eMedicine Specialties > Emergency Medicine > Rheumatology
Systemic Lupus Erythematosus: Treatment & Medication
Updated: Oct 27, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Emergency Department Care
- Emergent complications of systemic lupus erythematosus (SLE) are managed in the usual manner.
- These can include strokes, acute myocardial infarctions, hemoptysis, respiratory distress, and pulmonary emboli.
- In patients who present with fever, treating for an infection empirically may be necessary until culture results have been received.
- Individual treatment plans are beyond the scope of this article and are discussed in other articles (see Differentials and Complications).
Consultations
- Consultation with a rheumatologist indicated for patients who present with symptoms suggestive of SLE and for patients with known disease.
- For complications such as pericardial tamponade, pulmonary hemorrhage, renal failure, or cerebritis, the appropriate subspecialist needs to be consulted.
- For cases of pregnancy associated with antiphospholipid antibody, the patient should be followed up by a high-risk obstetrician due to the increased risk of preeclampsia and placental insufficiency.22
Medication
Conservative management with nonsteroidal anti-inflammatory drugs, including salicylates, is recommended for arthritis, arthralgia, and myalgia not requiring immunosuppression. Only initiate high-dose glucocorticoids and cytotoxic agents by or in consultation with a rheumatologist. Patients with thrombosis require anticoagulation with warfarin for a target international normalized ratio (INR) of 3-3.5. Antibiotics may be appropriate in the treatment of ordinary and opportunistic infections.
Nonacetylated salicylates
These agents are indicated to manage the inflammatory process.
Choline magnesium trisalicylate (Trilisate)
An excellent initial management drug, which has less GI symptoms and less impairment of platelets and renal function than acetylated agents.
Salicylates have analgesic, antipyretic, anti-inflammatory, and antirheumatic effects. The pharmacologic effects of these agents are qualitatively similar. Their anti-inflammatory and analgesic activity may be mediated through the inhibition of the prostaglandin synthetase enzyme complex.
Adult
500 mg to 1.5 g PO bid/tid
Maintenance dose: 1-4.5 g/d PO
Pediatric
<37 kg: 50 mg/kg/d PO divided bid
>37 kg: Administer as in adults
Salicylate intoxication may occur with coadministration of carbonic anhydrase inhibitors; corticosteroids decrease salicylate serum levels; may have additive hypoprothrombinemic effect and may increase bleeding time
Documented hypersensitivity; bleeding disorders
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
Associated with Reye syndrome in children and teenagers with influenza or chickenpox; discontinue if dizziness, ringing in ears, or impaired hearing occurs (may represent toxicity); caution in liver damage, preexisting hypoprothrombinemia, and vitamin K deficiency
Nonsteroidal anti-inflammatory drugs (NSAIDs)
These agents are most commonly used for the relief of mild-to-moderate pain. Although the effects of NSAIDs in the treatment of pain tend to be patient specific, ibuprofen is usually the DOC for initial therapy. Other options include fenoprofen, flurbiprofen, mefenamic acid, ketoprofen, indomethacin, and piroxicam.
Ibuprofen (Motrin, Nuprin, Ibuprin, Advil)
Usually DOC for treatment of mild-to-moderate pain, if no contraindications exist.
Inhibits inflammatory reactions and pain probably by decreasing activity of the enzyme cyclooxygenase, which results in inhibition of prostaglandin synthesis.
Adult
400 mg PO q4-6h, 600 mg PO q6h, or 800 mg PO q8h; while symptoms persist; not to exceed 3.2 g/d
Pediatric
<6 months: Not established
6 months to 12 years: 10-70 mg/kg/d PO divided tid/qid; start at lower end of dosing range and titrate upward to a maximum of 2.4 g/d
>12 years: Administer as in adults
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
Documented hypersensitivity; peptic ulcer disease; recent GI bleeding or perforation; renal insufficiency; high risk of bleeding
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Caution in congestive heart failure, hypertension, and decreased renal and hepatic function; caution in anticoagulation abnormalities or during anticoagulant therapy
Antimalarials
These agents are an alternative conservative therapy with both sunblocking and anti-inflammatory effects. Initiation of antimalarial therapy usually does not take place in the ED setting.
Hydroxychloroquine (Plaquenil)
Inhibits chemotaxis of eosinophils and locomotion of neutrophils and impairs complement-dependent antigen-antibody reactions.
