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Systemic Lupus Erythematosus (SLE)

The term ‘lupus’ (Latin for ‘wolf ’) was first used during the Middle Ages to describe erosive skin lesions evocative of a ‘wolf ’s bite’. In 1846 the Viennese physician Ferdinand von Hebra (1816–1880) introduced the butterfly metaphor to describe the malar rash. He also used the term ‘lupus erythematosus’ and published the first illustrations in his Atlas of Skin Diseases in 1856.

  • SLE is a complex autoimmune inflammatory disease characterized by the development of a variety of autoantibodies that result in clinical manifestations that can affect multiple organ systems. The multisystem manifestations can potentially result in serious morbidity or even mortality, and the disease presentation and course can be quite variable.
  • Lupus typically affects women of childbearing age and is also more common in certain ethnic minority groups such as African Americans, Asians, and Hispanics.
  • Lupus is a heterogeneous disease with a continuum of disease activity. For example, some patients can have predominant skin and joint involvement, whereas others can present with organ-threatening diseases such as nephritis or diffuse alveolar hemorrhage.

The following criteria are proposed to replace the American College of Rheumatology (ACR) classification criteria for SLE. Any person who satisfies four or more of the following clinical and immunological criteria including at least one clinical criterion and one immunological criterion, OR any person who has biopsy-proven lupus nephritis in the presence of antinuclear antibodies (ANAs) or anti-double-stranded (ds) DNA antibodies is considered to have SLE for the purposes of clinical studies (97% sensitivity and 84% specificity).

Systemic Lupus International Collaborating Clinics Classification Criteria for SLE (2012)

CRITERION

DEFINITION

FREQUENCY

Clinical Criteria

1. ACLE

Malar rash, bullous lupus, TEN variant, maculopapular, photosensitive, subacute cutaneous lupus

60% to 70%

2. CCLE

Classic discoid, hypertrophic, lupus panniculitis/profundus, mucosal, lupus erythematosus tumidus, chilblains lupus, discoid/lichen panus overlap

15% to 30%

3. Alopecia

Nonscarring, diffuse hair thinning or visible broken hairs

30% to 50%

4. Oral ulcers

Oral (palate, buccal, tongue) or nasal ulceration

15% to 45%

5. Synovitis

Arthritis involving two or more peripheral joints, characterized by tenderness, swelling, or effusion and morning stiffness >30 min

90%

6. Serositis

Pleuritis: convincing history of pleuritic pain for >1 day or pleural rub or evidence of pleural effusion, orPericarditis documented by ECG or rub or evidence of pericardial effusion

30% to 60%

10% to 40%

7. Renal disorder

Persistent proteinuria ≥0.5 g/day (24 h urine or urine protein/Cr ratio)

Or

Red blood cell casts

40% to 60%

8. Neurological disorder

Seizures, psychosis, myelitis, mononeuritis multiplex, peripheral or cranial neuropathy, acute confusional state

15% to 20%

9. Hemolytic anemia

Direct Coombs positive

5% to 10%

10. Leukopenia

Leukopenia <4000 mm3 at least once, or

Lymphopenia <1000/mm3 at least once

15% to 20%

15% to 20%

11. Thrombocytopenia

Platelets <100,000/mm3

15% to 20%

Immunological Criteria

1. ANA

Level above laboratory reference range

98%

2. Anti-dsDNA

Level above laboratory reference range (or >2-fold ELISA reference range)

60% to 70%

3. Anti-Sm

Presence of antibody to Sm nuclear antigen

20% to 30%

4. Antiphospholipid antibody positivity

30% to 50%

5. Low complement

Low C3, low C4, or low CH50

55% to 60%

6. Direct Coombs test

In the absence of hemolytic anemia

10% to 30%

2012 SLICC SLE Criteria

Although these criteria are extremely helpful when considering the diagnosis of SLE for an individual patient, it should be emphasized that these criteria were designed for research purposes and not diagnosis. In particular for mild cases and patients with early disease, the classification criteria may not be sensitive enough to make the diagnosis.

Routine Therapy

  • Topical steroids, intralesional steroids (for DLE)
  • Facial lesions: low to medium potency nonfluorinated (hydrocortisone, desonide).
  • Trunk/arm lesions: medium potency fluorinated (betamethasone valerate, triamcinolone acetonide).
  • Hypertrophic lesions: high potency fluorinated (betamethasone diproprionate, clobetasol). Only use 2 weeks.
  • Hydroxychloroquine: may take 3 months to see effect. Works best for tumid LE > SCLE > DLE.
  • Oral corticosteroids.
  • Dapsone: treatment of choice for bullous lesions.
  • Topical tacrolimus: 0.1% cream BID for 3 weeks as an alternative to topical steroids.

Advanced Therapy for Resistant Causes

  • Subacute cutaneous lupus—mycophenolate mofetil, retinoids, or cyclosporine.
  • Discoid lesions—chloroquine ± quinacrine, thalidomide, or cyclosporine. Rituximab does not work.
  • Lupus profundus—dapsone.
  • Chronic lesions over >50% of body—tacrolimus, mycophenolate mofetil.
  • Vasculitis—may need systemic immunosuppressives.
  • Hyperkeratotic lesions—oral retinoids.
  • Belimumab takes 4 to 6 months to help skin lesions.

The overall 10-year survival is 85% to 90%. However, mortality is increased three times compared to a healthy, age-matched population. Some have emphasized the bimodal pattern of mortality in SLE. Death early in the disease is generally a reflection of active lupus or its treatment (infection), whereas death late in disease is due to atherosclerosis and malignancy. The most common causes of death are:

  • Infection: accounts for 25% of all deaths. All infections (bacterial, fungal, TB, nontuberculous mycobacterial, viral) are increased mostly related to the complications of immunosuppressive therapy, especially to prolonged use of high-dose corticosteroids.
  • Active SLE: accounts for 35% of deaths especially during first 5 years of disease. Lupus nephritis with renal failure, CNS lupus, vasculitis, and pneumonitis are the most lethal.
  • Cardiovascular disease: accounts for 30% to 40% of deaths particularly after 10 years of disease. The risk of coronary artery disease is increased 2-fold to 8-fold in SLE patients.
  • Malignancy: accounts for 5% to 10% of all deaths. The overall standardized incidence ratio (SIR) is 1.15. HPV-related malignancies (cervical cancer) are increased (SIR 5) due to patient exposure to immunosuppressive medications. Patients should be offered the HPV vaccine if under age 26 years and should have annual PAP tests including testing for HPV DNA if at risk. Hematological malignancies (SIR 2.75) and lymphoma (SIR 3.64) are increased 5-fold. Lung cancer is increased (SIR 1.4) in smokers. Squamous cell skin cancers can arise in discoid lesions.


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Web:

American College of Rheumatology

Healthline

Medscape

MedicineNet

10 Common Signs of Lupus

Amboss.com

SouthernCross NZ

Patientenbroschüre Rheumaliga Schweiz (German)