Systemic Lupus Erythematosus (SLE) Medication

  • Author: Christie M Bartels, MD, MS; Chief Editor: Herbert S Diamond, MD   more...
 
Updated: Jan 18, 2012
 

Medication Summary

Treatment of systemic lupus erythematosus (SLE) is guided by the individual patient's manifestations. Fever, rash, musculoskeletal manifestations, and serositis generally respond to treatment with hydroxychloroquine, NSAIDS, and low-to-moderate–dose steroids, as necessary, for acute flares. Medications such as methotrexate may be useful in chronic lupus arthritis, and azathioprine and mycophenolate have been widely used in moderate severity lupus.

CNS involvement and renal disease constitute more serious disease and often require high-dose steroids and other immunosuppression agents such as cyclophosphamide, azathioprine, or mycophenolate. Class IV diffuse proliferative lupus nephritis has also been treated with aggressive cyclophosphamide induction therapy.[79, 80] Recent trials of mycophenolate have demonstrated efficacy for induction, particularly in black patients.[81, 82, 83] Rituximab trials have shown modest benefit to date.[65] The MAINTAIN trial offered data showing no statistically significant difference between mycophenolate and azathioprine for lupus nephritis maintenance.[84]

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Nonsteroidal Anti-inflammatory Drugs (NSAIDs)

Class Summary

These agents provide symptomatic relief for arthralgias, fever, and mild serositis. NSAIDs may cause elevated creatinine or liver function test results in patients with active SLE. Additionally, concomitant administration with prednisone may increase risk of GI ulceration.

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Ibuprofen (Advil, Motrin IB, Addaprin, Ibu, NeoProfen)

 

Ibuprofen is the drug of choice for patients with mild-to-moderate pain. It inhibits inflammatory reactions and pain by decreasing prostaglandin synthesis.

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Naproxen (Anaprox, Naprelan, Naprosyn)

 

Naproxen is used for relief of mild to moderate pain. It inhibits inflammatory reactions and pain by decreasing activity of the enzyme cyclooxygenase, resulting in prostaglandin synthesis.

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Diclofenac (Voltaren XR, Cataflam)

 

Diclofenac inhibits prostaglandin synthesis by decreasing activity of enzyme cyclo-oxygenase, which in turn decreases formation of prostaglandin precursors.

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Antimalarials

Class Summary

Antimalarials may work through numerous proposed mechanisms in SLE, mediating subtle immunomodulation without causing overt immunosuppression. They are useful in preventing and treating lupus skin rashes, constitutional symptoms, arthralgias, and arthritis. They also help to prevent lupus flares and have been associated with reduced morbidity and mortality in SLE patients followed in observational trials.[66]

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Hydroxychloroquine (Plaquenil)

 

This agent 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. Weight-based dose adjustment and monitoring help mitigate risk of retinal toxicity.[85]

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Immunosuppressant Agents

Class Summary

These agents act as immunosuppressives and cytotoxic and anti-inflammatory agents. Agent selection is generally indicated by organ involvement and severity. Due to toxicity, cyclophosphamide is reserved for severe organ-threatening disease. At the other end of the spectrum, methotrexate or azathioprine may be helpful for milder arthritis or skin disease. Azathioprine, mycophenolate, and cyclosporine have all been studied in lupus manifestations such as nephritis.

Griffiths et al compared the corticosteroid-sparing effect of cyclosporine with azathioprine in patients with severe SLE. The authors concluded that azathioprine may be considered first-line therapy since cyclosporine requires close monitoring of blood pressure and serum creatinine.[86] The MAINTAIN trial compared the efficacy of azathioprine and mycophenolate for lupus nephritis maintenance and showed nonsignificant reduction in lupus flares with mycophenolate.[84]

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Cyclophosphamide

 

Cyclophosphamide is used for immunosuppression in cases of serious SLE organ involvement, especially severe CNS involvement, vasculitis, and lupus nephritis. This agent is 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.

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Methotrexate (Trexall, Rheumatrex)

 

Methotrexate is used for managing arthritis, serositis, cutaneous, and constitutional symptoms. It blocks purine synthesis and 5-aminoimidazole-4-carboxamide ribonucleotide (AICAR), thus increasing anti-inflammatory adenosine concentration at sites of inflammation. Methotrexate ameliorates symptoms of inflammation and is particularly useful in arthritis treatment.

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Azathioprine (Imuran, Azasan)

 

Azathioprine is an immunosuppressant and a less toxic alternative to cyclophosphamide. It is used as a steroid-sparing agent in nonrenal disease. It antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. It may decrease proliferation of immune cells, which results in lower autoimmune activity.

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Mycophenolate (CellCept, Myfortic)

 

Mycophenolate is useful for maintenance in lupus nephritis and other serious lupus cases. This agent inhibits inosine monophosphate dehydrogenase (IMPDH) and suppresses de novo purine synthesis by lymphocytes, thereby inhibiting their proliferation. It inhibits antibody production.

