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Systemic Lupus Erythematosus Disease Summary

Last updated: 15 October 2026

Overview

Systemic lupus erythematosus (SLE) is a chronic, multisystem, inflammatory, autoimmune disorder. It is further described in the Introduction section.

Systemic lupus erythematosus frequently occurs in Asians, Africans and Hispanics. Information about the regional and worldwide incidence and prevalence of this disease can be found in the Epidemiology section.

The Pathophysiology section describes the interplay between genetic and environmental factors that alter the patient’s immunity that can cause the development of systemic lupus erythematosus.

Categories of disease activity in systemic lupus erythematosus are described in detail in the Classification section.

History and Physical Examination

Most patients with systemic lupus erythematosus present with arthralgia of the hand. But systemic signs and symptoms vary, and these are enumerated in the Clinical Presentation section.

Diagnosis

The European League Against Rheumatism (EULAR) or American College of Rheumatology (ACR) provides the criteria for the diagnosis of systemic lupus erythematosus, and these are enumerated and discussed in the Diagnosis or Diagnostic Criteria section. Disease activity indices that can be used in assessing disease activity of systemic lupus erythematosus are also in this section.

Complete blood count (CBC) and antinuclear antibodies (ANAs) may be performed to help with the diagnosis of systemic lupus erythematosus. The Laboratory Tests and Ancillaries section states the other tests that may be performed for the confirmation of the diagnosis of systemic lupus erythematosus and some of its specific manifestations like lupus nephritis and neuropsychiatric systemic lupus erythematosus (NPSLE).

The Differential Diagnosis section has enumerated conditions that can mimic systemic lupus erythematosus, and they should be identified and ruled out.

Management

The Evaluation section mentions the importance of multidisciplinary team approach in managing patients with systemic lupus erythematosus thus proper specialist referral should be done. This section also discusses the comorbidities of systemic lupus erythematosus.

The Principles of Therapy section enumerates the goals of therapy and the individualized approach in managing patients with systemic lupus erythematosus.

Therapeutic agents for systemic lupus erythematosus such as anti-inflammatory agents, disease modifying anti-rheumatic drugs (DMARDs), immunosuppressants, biologic agents, intravenous immunoglobulin and nonsteroidal anti-inflammatory drugs (NSAIDs) are enumerated and discussed in detail in the Pharmacological Therapy section. Drug options for specific manifestations of systemic lupus erythematosus and comorbidities are also in this section.

Patient education is discussed in detail in the Nonpharmacological section. In this section also there is a discussion on plasma exchange or plasmapheresis.

Lifetime Monitoring is essential in patients with systemic lupus erythematosus and this section mentions that the frequency of visits depends on the disease activity, severity and extent, response to treatment, type of treatment, and monitoring of toxicity. Parameters to assess during these visits are also enumerated in this section.

Patients in long-term treatment with systemic lupus erythematosus drugs may have Complications and these are enumerated and discussed in this section. 

Frequently Asked Questions

What causes organ damage in systemic lupus erythematosus?
Systemic lupus erythematosus is a chronic, multisystem autoimmune disease involving genetic and environmental factors. Loss of immune tolerance leads to autoantibody production, immune-complex formation, complement activation, and cytokine release. These immune responses cause inflammation and tissue damage that may affect individual organs or multiple body systems. Read more
What clinical manifestations suggest systemic lupus erythematosus?
Clinical manifestations vary among patients and may change with disease activity. Common findings include hand arthralgia, arthritis, fatigue, fever, weight loss, photosensitivity, butterfly rash, alopecia, and oral ulcers. Other manifestations include anemia, leukopenia, thrombocytopenia, proteinuria, hematuria, serositis, and neuropsychiatric symptoms. Read more
Which laboratory tests support systemic lupus erythematosus diagnosis?
Laboratory evaluation includes complete blood count, urinalysis, renal and liver function tests, erythrocyte sedimentation rate, C-reactive protein, antinuclear antibodies (ANA), anti-double-stranded DNA antibodies, complement C3 and C4 levels, and antiphospholipid antibodies. ANA positivity supports further evaluation but must be interpreted alongside clinical findings because a positive result alone does not establish the diagnosis. Read more
Why is hydroxychloroquine recommended for lupus patients?
Hydroxychloroquine is recommended for patients with systemic lupus erythematosus unless contraindicated or not tolerated. It has immunomodulatory and anti-inflammatory effects and is used to manage skin and joint manifestations, prevent disease flares, and maintain remission. It is also recommended as background treatment for patients with class III or IV lupus nephritis. Read more
When should immunosuppressants be added to SLE treatment?
Immunosuppressants may be added when patients are intolerant of or inadequately responsive to hydroxychloroquine, with or without corticosteroids, or when corticosteroid doses cannot be reduced to an acceptable maintenance level. They may also be initiated for severe organ-threatening or life-threatening manifestations. Treatment selection depends on the nature and severity of organ involvement, with agents such as azathioprine, methotrexate, mycophenolate mofetil, or cyclophosphamide considered according to clinical indications. Read more