Hydroxychloroquine sulfate 200 mg is equivalent to 155 mg hydroxychloroquine base and 250 mg chloroquine phosphate. Used for treatment of discoid and systemic lupus erythematosus and rheumatoid arthritis.
Adult
200-400 mg PO qd for several wk depending on response; 200 mg/d for prolonged maintenance therapy
Pediatric
3-5 mg base/kg/d PO qd or divided bid; not to exceed 7 mg/kg/d
Serum levels increase with cimetidine; magnesium trisilicate may decrease absorption
Documented hypersensitivity; psoriasis; retinal and visual field changes attributable to 4-aminoquinolones
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 in children; perform periodic (6 mo) ophthalmologic examinations; test periodically for muscle weakness
Glucocorticoids
These agents have both anti-inflammatory properties and cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli. High-dose glucocorticoids are used for severe SLE complications, such as hematologic or CNS disease, serositis, vasculitis, or glomerulonephritis.
Prednisone (Deltasone, Orasone, Sterapred)
Used as an immunosuppressant in treatment of autoimmune disorders. By reversing increased capillary permeability and suppressing PMN activity, it may decrease inflammation.
Adult
5-60 mg/d PO qd or divided bid/qid; taper over 2 wk as symptoms resolve
Pediatric
4-5 mg/m2/d PO; alternatively, 1-2 mg/kg PO qd; taper over 2 wk as symptoms resolve
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; fungal, tubercular skin, connective tissue, or viral infections; peptic ulcer disease; hepatic dysfunction; GI disease
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use
Methylprednisolone (Solu-Medrol, Depo-Medrol, Adlone)
Useful in treatment of inflammatory and autoimmune reactions. By reversing increased capillary permeability and suppressing PMN activity, it may decrease inflammation.
Adult
Loading dose: 125-250 mg IV
Maintenance dose: 0.5-1 mg/kg/dose IV q6h for up to 5 d
Pediatric
Loading dose: 2 mg/kg IV
Maintenance dose: 0.5-1 mg/kg/dose IV q6h for up to 5 d
Coadministration with digoxin may increase digitalis toxicity secondary to hypokalemia; estrogens may increase levels of methylprednisolone; phenobarbital, phenytoin, and rifampin may decrease levels of methylprednisolone (adjust dose); monitor patients for hypokalemia when taking medication concurrently with diuretics
Documented hypersensitivity; viral, fungal or tubercular skin infections
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
Hyperglycemia, edema, osteonecrosis, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, growth suppression, myopathy, and infections are possible complications of glucocorticoid use
Immunosuppressives/cytotoxic agents
These agents are used in active SLE cases resistant to corticosteroids. Patients should be monitored closely because of the adverse effects, and these agents should be administered by experienced specialist physicians. These drugs are more beneficial when used in conjunction with glucocorticoids. Treatment with glucocorticoids plus cyclophosphamide is more beneficial, although more toxic, than treatment with glucocorticoids plus azathioprine.
Cyclophosphamide (Cytoxan, Neosar)
Chemically related to nitrogen mustards. As an alkylating agent, the mechanism of action of the active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells. Pediatric dosing is controversial and not first line.
Adult
10-20 mg/kg IV q3-4wk or 1.5-2.5 mg/kg PO qd
Pediatric
Not established
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
Documented hypersensitivity; severely depressed bone marrow function
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 examine urine for RBCs, which may precede hemorrhagic cystitis
Azathioprine (Imuran)
Antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. May decrease proliferation of immune cells, which results in lower autoimmune activity. Pediatric dosing is controversial and not first line.
Adult
1.5-2.5 mg/kg PO qd
Pediatric
Not established
Toxicity increases with allopurinol; 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; low levels of serum thiopurine methyltransferase (TPMT)
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Increases risk of neoplasia; caution with liver disease and renal impairment; hematologic toxicities may occur; check TPMT level before therapy and monitor liver, renal, and hematologic function; pancreatitis rarely associated
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 |
| Multimedia: Systemic Lupus Erythematosus |
| References |
| « Previous Page | Next Page » |
References
Munoz LE, Gaipl US, Franz S, Sheriff A, Voll RE, Kalden JR. SLE--a disease of clearance deficiency?. Rheumatology (Oxford). Sep 2005;44(9):1101-7. [Medline].
