eMedicine Specialties > Physical Medicine and Rehabilitation > Arthritis & Connective Tissue Disorders

Systemic Lupus Erythematosus

Author: Bertram Greenspun, DO, Director of Spinal Dysfunction Clinic, Director of Amputee Clinic, Clinical Associate Professor, Department of Physical Medicine and Rehabilitation, Christiana Care Health System, Jefferson Medical College
Contributor Information and Disclosures

Updated: Apr 23, 2009

Introduction

Background

Systemic lupus erythematosus (SLE) is an inflammatory connective tissue disease with variable manifestations.1,2,3 SLE may affect many organ systems with immune complexes and a large array of autoantibodies, particularly antinuclear antibodies (ANAs). (See image below and Image 1.)

The classic malar rash, also known as a butterfly...

The classic malar rash, also known as a butterfly rash, of systemic lupus erythematosus, 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.

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The classic malar rash, also known as a butterfly...

The classic malar rash, also known as a butterfly rash, of systemic lupus erythematosus, 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.


Pathophysiology

The damage that results from systemic lupus erythematosus is caused by the excessive deposition of immune complexes and the resultant organ-specific inflammatory response found in the blood vessels, kidneys, connective tissue,4 and skin.  It has not been explained why there is a female preponderance.

Frequency

United States

The incidence of systemic lupus erythematosus (SLE) in the United States is 50-100 cases per 100,000 population. A report indicates that 239,000 people in the United States have suspected or definite SLE.

International

Prevalence of systemic lupus erythematosus is about the same worldwide (50-100 cases per 100,000 population); the condition appears to be common in China and Southeast Asia, as well as in the black population of the Caribbean.5

Mortality/Morbidity

  • Since the mid-20th century, the 10-year survival rate for systemic lupus erythematosus (SLE) has improved from 10% to 70-90% for individuals with SLE.6
  • Female patients with onset of the disease after age 60 years have the most favorable prognosis.
  • Children with SLE have a less favorable prognosis.
  • Renal failure and intercurrent infections are the most common causes of death associated with SLE.
  • Diffuse central nervous system (CNS) disease is another common cause of death.
  • The 10-year survival in Dubois group from 1950 to 1971 was 87%, but it was only 65% in those with renal involvement.1
  • It is not known why there is a female preponderance.

Race

  • The highest incidence of systemic lupus erythematosus (SLE) is in Asians in Hawaii, blacks, and certain groups of Native Americans.
  • African American women are 3 times more likely to be affected by SLE than are white American women.

Sex

  • Systemic lupus erythematosus (SLE) occurs more frequently in women, with a female-to-male ratio of 8:1-13:1 in adults. SLE is 3 times more common in African-American women than in white women in United States.
  • Women are more prone to SLE in their childbearing years.
  • During the first 10 years of life, girls develop SLE 3-7 times more often than do boys.
  • It is not known why there is a female preponderance.
  • SLE poses a risk to pregnant women, although treatment for the disease can harm their fetus.

Age

The onset of systemic lupus erythematosus occurs primarily in patients aged 16-55 years. It is uncommon after the age of 50.

Clinical

History

Potential symptoms of systemic lupus erythematosus (SLE) include the following:

  • Constitutional - These symptoms affect most patients at some point during the disease.
    • Fatigue
    • A general feeling of unwellness
    • Fever
    • Weakness
  • Musculoskeletal - These symptoms most commonly affect patients early in the course of SLE, with 90% of patients having arthralgias and 50% of them experiencing inflammatory arthritis.
    • Arthralgia
    • Myalgia
    • Nonrestorative sleep
  • Cardiovascular - Raynaud phenomenon
  • Pulmonary - Pleuritic chest pain
  • Genitourinary - Menstrual irregularities
  • Neuropsychiatric
    • Headaches
    • Seizures
    • Anxiety disorders
    • Phobias
    • Manias
    • Depression
  • Gastrointestinal (GI)
    • Diffuse abdominal pain
    • Nausea/vomiting
    • Dysphagia
  • Photosensitivity (See image below and Image 2.)
Photosensitive systemic lupus erythematosus rashe...

