, thrombocytopenia, leukopenia, lymphopenia, nephritis, nephrotic syndrome, serositis, arthritis, memory loss, psychosis, transverse myelitis, hemoptysis, edema of the lower extremities, headache, painful mouth sores, pleuritis, pericarditis, livedo reticularis, alopecia, Raynaud phenomenon, tendonitis, myositis, lymphadenopathy, hepatosplenomegaly, stroke, pseudotumor cerebri, cerebral venous thrombosis, aseptic meningitis, chorea, global cognitive deficits, mood disorders, transverse myelitis, hyperthyroidism
