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Systemic lupus erythematosus

Last updated: January 8, 2021

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Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that predominantly affects women of childbearing age. The exact cause is still unknown, but hormonal and immunological features as well as genetic predisposition are considered likely etiological factors. The presentation of the disease is variable but usually characterized by phases of remission and relapse. Symptoms can range from mild localized symptoms to life-threatening systemic disease. SLE can affect any organ, but typical findings include arthritis, a malar rash (facial butterfly rash), and constitutional symptoms such as fever and fatigue. The diagnosis of SLE is based on clinical findings and is further supported by antibody tests, particularly for ANA, anti-dsDNA, and anti-Sm. Management consists of supportive measures, such as avoiding sun exposure, and medication adapted to disease severity. Long-term pharmacotherapy typically consists of hydroxychloroquine, which has been shown to reduce flares and decrease mortality. For acute flares, glucocorticoids are given as induction therapy, with dose and treatment duration adapted to the severity of the flares. In severe cases, additional immunosuppressants (e.g., mycophenolate, azathioprine) may be given. Cardiovascular, neurological, and renal disease (lupus nephritis), together with infections, are the main causes of death in patients with SLE.

  • Sex: > (10:1) [1]
  • Peak incidence and prevalence [1][2]
    • Age of onset
      • Women: 15–44 years
      • Men: no particular age
    • Race: highest in black populations

Epidemiological data refers to the US, unless otherwise specified.

The exact etiology is unknown, but several predisposing factors have been identified.

The exact pathomechanism of SLE is not fully understood, but the following two processes are the most widely accepted hypotheses: [5]

SLE is a systemic disease characterized by phases of remission and relapse. Some individuals only experience mild symptoms, while others experience severe symptoms and rapid disease progression. SLE can affect any organ.

Common [6]

Both rheumatoid arthritis and SLE arthritis affect the MCP and PIP joints, but SLE does not usually lead to deformities.

Less common [3]

SLE can cause LSE (Libman-Sacks Endocarditis).

Cutaneous lupus erythematosus (CLE)

Discoid lupus erythematosus (DLE) [7][9]

Subacute cutaneous lupus erythematosus (SCLE) [3][9]

Acute cutaneous lupus erythematosus (ACLE) [6][9][10]

Drug-induced lupus erythematosus (DILE) [11]

My Two HIPS”: Methyldopa/Minocycline, TNF-α inhibitors, Hydralazine, Isoniazid, Procainamide/Phenytoin, and Sulfa drugs are triggers for DILE.

DILE may manifest with a variety of the features also seen in idiopathic SLE, e.g., fever, arthritis, malar rash, and serositis, but typically does not affect the CNS or kidneys in the way that SLE does.

Approach [6][10]

  1. If symptoms suggest SLE, request an ANA test.
  2. ANA test: If titer is ≥ 1:80, evaluate the patient according to the EULAR/ACR classification criteria.
  3. Diagnosis involves consideration of the EULAR/ACR criteria, the clinical presentation, and any additional special investigation results.

EULAR/ACR 2019 classification criteria for SLE [6][10]

The EULAR/ACR classification criteria were designed for research studies. While they can raise suspicion for SLE, the diagnosis of SLE cannot be confirmed or ruled out based solely on the criteria. The entire clinical picture and special investigation findings need to be considered.

EULAR/ACR 2019 classification criteria for SLE

Entry criterion


  • Constitutional
  • Hematologic
  • Neuropsychiatric
  • Mucocutaneous
  • Serosal
  • Musculoskeletal
  • Joint involvement (one of the following):
    • Synovitis involving ≥ 2 joints characterized by swelling or effusion
    • Tenderness in ≥ 2 joints and at least 30 minutes of morning stiffness
  • Renal


  • ↓ C3 or ↓ C4
  • ↓ C3 and ↓ C4
  • Anti-dsDNA antibody
    • Autoantibody against double-stranded DNA
    • Positive in 60–70% of patients and highly specific for SLE [12]
    • Levels correlate with disease activity.
    • Associated with lupus nephritis
  • Anti-Sm antibody
  • Do not count a criterion that can be attributed to a diagnosis more likely than SLE.
  • Criteria do not need to occur simultaneously.
  • The occurrence of any criterion at least once is sufficient.
  • SLE classification requires all of the following:
    • Entry criterion
    • At least one clinical criterion
    • Threshold total score according to the EULAR/ACR classification for SLE weighting [6]

Antinuclear antibody (ANA) testing has the highest sensitivity but low specificity for SLE. Anti-dsDNA and anti-Smith antibody testing are the most specific for SLE.

Laboratory tests [12]

Further diagnostics [12]

Goals of treatment [14]

  • Remission of symptoms
  • Prevention of disease flares and organ damage
  • Minimizing drug side effects

General management [12][14]

Systemic therapy [14]

Systemic therapy of SLE
Mild symptoms, no vital organs affected Severe symptoms, no vital organs affected Organ damage
Basic therapy
Induction therapy

UV phototherapy and UV photochemotherapy (PUVA) are contraindicated!

Lupus nephritis

It is important to regularly screen the urine of patients with SLE, as early treatment can prevent progression to severe renal disease.

Neuropsychiatric systemic lupus erythematosus (NPSLE)

Other comorbidities [3][12]

We list the most important complications. The selection is not exhaustive.

Redness In Cheeks” (malar rash): Renal disease, Infections, and Cardiovascular complications are the three most common causes of death in SLE.

Damage to the kidneys or nervous system is associated with a poor prognosis.

SLE and pregnancy

Neonatal lupus syndrome [22][23]

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