Photosensitive systemic lupus erythematosus rashes typically occur on the face or extremities, which are sun-exposed regions. Photo courtesy of Dr. Erik Stratman, Marshfield Clinic.

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Photosensitive systemic lupus erythematosus rashe...

Photosensitive systemic lupus erythematosus rashes typically occur on the face or extremities, which are sun-exposed regions. Photo courtesy of Dr. Erik Stratman, Marshfield Clinic.


Physical

Potential physical findings in systemic lupus erythematosus include the following7 :

  • Constitutional
    • Weight loss
    • Weight gain (in patients treated with prednisone)
    • Fever
  • Musculocutaneous
    • Hair loss
    • Discoid lupus
    • Acute cutaneous lupus
    • Butterfly rash (See image below and Image 1.)
      • Malar
      • Erythematosus
      • Elevated
      • Pruritic
      • Painful
The classic malar rash, also known as a butterfly...

The classic malar rash, also known as a butterfly rash, of systemic lupus erythematosus, 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.

[ CLOSE WINDOW ]
The classic malar rash, also known as a butterfly...

The classic malar rash, also known as a butterfly rash, of systemic lupus erythematosus, 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.


    • Livedo reticularis
    • Vesicular or bullous lesions
    • Acute or chronic urticaria
    • Telangiectasis
    • Periungual erythema
    • Palmar erythema or nodules
    • Purpura
    • Panniculitis
    • Ulcerations
    • Dry eyes
    • Dry mouth
  • Musculoskeletal
    • Migratory, transient, and symmetric arthritis, most commonly in the hands and knees
    • Secondary fibromyalgia
  • Cardiovascular
    • Raynaud phenomenon - Occurs in about half of patients, especially in hands and feet; skin color changes occur.
    • Hypertension
  • Pulmonary - Effusions
  • Renal - Edema
  • Neuropsychiatric
    • Chorea
    • Ataxia
    • Transverse myelitis
    • Asymmetric mononeuritis multiplex
    • Cranial nerve neuropathies
  • Ophthalmologic
    • Cotton-wool exudates
    • Conjunctivitis or episcleritis

Causes

  • The cause of systemic lupus erythematosus (SLE) is unknown.
  • SLE is an immunologic disorder that involves the production of autoantibodies.8
  • Sunlight can trigger the onset of SLE.
  • Certain medicines may trigger SLE by initiating the immune response in susceptible individuals. The 2 most common medications that can lead to SLE are the following:
    • Hydralazine
    • Procainamide
  • Others medications that may trigger SLE include the following:
    • Anticonvulsants
    • Antiemetics
    • Antituberculars
    • Antibiotics

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
Further Reading
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References

  1. Wallace DJ, Hahn BH, eds. Dubois' Lupus Erythematosus. 7th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2007.

  2. Mackillop LH, Germain SJ, Nelson-Piercy C. Systemic lupus erythematosus. BMJ. Nov 3 2007;335(7626):933-6. [Medline].

  3. Rahman A, Isenberg DA. Systemic lupus erythematosus. N Engl J Med. Feb 28 2008;358(9):929-39. [Medline].

  4. Perkins K, Hoffman RW, Bezruczko N. A Rasch analysis for classification of systemic lupus erythematosus and mixed connective tissue disease. J Appl Meas. 2008;9(2):136-50. [Medline].

  5. Peschken CA, Katz SJ, Silverman E, et al. The 1000 Canadian faces of lupus: determinants of disease outcome in a large multiethnic cohort. J Rheumatol. Apr 15 2009;[Medline].

  6. Goldblatt F, Isenberg DA. New therapies for systemic lupus erythematosus. Clin Exp Immunol. May 2005;140(2):205-12. [Medline]. [Full Text].

  7. Fernando MM, Isenberg DA. How to monitor SLE in routine clinical practice. Ann Rheum Dis. Apr 2005;64(4):524-7. [Medline]. [Full Text].