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Immune globulin intravenous (Hizentra, Gammagard, Octagam, Privigen)

 

This agent is used for immunosuppression in serious SLE flares. It neutralizes circulating myelin antibodies through anti-idiotypic antibodies. It down-regulates proinflammatory cytokines, including interferon-gamma; blocks Fc receptors on macrophages; suppresses inducer T and B cells; and augments suppressor T cells. It also blocks complement cascade, promotes remyelination, and may increase cerebrospinal fluid IgG (10%).

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Immunomodulators

Class Summary

These agents restore the potential to minimize self-immunity.

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Belimumab (Benlysta)

 

Belimumab inhibits the biological activity of B-lymphocyte stimulator (BLyS); BLyS is a naturally occurring protein required for survival and for development of B-lymphocyte cells into mature plasma B cells that produce antibodies. In autoimmune diseases, elevated BLyS levels are thought to contribute to production of autoantibodies.

This agent is indicated for active, autoantibody-positive SLE in patients in whom standard therapy, including corticosteroids, antimalarials, immunosuppressives, and nonsteroidal anti-inflammatory drugs, is failing.

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Rituximab (Rituxan)

 

B-cell depletion with rituximab has been used successfully for rheumatoid arthritis but has shown mixed results for the treatment of SLE. One open study using rituximab showed excellent results as rescue therapy for patients with active SLE and unresponsive to standard immunosuppressant therapy.[64] However, a placebo-controlled study failed to show an overall significant response.[65]

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Corticosteroids

Class Summary

These agents are used predominately for anti-inflammatory activity and as immunosuppressants. Preparations include oral, intravenous, topical, and intraarticular injections.

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Methylprednisolone (A-Methapred, Medrol, Solu-Medrol, Depo-Medrol)

 

Methylprednisolone is used for acute organ-threatening exacerbations. It decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing increased capillary permeability.

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Prednisone

 

Prednisone is an immunosuppressant for treatment of autoimmune disorders. It may decrease inflammation by reversing increased capillary permeability and suppressing polymorphonuclear neutrophil activity. It stabilizes lysosomal membranes and suppresses lymphocytes and antibody production. Low-dose oral prednisone can be used for milder SLE, but more severe involvement necessitates high doses of oral or intravenous therapy

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Contributor Information and Disclosures
Author

Christie M Bartels, MD, MS  Assistant Professor of Rheumatology, Department of Medicine, University of Wisconsin School of Medicine and Public Health

Christie M Bartels, MD, MS is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine and American College of Rheumatology

Disclosure: Nothing to disclose.

Coauthor(s)

Richard S Krause, MD  Senior Clinical Faculty/Clinical Assistant Professor, Department of Emergency Medicine, University of Buffalo State University of New York School of Medicine and Biomedical Sciences

Richard S Krause, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Emergency Medicine, American College of Emergency Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Viraj S Lakdawala, MD  Clinical Instructor of Emergency Medicine, University of California, San Francisco, School of Medicine; Attending Physician, San Francisco General Hospital

Viraj S Lakdawala, MD is a member of the following medical societies: American Academy of Emergency Medicine and American College of Emergency Physicians

Disclosure: Nothing to disclose.

Mark J Leber, MD, MPH  Assistant Professor of Emergency Medicine in Clinical Medicine, Weill Cornell Medical College; Attending Physician, Lincoln Medical and Mental Health Center

Mark J Leber, MD, MPH is a member of the following medical societies: American College of Emergency Physicians and American College of Physicians

Disclosure: Nothing to disclose.

Daniel Muller, MD, PhD  Associate Professor of Rheumatology, Department of Medicine, University of Wisconsin School of Medicine and Public Health

Daniel Muller, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, American College of Rheumatology, and American Holistic Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Carlos J Lozada, MD  Director of Rheumatology Fellowship Program, Professor, Department of Medicine, Division of Rheumatology and Immunology, University of Miami, Leonard M Miller School of Medicine

Carlos J Lozada, MD is a member of the following medical societies: American College of Physicians and American College of Rheumatology

Disclosure: Pfizer Honoraria Speaking and teaching; Amgen Honoraria Speaking and teaching

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Elliot Goldberg, MD  Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine, Temple University School of Medicine

Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, and American College of Rheumatology

Disclosure: Nothing to disclose.

Gino A Farina, MD, FACEP, FAAEM  Associate Professor of Clinical Emergency Medicine, Albert Einstein College of Medicine; Program Director, Department of Emergency Medicine, Long Island Jewish Medical Center

Gino A Farina, MD, FACEP, FAAEM is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Chief Editor

Herbert S Diamond, MD  Adjunct Professor of Medicine, Division of Rheumatology, University of Pittsburgh School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, and Phi Beta Kappa

Disclosure: Merck Ownership interest Other; Smith Kline Ownership interest Other; Zimmer Ownership interest Other

Additional Contributors

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors Julie Hildebrand, MD, and Anuritha Tirumani, MD, to the development and writing of the source articles.