Rahman A, Isenberg DA. Systemic lupus erythematosus. N Engl J Med. Feb 28 2008;358(9):929-39. [Medline].
D'Cruz DP, Khamashta MA, Hughes GR. Systemic lupus erythematosus. Lancet. Feb 17 2007;369(9561):587-96. [Medline].
Uramoto KM, Michet CJ Jr, Thumboo J, Sunku J, O'Fallon WM, Gabriel SE. Trends in the incidence and mortality of systemic lupus erythematosus, 1950-1992. Arthritis Rheum. Jan 1999;42(1):46-50. [Medline].
Danchenko N, Satia JA, Anthony MS. Epidemiology of systemic lupus erythematosus: a comparison of worldwide disease burden. Lupus. 2006;15(5):308-18. [Medline].
Abu-Shakra M, Urowitz MB, Gladman DD, Gough J. Mortality studies in systemic lupus erythematosus. Results from a single center. II. Predictor variables for mortality. J Rheumatol. Jul 1995;22(7):1265-70. [Medline].
Gladman DD, Urowitz MB. Prognosis, mortality and morbidity in systemic lupus erythematosus. In: Wallace DJ, Hahn BH. Dubois' lupus erythematosus. 7th ed. Philadelphia: Lippincott Williams & Wilkins; 2007:1333-53.
Cervera R, Khamashta MA, Font J, Sebastiani GD, Gil A, Lavilla P. Morbidity and mortality in systemic lupus erythematosus during a 5-year period. A multicenter prospective study of 1,000 patients. European Working Party on Systemic Lupus Erythematosus. Medicine (Baltimore). May 1999;78(3):167-75. [Medline].
Manzi S, Meilahn EN, Rairie JE, Conte CG, Medsger TA Jr, Jansen-McWilliams L, et al. Age-specific incidence rates of myocardial infarction and angina in women with systemic lupus erythematosus: comparison with the Framingham Study. Am J Epidemiol. Mar 1 1997;145(5):408-15. [Medline].
Murali R, Jeyaseelan L, Rajaratnam S, John L, Ganesh A. Systemic lupus erythematosus in Indian patients: prognosis, survival and life expectancy. Natl Med J India. Jul-Aug 1997;10(4):159-64. [Medline].
Xie SK, Feng SF, Fu H. Long term follow-up of patients with systemic lupus erythematosus. J Dermatol. Jun 1998;25(6):367-73. [Medline].
Wang F, Wang CL, Tan CT, Manivasagar M. Systemic lupus erythematosus in Malaysia: a study of 539 patients and comparison of prevalence and disease expression in different racial and gender groups. Lupus. 1997;6(3):248-53. [Medline].
Manzi S. Epidemiology of systemic lupus erythematosus. Am J Manag Care. Oct 2001;7(16 Suppl):S474-9. [Medline].
Klein-Gitelman M, Reiff A, Silverman ED. Systemic lupus erythematosus in childhood. Rheum Dis Clin North Am. Aug 2002;28(3):561-77, vi-vii. [Medline].
Dubois EL, Tuffanelli DL. Clinical manifestations of systemic lupus erythematosus. Computer analysis of 250 cases. JAMA. Oct 12 1964;190:104-11. [Medline].
Harvey AM, Shulman LE, Tumulty PA, Conley CL, Schoenrich EH. Systemic lupus erythematosus: review of the literature and clinical analysis of 138 cases. Medicine (Baltimore). Dec 1954;33(4):291-437. [Medline].
Tan EM, Cohen AS, Fries JF, Masi AT, McShane DJ, Rothfield NF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. Nov 1982;25(11):1271-7. [Medline].
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].
Ramos-Casals M, Cuadrado MJ, Alba P, Sanna G, Brito-Zerón P, Bertolaccini L. Acute viral infections in patients with systemic lupus erythematosus: description of 23 cases and review of the literature. Medicine (Baltimore). Nov 2008;87(6):311-8. [Medline].
Fessler BJ. Infectious diseases in systemic lupus erythematosus: risk factors, management and prophylaxis. Best Pract Res Clin Rheumatol. Apr 2002;16(2):281-91. [Medline].
Tierney, et al. Current Medical Diagnosis and Treatment. 2001:841-844.