  8. Bonelli M, Savitskaya A, von Dalwigk K, et al. Quantitative and qualitative deficiencies of regulatory T cells in patients with systemic lupus erythematosus (SLE). Int Immunol. May 9 2008;[Medline].

  9. Al Arfaj AS, Khalil N, Al Saleh S. Lupus nephritis among 624 cases of systemic lupus erythematosus in Riyadh, Saudi Arabia. Rheumatol Int. Apr 21 2009;[Medline].

  10. Clarke-Jenssen AC, Fredriksen PM, Lilleby V, et al. Effects of supervised aerobic exercise in patients with systemic lupus erythematosus: a pilot study. Arthritis Rheum. Apr 15 2005;53(2):308-12. [Medline]. [Full Text].

  11. Hicks JE, Miller F, Plotz P, et al. Isometric exercise increases strength and does not produce sustained creatinine phosphokinase increases in a patient with polymyositis. J Rheumatol. Aug 1993;20(8):1399-401. [Medline].

  12. [Best Evidence] Fortin PR, Abrahamowicz M, Ferland D, et al. Steroid-sparing effects of methotrexate in systemic lupus erythematosus: a double-blind, randomized, placebo-controlled trial. Arthritis Rheum. Dec 15 2008;59(12):1796-804. [Medline].

  13. Levine JS, Branch DW, Rauch J. The antiphospholipid syndrome. N Engl J Med. Mar 7 2002;346(10):752-63. [Medline].

  14. Cozen L, Wallace DJ. Avascular necrosis in systemic lupus erythematosus: clinical associations and a 47-year perspective. Am J Orthop. May 1998;27(5):352-4. [Medline].

  15. Chambers SA, Isenberg D. Anti-B cell therapy (rituximab) in the treatment of autoimmune diseases. Lupus. 2005;14(3):210-4. [Medline].

  16. Abetimus: abetimus sodium, LJP 394. BioDrugs. 2003;17(3):212-5. [Medline].

  17. Petri M, Barr SG, Zonana-Nach A, et al. Measures of disease activity, damage, and health status: the Hopkins Lupus Cohort experience. J Rheumatol. Feb 1999;26(2):502-3. [Medline].

  18. Petri M. Lupus in Baltimore: evidence-based 'clinical pearls' from the Hopkins Lupus Cohort. Lupus. 2005;14(12):970-3. [Medline].

  19. The American College of Rheumatology response criteria for systemic lupus erythematosus clinical trials: measures of overall disease activity. Arthritis Rheum. Nov 2004;50(11):3418-26. [Medline]. [Full Text].

  20. Sinaki M, ed. Basic Clinical Rehabilitation Medicine. 2nd ed. St Louis, Mo: Mosby; 1993.

  21. Brodsky RA, Petri M, Jones RJ. Hematopoietic stem cell transplantation for systemic lupus erythematosus. Rheum Dis Clin North Am. May 2000;26(2):377-87, viii. [Medline].

  22. D'Cruz DP. Systemic lupus erythematosus. BMJ. Apr 15 2006;332(7546):890-4. [Medline]. [Full Text].

  23. Esdaile JM, Abrahamowicz M, Joseph L, et al. Laboratory tests as predictors of disease exacerbations in systemic lupus erythematosus. Why some tests fail. Arthritis Rheum. Mar 1996;39(3):370-8. [Medline].

  24. Frontera WR, ed. Exercise in Rehabilitation Medicine. 2nd ed. Champaign, Ill: Human Kinetics; 2006:175-6.

  25. Foote RA, Kimbrough SM, Stevens JC. Lupus myositis. Muscle Nerve. Jan 1982;5(1):65-8. [Medline].

  26. Gaffney PM, Moser KL, Graham RR, et al. Recent advances in the genetics of systemic lupus erythematosus. Rheum Dis Clin North Am. Feb 2002;28(1):111-26. [Medline].

  27. Ginzler EM, Moldovan I. Systemic lupus erythematosus trials: successes and issues. Curr Opin Rheumatol. Sep 2004;16(5):499-504. [Medline].

  28. Goodman D, Morrissey S, Graham D, et al. Illness representations of systemic lupus erythematosus. Qual Health Res. May 2005;15(5):606-19. [Medline].

