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SLE classically presents in a young woman with fatigue and arthritis, commonly of the hands. There are often suspicious findings in the history such as an episode of pleuritis or undiagnosed anemia.


  1. SLE is a systemic autoimmune disease primarily affecting women of childbearing age.

  2. Various groups are more prone to disease.

    1. Female:male ratio is about 9:1.

    2. About 5% of patients report a first-degree relative with the disease.

    3. Women of color are most commonly affected.

  3. Almost every organ can be involved, although the joints, skin, serosa, and kidneys are most commonly affected.

  4. The pathogenesis of the disease is related to the formation of autoantibodies to a number of nuclear antigens. The ANA is the most common.

  5. The most common features of SLE, both at presentation and later in follow-up, are listed in Table 27-7.

Table 27-7.Clinical manifestations of SLE at onset and during disease.


  1. The diagnosis of SLE, especially in people with mild disease, can be difficult.

  2. The ACR has developed criteria to standardize the diagnosis for research purposes.

    1. The criteria are:

      1. Malar rash

      2. Discoid rash

      3. Photosensitivity

      4. Oral ulcers

      5. Arthritis (nonerosive arthritis)

      6. Serositis (pleuritis or pericarditis)

      7. Kidney disorder (proteinuria or cellular casts)

      8. Neurologic disorder (headache, seizures, or psychosis without other cause)

      9. Hematologic disorder (hemolytic anemia or any cytopenia)

      10. Immunologic disorder (anti-ds-DNA, anti-SM, or antiphospholipid antibodies)

      11. Positive ANA

    2. The diagnosis of SLE requires the presence of 4 or more of these criteria.

    3. Although the same reservations about using diagnostic criteria clinically that were discussed above in the section on RA apply here, the SLE criteria are frequently used.

  3. Newer diagnostic criteria have also been developed by the Systemic Lupus International Collaborating Clinics (SLICC) group.

    1. These criteria, referenced at the end of the chapter, give somewhat greater primacy to serologic and pathologic evidence of SLE.

    2. A patient may fulfill the criteria with biopsy-proven SLE nephritis and the presence of ANA or anti-ds-DNA antibodies.

  4. The test characteristics for the diagnostic criteria are given in Table 27-8. Also included in this table are the test characteristics for the various individual criteria.

  5. Satisfaction of the ACR criteria or the presence of the anti-Sm antibody is highly supportive of the diagnosis of SLE. Failure to fulfill the SLICC criteria or the absence of ANA argue strongly against SLE.

  6. Autoantibodies

    1. Measuring autoantibodies in SLE provides important diagnostic information.

    2. ANA ...

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