SLE is a complex disorder with variable presentations, course, and prognosis. Since its prevalence is only 1/1,000, most primary care physicians and general internists will not have sufficient experience in the management of moderate-to-severe life-threatening disease. The major tasks of the primary care physician in the diagnosis and management of patients with SLE include early diagnosis, appropriate referral, monitoring patients with mild, stable disease, and collaboration with a specialist in the management of severe disease. Guidelines for the initial evaluation, reasons for referral, and management of mild and severe SLE are provided.