Lupus

Understanding lupus (also known as systemic lupus erythematosus or SLE) can be a very difficult task because it is such a complex disease. Lupus can affect different organ systems, such as the skin, joints, kidneys, heart, lungs, blood vessels, and brain. Lupus is an autoimmune disease in which the autoantibodies attack different tissues, which can lead to different manifestations. Lupus occurs 10 times more commonly in women than [Page 1038]in men. It usually occurs in people in their 20s and 30s, and people with certain ethnic backgrounds, especially Asian and African American. The etiology of lupus is unknown, but researchers are still investigating the connection between the environment, genetics, and even hormonal factors as possible causes of the disease.

Lupus is described as periods of disease activity, which are called relapses, and periods of alleviation, which is called remission. Flares can be triggered by the exposure to ultraviolet rays, which can cause a malar rash. The cause of the rash has to do with the autoantibodies depositing on self-antigen creating immune complexes deposited in the epidermis, triggering inflammation, which ultimately recruit other immunoregulatory cells. These immunoregulatory cells can release proinflammatory cytokines that can induce tissue damage or cause deposition of immune complexes. This can manifest as malar rash or rash anywhere on the body, severe arthralgias, persistent weakness, involuntary weight loss, involuntary hair loss, high fever, and blood in the urine, to name a few.

The diagnosis of lupus is based on criteria developed by the American College of Rheumatology (ACR) to help distinguish the severity of SLE based on clinical symptoms. To be diagnosed with SLE, a patient must have four or more of the 11 criteria to qualify for the classification category of SLE:

• 1)
  Malar rash: Fixed rosy rash, usually flat or raised over the cheekbones, sparing the upper lip region. The malar rash is sometimes referred to as the “butterfly rash.”
• 2)
  Discoid rash: Reddish, rosy patches with keratotic scaling anywhere on the body.
• 3)
  Photosensitivity: Skin rash due to exposure to ultraviolet rays observed by the patient or physician.
• 4)
  Oral ulcers: Painless ulcers in the mouth, nose, or throat lasting more than two weeks observed by the physician and or patient.
• 5)
  Arthritis: Tenderness, swelling, or effusion affecting joints without causing joint erosion. To include this criterion, the patient has to have arthritis affecting two or more peripheral joints.
• 6)
  Serositis: Inflammation of the tissue sac surrounding the lungs (pleuritis) or the heart (pericarditis).
• 7)
  Renal disorder: Persistent loss of protein in the urine (proteinuria) greater than 0.5 g per day and as high as 3 g per day. The presence of proteinuria can be determined by the physician. The involvement of the kidneys can be further categorized by the World Health Organization stages. The stages range from one to four, stage one being mild involvement of immune complexes depositing in renal tissue. Stage four is categorized as being the most severe stage of renal involvement, which would be caused by severe damage to renal glomeruli.
• 8)
  Neurologic disorder: The presence of seizures, without the cause of seizures being drugs or any metabolic disorder (e.g., uremia, ketoacidosis, or electrolyte imbalances).
• 9)
  Hematologic disorder: Hemolytic anemia, any major loss of white blood cells or platelets.
• 10)
  Immunologic disorder: Presence of autoantibodies, especially anti-dsDNA, anti-Sm, or anti-phosholipid antibodies.
• 11)
  Antinuclear antibody: An abnormal titer of antinuclear antibody (ANA) by immunofluorescence.

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