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Medical Author:

William C. Shiel Jr., MD, FACP, FACR

William C. Shiel Jr., MD, FACP, FACR

Dr. Shiel received a Bachelor of Science degree with honors from the University of Notre Dame. There he was involved in research in radiation biology and received the Huisking Scholarship. After graduating from St. Louis University School of Medicine, he completed his Internal Medicine residency and Rheumatology fellowship at the University of California, Irvine. He is board-certified in Internal Medicine and Rheumatology.

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Medical Editor:

Melissa Conrad Stöppler, MD

Melissa Conrad Stöppler, MD

Melissa Conrad Stöppler, MD, is a U.S. board-certified Anatomic Pathologist with subspecialty training in the fields of Experimental and Molecular Pathology. Dr. Stöppler's educational background includes a BA with Highest Distinction from the University of Virginia and an MD from the University of North Carolina. She completed residency training in Anatomic Pathology at Georgetown University followed by subspecialty fellowship training in molecular diagnostics and experimental pathology.

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Medical Editor:

Catherine Burt Driver, MD

Catherine Burt Driver, MD

Catherine Burt Driver, MD, is board certified in internal medicine and rheumatology by the American Board of Internal Medicine. Dr. Driver is a member of the American College of Rheumatology. She currently is in active practice in the field of rheumatology in Mission Viejo, Calif., where she is a partner in Mission Internal Medical Group.

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In this Article

• Systemic lupus erythematosus facts
• What is systemic lupus erythematosus? What are the types of lupus?
• What causes systemic lupus erythematosus? Is lupus hereditary?
• What is drug-induced lupus?
• What are lupus symptoms and signs?
• How is systemic lupus erythematosus diagnosed?
• What is the treatment for systemic lupus?
• How can a lupus patient help prevent disease activity (flares)?
• How can systemic lupus erythematosus affect pregnancy or the newborn?
• What does the future hold for people with systemic lupus?
• Where can one get more information about systemic lupus erythematosus?
• Systemic Lupus Erythematosus FAQs
• Patient Comments: Lupus - Treatment
• Patient Comments: Lupus - Symptoms
• Patient Comments: Lupus - Experience
• Find a local Rheumatologist in your town
• Systemic Lupus Index

Since individuals with SLE can have a wide variety of symptoms and different combinations of organ involvement, no single test establishes the diagnosis of systemic lupus. To help doctors improve the accuracy of the diagnosis of SLE, 11 criteria were established by the American Rheumatism Association. These 11 criteria are closely related to the symptoms discussed above. Some people suspected of having SLE may never develop enough criteria for a definite diagnosis. Other people accumulate enough criteria only after months or years of observation. When a person has four or more of these criteria, the diagnosis of SLE is strongly suggested. Nevertheless, the diagnosis of SLE may be made in some settings in people with only a few of these classical criteria, and treatment may sometimes be instituted at this stage. Of these people with minimal criteria, some may later develop other criteria, but many never do.

The following are 11 criteria used for diagnosing systemic lupus erythematosus:

• Malar (over the cheeks of the face) "butterfly" rash
• Discoid skin rash (patchy redness with hyperpigmentation and hypopigmentation that can cause scarring)
• Photosensitivity (skin rash in reaction to sunlight [ultraviolet light] exposure)
• Mucous membrane ulcers (spontaneous sores or ulcers of the lining of the mouth, nose, or throat)
• Arthritis (two or more swollen, tender joints of the extremities)
• Pleuritis or pericarditis (inflammation of the lining tissue around the heart or lungs, usually associated with chest pain upon breathing or changes of body position)
• Kidney abnormalities (abnormal amounts of urine protein or clumps of cellular elements called casts detectable with a urinalysis)
  Note: Ultimately, in patients with kidney disease from systemic lupus erythematosus (lupus nephritis), a kidney biopsy may be necessary to both define the cause of the kidney disease as being lupus-related as well as to determine the stage of the kidney disease in order to optimally guide treatments. Kidney biopsies are often performed by fine needle aspiration of the kidney under radiology guidance, but in certain circumstances, a kidney biopsy can be done during an open abdominal operation.
• Brain irritation (manifested by seizures [convulsions] and/or psychosis, referred to as "lupus cerebritis")
• Blood-count abnormalities: low white blood count (WBC) or red blood count (RBC), or platelet count on routine complete blood count testing)
• Immunologic disorder (abnormal immune tests include anti-DNA or anti-Sm [Smith] antibodies, falsely positive blood test for syphilis, anticardiolipin antibodies, lupus anticoagulant, or positive LE prep test)
• Antinuclear antibody (positive ANA antibody testing [antinuclear antibodies in the blood])

In addition to the 11 criteria, other tests can be helpful in evaluating people with SLE to determine the severity of organ involvement. These include routine testing of the blood to detect inflammation (for example, tests called the sedimentation rate and C-reactive protein), blood-chemistry testing, direct analysis of internal body fluids, and tissue biopsies. Abnormalities in body fluids (joint or cerebrospinal fluid) and tissue samples (kidney, skin, and nerve biopsies) can further support the diagnosis of SLE. The appropriate testing procedures are selected for the patient individually by the doctor.