Systemic lupus erythematosus

Last Updated: 2023-10-12

Author(s): Anzengruber F., Navarini A.

ICD11: 4A40.0Z

SLE, integumentary lupus erythematosus, visceral lupus erythematosus, visceral lupus erythematosus.

Potentially lethal, chronic inflammatory, relapsing autoimmune disease of the vascular connective tissue with cutaneous and visceral involvement. Typical is the occurrence of autoantibodies against double-stranded DNA.

  • Prevalence: 40/100,000.
  • Incidence: 5-10/100,000.
  • 4 times more common in Africans than in Caucasians or Asians.
  • Familial cluster: in about 10%.
  • Severity: Hispanic-South American and African American ancestry often have more pronounced courses.
  • Females: Males= 8-10:1.
  • Patients are rarely younger than 5. years.
  • First manifestation usually occurs in young adulthood (15th and 30th years), but "late onset SLE" is possible.

  • Multifactorial aetiology and trigger factors.
  • UV radiation.
  • Genetics (e.g.: Mutation in the TREX1 gene, association with HLA-DR3 (in Caucasians), HLA-DR1 and -DR2 (in Asians)).
  • Drugs (see drug-induced lupus erythematosus).
  • Estrogens (e.g.: exacerbation in pregnancy).
  • Viral infections.
  • CD4+ T lymphocytes secrete IL-4 & 6, leading to B-cell activation and eventually autoantibody production and, by extension, immune complex production. Circulating immune complexes can cause deposition in small vessels of visceral organs or the skin, where they can act as triggers for vasculitis. In addition, formation of immune complexes in the basement membrane is possible.

  • General symptoms: ↑ temperature, fatigue, weight loss.
  • Skin (in about 80% of patients)
  • Face: Erythema perstans (scaly, blurred butterfly erythema), in some cases the entire face may be affected. Approx. 60% of all patients with SLE show butterfly erythema.
  • Trunk: multiforme or livedo-like often haemorrahgic, rarely bullous exanthema of the upper trunk. 20-30% of patients show SCLE or DLE-like skin lesions. Up to 20% of patients remain skin symptom-free.
  • Palmoplantar: patchy, diffuse, sometimes keratotic erythema.
  • Livedo racemosa.
  • Teleangiectasia on fingertips and subungual haemorrhages at the nail fold.
  • Vasculitic changes can lead to pain and also to gangrene and ulceration.
  • Mouth mucosa (in approx. 40% of patients): Enanthema, erosions, ulcerations.
  • Lips: Cheilitis
  • Myalgias (almost all patients).
  • Arthritis (almost all patients) mostly without visible swelling. In 10% there is also polyarthritis.
  • Serositis (approx. 50% of patients): In the sense of pleuritis, pericarditis (Libman-Sacks syndrome) or peritonitis.
  • Lupus nephritis or nephrotic syndrome (in approx. 40-65% of patients).
  • Lymph node swelling.
  • Hepatosplenomegaly.
  • Gastritis.
  • Colitis.
  • Psychosis.
  • Seizures.
  • Scarring alopecia.
  • Raynaud's phenomenon.
  • Associations:
    • Sweet syndrome.
    • Livedovasculopathy.
  • Autoantibodies (except DNA- antibodies) do not correlate with disease activity.
  • Autoantibodies against double-stranded DNA and immune complexes with complement activation, which can trigger vasculitis of various organs.

  1. Clinical.
  2. ARA criteria (not an absolute standard).
  3. Serological parameters.
  4. Dermatopathology and direct immunofluorescence.
  5. Light testing.

  1. Clinical
    • s.
  2. ARA criteria (not an absolute standard).
    • Classification criteria of the American Colleague of Rheumatology:
      1. Butterfly erythema
      2. Cutaneous LE (SCLE, DLE, scarring alopecia)
      3. Photosensitivity
      4. Oral, nasopharyngeal, painless ulcerations
      5. Non-erosive, painful arthritis (≥ joints)
      6. Serositis
        • Pleuritis
        • Pericarditis
        • Sometimes peritonitis described.
      7. Neurological abnormalities: non-drug-induced seizures, psychoses
      8. Kidney involvement
        • Proteinuria > 0.5g/24 h or pathological sediment)
      9. Hematological abnormalities
        • Hemolytic anaemia with reticulocytosis.
        • Leucopenia <4'000/mm3.
        • Lymphopenia <1'500 mm3.
        • Thrombocytopenia <100'000/mm3.
      10. Imunological findings (Ak gg. dsDNA, anti-Sm- Ak, antiphospholipid (=anticardiolipin)-Ak, lupus anticoagulant.
      11. ↑ ANAs
        1. If 4 (of 11) criteria apply, SLE can be diagnosed (sensitivity: 96%, specificity: 96%)
  3. Serological parameters.
    • Blood count: leukopenia, neutropenia, lymphopenia, eosinopenia, thrombocytopenia, macrocytic anaemia possible.
    • Inflammatory values: CRP and ESR (↑ ESR often correlates with severity of disease)
    • Circulating immune complexes: C3↓, C4↓
    • Electrophoresis: ↑ γ-globulin.
    • Cryoglobulins.
    • Antibody profile!


Antigen Frequency
Native DNA 50-90%
U1-RNP (nuclear) 30-60%
rRNP (ribosomal) 10%
Ro(SSA) 40-60%
La(SSB) 20-.30%
Ku 10%
Histone 70%

Source: Plewig, Gerd. Braun-Falco's Dermatology, Venereology And Allergology. Berlin: Springer, 2012. print.


  • False-positive Rh-fact. (in approx. 33%)
  • False-positive syphilisserology (VDRL) (in approx. 25%).
  1. Dermatopathology and direct immunofluorescence.
    • Dermatopathology
      • Interface dermatitis, perivascular lymphocytic, oedematous infiltrate.
      • Direct immunofluorescence
        • Lupus band test: banded granular, IgG, C3, (but also IgM and IgA)- deposits at the basement membrane (also in non-lesional, unexposed skin).

  1. Light testing
    • Light staircase, then photoprovocation.
    • Screening:
    • Blood pressure and temperature measurement.
    • Urine status:
      • 24h- collected urine (creatinine clearance, immunophoresis).
    • Thoracic X-ray.
    • Pulmonary function (CO diffusion capacity e.g. of interstitial fibrosis, pneumonitis).
    • X-ray resp. Ultrasound of painful joints.
    • Overabdominal sonography.
    • Kidney biopsy: lupus nephritis.
    • Echocardiography: carditis.
    • Capillary microscopy.
    • Lumbar puncture.
    • EEG.
    • MRI with gadolinium contrast, angio-MRI, angiography: in case of v.a. cerebral vasculitis.
    • Ophthalmological presentation: conjunctivitis, sicca symptoms.

  • Rapid, chronic course.
  • Depending on organ involvement, the disease can lead to death

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