Systemic lupus erythematosus (SLE) is a multisystemic chronic inflammatory disease that affects patients in many different ways over a varying course of time. The disease is typically characterized by periods of remission and relapse, although the causes of exacerbation remain uncertain. SLE, like most autoimmune diseases, has a clear predilection for women. Indeed, women are affected 7 times more frequently than men. The disorder may be diagnosed between the ages of 15 and 50 years, although it is most often detected in women in their twenties. Therefore, SLE is the most commonly encountered autoimmune disease in pregnancy. Although no specific gene mutation for SLE has been identified, the disease likely has a genetic component.1 Approximately 10% of affected patients have a relative with SLE and monozygotic twin studies demonstrate that 50% of affected twins are concordant for the disease. It is estimated that about 2% of children born to mothers with SLE will develop the disease themselves.2 The symptoms of SLE are extremely heterogeneous which can make the diagnosis difficult. The disease may affect joints, skin, kidneys, lung, nervous system, and other organs. The most common presenting complaints are extreme fatigue, arthralgias, fever, and rash (Table 27-1).
TABLE 27-1.Frequency of Clinical Symptoms in Patients With SLE |Favorite Table|Download (.pdf) TABLE 27-1. Frequency of Clinical Symptoms in Patients With SLE
|Clinical symptoms ||Frequency (%) |
|Fatigue ||80-100 |
|Fever ||80-100 |
|Arthritis ||95 |
|Myalgia ||70 |
|Weight loss ||60 |
|Photosensitivity ||60 |
|Malar rash ||50 |
|Nephritis ||50 |
|Pleurisy ||50 |
|Lymphadenopathy ||50 |
|Pericarditis ||30 |
|Neuropsychiatric ||20 |
In 1982, the American Rheumatism Association (ARA) revised previously set criteria for the diagnosis of SLE3 (Table 27-2). According to the ARA, a person must have had at least 4 of the 11 specific criteria in order to carry the diagnosis of SLE. However, many patients have fewer than 4 clinical or laboratory features of SLE and do not meet strict diagnostic criteria. These patients should not be considered to have SLE, but are often referred to as having lupus-like disease. Such individuals may benefit from therapies for SLE and some will ultimately develop the clinical syndrome.
TABLE 27-2.American Rheumatic Association Criteria for the Diagnosis and Classification of SLE |Favorite Table|Download (.pdf) TABLE 27-2. American Rheumatic Association Criteria for the Diagnosis and Classification of SLE
Nephritis (proteinuria ≥500 mg/d or cellular casts)
Neurologic disorder (seizures, psychosis, stroke)
Hematologic disorder (hemolytic anemia, thrombocytopenia, leukopenia, lymphopenia)
Immunologic disorder (anti-dsDNA, anti-Sm, positive LE, false-positive RPR)
The Effect of Pregnancy on SLE
Typically, patients with SLE do not have impaired ...