Systemic lupus erythematosus (SLE)

Systemic lupus erythematosus

Systemic lupus erythematosus (SLE), (Lupus is Latin for wolf): is an autoimmune disease that can affect any part of the body. Like every other autoimmune diseases, the immune system attacks the body’s own cells and tissue, resulting in inflammation and tissue damage. SLE is a Type III hypersensitivity reaction in which antibody-immune complexes precipitate and cause a further immune response.

Systemic lupus erythematosus most often harms the heart, joints, skin, lungs, blood vessels, liver, kidneys, and nervous system. Unfortunately the course of the disease is random, it presents with periods of illness also called flares, and alternating with remissions. The disease occurs nine 9x more often in women than in men just like other autoimmune diseases, especially in women in child-bearing years ages 15 to 35, and is also more common in those of non-European descent & African American decent.

 

Signs and Symptoms of Systemic lupus erythematosus

The diagnosis of Systemic lupus erythematosus (SLE) is based on the presence of at least 4 of 11 known manifestations of the disease, also known as ACR criteria:

Systemic lupus erythematosus

  • Malar rash (rash on cheeks),
  • Discoid rash (red, scaly patches on skin that cause scarring),
  • Serositis: Pleurisy (inflammation of the membrane around the lungs) or pericarditis (inflammation of the membrane around the heart),
  • Oral ulcers (includes oral or nasopharyngeal ulcers),
  • Arthritis: non-erosive arthritis of two or more peripheral joints, with tenderness, swelling, or effusion,
  • Photosensitivity (exposure to ultraviolet light causes rash, or other symptoms of SLE flareups),
  • Hematologic disorder:
    • Autoimmune hemolytic anemia,
    • Antiphospholipid syndrome,
    • Leukopenia,
    • Lymphopenia,
    • Thrombocytopenia,
    • Hypocomplementemia (complement deficiency) is also seen, due to either consumption of C3 and C4 by immune complex-induced inflammation or to congenitally complement deficiency, which may predispose to SLE.
  • Renal disorder: Lupus nephritis, protein in urine or cellular casts seen in urine under a microscope.
  • Antinuclear antibody test positive (ANA): Found in 95%-99% of cases. A negative ANA is extremely sensitive for lupus, but a positive ANA has a little specificity. In other words almost all patients with lupus have a positive ANA. If the test for ANA is negative then the patient does not have SLE.
  • Immunologic disorder:
    • Positive anti-Smith,
    • Anti-ds DNA,
    • Antiphospholipid antibody,
    • False positive serological test for syphilis,
    • Anti-SSA and Anti-SSB: found in 10% to 20% of cases. They add little to the diagnosis. These tests are most often found in Sjögren syndrome (65% of cases),
    • Antihistones antibody: this is due to some drugs (for example: Sulfa drugs, Hydralazine, Isoniazid, Procainamide, Phenytoin, Quinidine.)
  • Neurologic disorder: Seizures or psychosis.

Other than the ACR criteria, people with Systemic lupus erythamatosus may also have:

  • Fever
  • Extreme fatigue
  • Hair loss
  • Raynaud’s phenomenon: Fingers turning white and/or blue when cold

 

Treatment of Systemic lupus erythematosus

There is currently no cure for Systemic lupus erythematosus. However There are treatments available which involves preventing flares and reducing their severity and duration when they occur.

  • Corticosteroids (for acute lupus flares)
  • Hydroxychloroquine (Control mildly chronic disease limited to skin and joint manifestations)
  • Belimumab (controls progression of the disease)
  • Cyclophosphamide (used in combination with steroids for lupus nephritis)
  • Mycophenolate (used in combination with steroids for lupus nephritis)
  • NSAIDs
  • Intravenous immunoglobulins (may be used to control SLE with organ involvement, or vasculitis)
  • Aspirin, Heparin, Warfarin: For Antiphospholipid syndrome. (Heparin is used during pregnancy)

 
References for Systemic lupus Erythematosus:

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