Nodler J, Moolamalla SR, Ledger EM, Nuwayhid BS, Mulla ZD. Elevated antiphospholipid antibody titers and adverse pregnancy outcomes: analysis of a population-based hospital dataset. BMC Pregnancy Childbirth. Mar 16 2009;9:11. [Medline].
Ruiz-Irastorza G, Khamashta MA, Castellino G, Hughes GR. Systemic lupus erythematosus. Lancet. Mar 31 2001;357(9261):1027-32. [Medline].
Amit M, Molad Y, Levy O, Wysenbeek AJ. Headache in systemic lupus erythematosus and its relation to other disease manifestations. Clin Exp Rheumatol. Jul-Aug 1999;17(4):467-70. [Medline].
Braunwald E, Fauci AS, Kasper DL. Systemic Lupus Erythematosus. In: Harrison's Principles of Internal Medicine. 16th ed. 2005:1960-1967.
Goldsmith L, Lazarus G, Tharp M. Adult and Pediatric Dermatology: A Color Atlas of Disease and Treatment. 1997.
Gourley MF, Austin HA 3rd, Scott D, Yarboro CH, Vaughan EM, Muir J, et al. Methylprednisolone and cyclophosphamide, alone or in combination, in patients with lupus nephritis. A randomized, controlled trial. Ann Intern Med. Oct 1 1996;125(7):549-57. [Medline].
Hahn BH. Management of systemic lupus erythematosus. In: Textbook of Rheumatology. Vol 2. WB Saunders; 1997:1040-56.
Hochberg MC, Silman AJ, Smolen JS, et al. Systemic lupus erythematosus. In: Practical Rheumatology. 3rd ed. 2004:417-437.
Khoshbin S, Glanz BI, Schur PH. Neuropsychiatric syndromes in systemic lupus erythematosus: a new look. Clin Exp Rheumatol. Jul-Aug 1999;17(4):395-8. [Medline].
Lahita RG. Clinical presentation of systemic lupus erythematosus. In: Textbook of Rheumatology. Vol 2. WB Saunders; 1997:1028-39.
Marks R. Skin Diseases in Old People. 2nd ed. Martin Dunitz Ltd; 1999.
Mills JA. Systemic lupus erythematosus. N Engl J Med. Jun 30 1994;330(26):1871-9. [Medline].
Mochizuki T, Aotsuka S, Satoh T. Clinical and laboratory features of lupus patients with complicating pulmonary disease. Respir Med. Feb 1999;93(2):95-101. [Medline].
Mok CC, Wong RW. Pregnancy in systemic lupus erythematosus. Postgrad Med J. Mar 2001;77(905):157-65. [Medline].
Molokhia M, Hoggart C, Patrick AL, Shriver M, Parra E, Ye J, et al. Relation of risk of systemic lupus erythematosus to west African admixture in a Caribbean population. Hum Genet. Mar 2003;112(3):310-8. [Medline].
Roman MJ, Shanker BA, Davis A, Lockshin MD, Sammaritano L, Simantov R, et al. Prevalence and correlates of accelerated atherosclerosis in systemic lupus erythematosus. N Engl J Med. Dec 18 2003;349(25):2399-406. [Medline].
Stevenson FK, Natvig J. Autoantibodies revealed: the role of B cells in autoimmune disease. Immunol Today. Jul 1999;20(7):296-8. [Medline].
Urowitz MB, Gladman DD. How to improve morbidity and mortality in systemic lupus erythematosus. Rheumatology (Oxford). Mar 2000;39(3):238-44. [Medline].
Wang CR, Chou CC, Hsieh KH, Chuang CY, Chen CY. Lupus patients with peripheral vascular thrombosis: the significance of measuring anticardiolipin antibody. Am J Emerg Med. Sep 1993;11(5):468-70. [Medline].
Further Reading
Keywords
systemic lupus erythematosus, SLE symptoms, SLE causes, SLE, autoimmune disease, lupus nephritis, lupus pneumonitis, pulmonary hypertension, stroke, myocardial infarction, septic arthritis, avascular necrosis, seizures, sensory neuropathies, sensorimotor neuropathies, retinal vasculitis, nephrotic syndrome, renal failure, vasculitis with digital infarcts, Libman-Sacks endocarditis, pericarditis, myocarditis, heart failure, arrhythmias
