Systemic Lupus (cont.)

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How is systemic lupus erythematosus diagnosed?

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 standard 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; leukopenia, anemia, and thrombocytopenia, respectively. Each of these are detectable with standard complete blood count testing (CBC).
  • 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, the erythrocyte sedimentation rate, or ESR, and the C-reactive protein, or CRP), blood-chemistry testing, direct analysis of internal body fluids, and tissue biopsies. Abnormalities in body fluids (joint or cerebrospinal fluid) and tissue samples (kidney biopsy, skin biopsy, and nerve biopsy) can further support the diagnosis of SLE. The appropriate testing procedures are selected for the patient individually by the doctor.

Medically Reviewed by a Doctor on 10/9/2015

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