  29. Lehman JF, Brunner GD, Martinis AJ, et al. Ultrasonic effects as demonstrated in live pigs with surgical metallic implants. Arch Phys Med. 1959;40:483.

  30. McElhone K, Abbott J, Teh LS. A review of health related quality of life in systemic lupus erythematosus. Lupus. 2006;15(10):633-43. [Medline].

  31. Omdal R, Brokstad K, Waterloo K, et al. Neuropsychiatric disturbances in SLE are associated with antibodies against NMDA receptors. Eur J Neurol. May 2005;12(5):392-8. [Medline].

  32. Lahita R, ed. Systemic Lupus Erythematosus. 4th ed. San Diego, Calif: Academic Press; 2004.

  33. Traynor AE, Schroeder J, Rosa RM, et al. Treatment of severe systemic lupus erythematosus with high-dose chemotherapy and haemopoietic stem-cell transplantation: a phase I study. Lancet. Aug 26 2000;356(9231):701-7. [Medline].

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Keywords

systemic lupus erythematosus, lupus, SLE lupus symptoms, lupus erythematosus, erythematosus, systemic lupus, lupus disease, rash lupus, malar rash, lupus discoid, lupus skin rash, lupus causes, lupus nephritis, cutaneous lupus, lupus butterfly rash, cutaneous lupus erythematosus, lupus rashes, disseminated lupus erythematosus, antinuclear antibodies, arthralgia, myalgia, Raynaud phenomenon, Raynaud's phenomenon, polymyositis, dermatomyositis, arthritis

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Contributor Information and Disclosures

Author

Bertram Greenspun, DO, Director of Spinal Dysfunction Clinic, Director of Amputee Clinic, Clinical Associate Professor, Department of Physical Medicine and Rehabilitation, Christiana Care Health System, Jefferson Medical College
Bertram Greenspun, DO is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation, American Association of Neuromuscular and Electrodiagnostic Medicine, American Congress of Rehabilitation Medicine, American Medical Association, American Physicians Fellowship for Medicine in Israel, and International Society of Physical and Rehabilitation Medicine
Disclosure: Nothing to disclose.

Medical Editor

Martin K Childers, DO, PhD, Associate Professor, Department of Neurology, Wake Forest University Health Services
Martin K Childers, DO, PhD is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation, American Congress of Rehabilitation Medicine, American Osteopathic Association, Christian Medical & Dental Society, and Federation of American Societies for Experimental Biology
Disclosure: Allergan pharma Consulting fee Consulting

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Patrick M Foye, MD, FAAPMR, FAAEM, Associate Professor of Physical Medicine and Rehabilitation, Co-Director of Musculoskeletal Fellowship, Co-Director of Back Pain Clinic, Director of Coccyx Pain Service (Tailbone Pain Service: www.TailboneDoctor.com), University of Medicine and Dentistry of New Jersey, New Jersey Medical School
Patrick M Foye, MD, FAAPMR, FAAEM is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation, American Association of Neuromuscular and Electrodiagnostic Medicine, Association of Academic Physiatrists, and International Spine Intervention Society
Disclosure: Nothing to disclose.

CME Editor

Kelly L Allen, MD, Regional Medical Director, IMX-Medical Management Services
Disclosure: Nothing to disclose.

Chief Editor

Denise I Campagnolo, MD, MS, Director of Multiple Sclerosis Clinical Research and Staff Physiatrist, Barrow Neurology Clinics, St Joseph's Hospital and Medical Center; Investigator for Barrow Neurology Clinics; Director, NARCOMS Project for Consortium of MS Centers
Denise I Campagnolo, MD, MS is a member of the following medical societies: Alpha Omega Alpha, American Association of Neuromuscular and Electrodiagnostic Medicine, American Paraplegia Society, Association of Academic Physiatrists, and Consortium of Multiple Sclerosis Centers
Disclosure: Teva Neuroscience Honoraria Speaking and teaching; Serono-Pfizer Honoraria Speaking and teaching

 
 
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