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In systemic lupus erythematosus (SLE), many genetic-susceptibility factors, environmental triggers, antigen-antibody responses, B-cell and T-cell interactions, and immune clearance processes interact to generate and perpetuate autoimmunity.
The classic malar rash, also known as a butterfly rash, with distribution over the cheeks and nasal bridge. Note that the fixed erythema, sometimes with mild induration as seen here, characteristically spares the nasolabial folds.
Photosensitive systemic lupus erythematosus (SLE) rashes typically occur on the face or extremities, which are sun-exposed regions. Photo courtesy of Dr. Erik Stratman, Marshfield Clinic.
This axial, T2-weighted brain MRI demonstrates an area of ischemia in the right periventricular white matter of a 41-year-old woman with long-standing systemic lupus erythematosus (SLE). She presented with headache and subtle cognitive impairments but no motor deficits. Faintly increased signal intensity was also seen on T1-weighted images, with a trace of enhancement following gadolinium that is too subtle to show on reproduced images. Distribution of the abnormality is consistent with occlusion of deep penetrating branches, such as may result from local vasculopathy, with no clinical or laboratory evidence of lupus anticoagulant or anticardiolipin antibody. Cardiac embolus from covert Libman-Sacks endocarditis remains less likely due to distribution.
Table 1. Drugs Associated With Lupus Erythematosus
Definite Association
ChlorpromazineMethyldopa
HydralazineProcainamide
IsoniazidQuinidine
Possible Association
Beta-blockersMethimazole
CaptoprilNitrofurantoin
CarbamazepinePenicillamine
CimetidinePhenytoin
EthosuximidePropylthiouracil
HydrazinesSulfasalazine
LevodopaSulfonamides
LithiumTrimethadione
Unlikely Association
AllopurinolPenicillin
ChlorthalidonePhenylbutazone
Gold



salts



Reserpine
GriseofulvinStreptomycin
MethysergideTetracyclines
Oral contraceptives
*Data from Tierney et al.[51]
Table 1. American College of Rheumatology Diagnostic Criteria
CriterionDefinition
1. Malar rashFixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds
2. Discoid rashErythematous raised patches with adherent keratotic scaling and follicular plugging (Atrophic scarring may occur in older lesions)
3. PhotosensitivitySkin rash as a result of unusual reaction to sunlight, by patient history or physician observation
4. Oral ulcersOral or nasopharyngeal ulceration, usually painless, observed by a physician
5. ArthritisNonerosive arthritis involving ≥2 peripheral joints, characterized by tenderness, swelling, or effusion
6. Serositis(A) Pleuritis: Convincing history of pleuritic pain or rub heard by a physician or evidence of pleural effusion



or



(B) Pericarditis: Documented by ECG or rub or evidence of pericardial effusion
7. Renal disorder(A) Persistent proteinuria >0.5 g/d or >3+ if quantitation not performed



or



(B) Cellular casts: May be red blood cell, hemoglobin, granular, tubular, or mixed
8. Neurologic disorder(A) Seizures: In the absence of offending drugs or known metabolic derangements (eg, uremia, ketoacidosis, electrolyte imbalance)



or



(B) Psychosis: In the absence of offending drugs or known metabolic derangements (eg, uremia, ketoacidosis, electrolyte imbalance)
9. Hematologic disorder(A) Hemolytic anemia: With reticulocytosis



or



(B) Leukopenia: < 4000/mm3 total on ≥2 occasions



or



(C) Lymphopenia: < 1500/mm3 on ≥2 occasions



or



(D) Thrombocytopenia: < 100,000/mm3 in the absence of offending drugs
10. Immunologic disorder(A) Anti-DNA: Antibody to native DNA in abnormal titer



or



(B) Anti-Sm: Presence of antibody to Sm nuclear antigen



or



(C) Positive finding of antiphospholipid antibodies based on (1) an abnormal serum level of IgG or IgM anticardiolipin antibodies, (2) a positive test result for lupus anticoagulant using a standard method, or (3) a false-positive serologic test for syphilis known to be positive for at least 6 months and confirmed by Treponema pallidum immobilization or fluorescent treponemal antibody absorption tests
11. Antinuclear antibodyAn abnormal titer of antinuclear antibody by immunofluorescence or an equivalent assay at any point in time and in the absence of drugs known to be associated with drug-induced lupus syndrome
SLE can be diagnosed if any 4 or more of the 11 criteria are present, serially or simultaneously, during any interval of observation.
Table 2. International Society of Nephrology 2003 Revised Classification of SLE Nephritis[57]
ClassClassificationFeatures
Class IMinimal mesangialNormal light microscopy findings; abnormal electron microscopy findings
Class IIMesangial proliferativeHypercellular on light microscopy
Class IIIFocal proliferative< 50% of glomeruli involved
Class IVDiffuse proliferative>50% of glomeruli involved; classified segmental or global; treated aggressively
Class VMembranousPredominantly nephrotic disease
Class VIAdvanced sclerosingChronic lesions and sclerosis
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