Systemic lupus erythematosus

Last Updated: 2021-10-15

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

ICD11: 4A40.0Z

SLE, lupus erythematosus integumentalis, lupus erythematosus visceralis, visceral lupus erythematosus

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

  • Prevalence: 40/100,000.
  • Incidence: 5-10/100,000.
  • Four times more common among Africans than Caucasians or Asians.
  • Family accumulation: in approx. 10%.
  • Severity: In the case of Spanish-South American and African-American descent, more pronounced progressions can often be observed.
  • Women: Men = 8-10: 1.
  • Patients are rarely younger than 5 years.
  • Initial manifestation usually occurs in young adults (15th and 30th LJ), but a "late onset SLE" is possible.

  • Multifactorial etiology and trigger factors.
  • UV rays.
  • Genetics (e.g.: Mutation in the TREX1 gene, association with HLA-DR3 (in Caucasians), HLA-DR1 and -DR2 (in Asians).
  • Medicines (see drug-induced lupus erythematosus).
  • Estrogens (e.g. exacerbation during pregnancy).
  • Viral infections.
  • CD4+ T lymphocytes secrete IL-4 & 6, which leads to B-cell activation and ultimately to autoantibody production and, in the broader sense, to the production of immune complexes. 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, the formation of immune complexes in the basement membrane is possible.

  • Allgemeinsymptome: Erhöhte Temperatur, Müdigkeit, Gewichtsabnahme. 
  •  Haut (bei ca. 80% der Patienten) 
  •  Gesicht: Erythema perstans (schuppiges, unscharf begrenztes Schmetterlingserythem), in manchen Fällen kann das gesamte Gesicht erfasst sein. Ca. 60% aller Patienten mit SLE zeigen ein Schmetterlingserythem. 
  •  Stamm: multiforme oder livedoartige oft hämorrhagische, selten bullöse Exantheme des oberen Rumpfes. 20-30% der Patienten zeigen SCLE oder DLE-ähnliche Hautveränderungen. Bis zu 20% der Patienten bleiben hauterscheinungsfrei. 
  •  Palmoplantar: fleckige, diffuse, teils keratotische Erytheme. 
  •  Livedo racemosa. 
  •  Teleangiektasien an Fingerspitzen und subunguale Blutungen am Nagelfalz. 
  •  Vaskulitische Veränderungen können zu Schmerzen und auch zu Gangränen und Ulzerationen führen. 
  •  Mundschleimhaut (in ca. 40% der Patienten): Enantheme, Erosionen, Ulzerationen. 
  •  Lippen: Cheilitis  
  •  Myalgien (fast alle Patienten). 
  •  Arthritis (fast alle Patienten) meist ohne sichtbare Schwellung. Bei 10% besteht zusätzlich eine Polyarthritis. 
  •  Serositis (ca. 50% der Patienten): Im Sinne einer Pleuritis, Perikarditis (Libman-Sacks-Syndrom) oder Peritonitis. 
  •  Lupusnephritis bzw. nephrotisches Syndrom (bei ca. 40–65% der Patienten).  
  •  Lymphknotenschwellungen. 
  •  Hepatosplenomegalie. 
  •  Gastritis. 
  •  Kolitis. 
  •  Psychosen. 
  •  Krampfanfälle. 
  •  vernarbende Alopezie. 
  •  Raynaud-Phänomen. 
  •  Assozationen:   
  •  Sweet Syndrom. 
  •  Livedovaskulopathie. 
  •  Die Autoantikörper (außer DNA-Antikörper) korrelieren nicht mit der Krankheitsaktivität. 
  •  Autoantikörper gegen doppelsträngige DNA und Immunkomplexe mit Komplementaktivierung, welche eine Vaskulitis verschiedenster Organe auslösen kann. 

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


  1. Clinic 
    • s.o.
  2. ARA criteria (not absolute scale). 
    • 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 
        • Pleurisy 
        • Pericarditis 
        • Sometimes also described peritonitis.
      7. Neurological abnormalities: non-drug seizures, psychoses 
      8. Kidney involvement 
        • Proteinuria > 0.5g/24 h or pathological sediment)
      9. Hematological abnormalities 
        • Hemolytic anemia with reticulocytosis.
        • Leucopenia <4,000/mm3.
        • Lymphopenia <1,500 mm3.
        • Thrombocytopenia <100,000/mm3.
      10. Imunological findings (AK vs. dsDNA, anti-Sm- Ak, antiphospholipid (= anticardiolipin)-Ak, lupus anticoagulant. 
      11. Increased ANAs 
        1. With 4 (of 11) applicable criteria an SLE can be diagnosed (sensitivity: 96%, specificity: 96%)
  3. Serological parameters. 
  • Blood count: leukopenia, neutropenia, lymphopenia, eosinopenia, Thrombopenia, macrocytic anemia possible.
  • Inflammation values: CRP and BSG (increased BSG often correlates with severity of the disease)
  • Circulating immune complexes: C3 reduced, C4 reduced 
  • Electrophoresis: Increased γ globulin.
  • Cryoglobulins.
  • Antibody profile!


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

Source: Plewig, Gerd.  Braun-Falco's Dermatology, Venerology 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 edematous infiltrate.
      • Direct immunofluorescence 
        • Lupus band test: band-shaped granular, IgG, C3, (but also IgM and IgA) - deposits on the basement membrane (also in non lesional, unexposed skin).


  1. Light testing 
  • Light stairs, then photo provocation.
  • Screening:
  • Blood pressure and temperature measurement.
  • Urine status: 
    • 24-hour collecting urine (creatinine clearance, immunophoresis).
  • Thoracic x-ray.
  • Lung function (CO diffusion capacity e.g. of interstitial fibrosis, pneumonitis).
  • X-ray or ultrasound of aching joints.
  • Sonography of the upper abdomen.
  • Kidney biopsy: lupus nephritis.
  • Echocardiography: Carditis.
  • Capillary microscopy.
  • Spinal tap.
  • EEG.
  • MRI with gadolinium contrast, angio-MRI, angiography: in v.a. cerebral vasculitis.
  • Ophthalmological presentation: conjunctivitis, sicca symptoms.

  • Chronic progression. 
  • Depending on the organ involvement, the disease can lead to death.

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