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Symptoms and treatment of lupus

Our immune system defends us from infection, by neutralizing invasive microorganisms (microbes). However, in certain conditions, it may also attack ourselves, through abnormally triggered white blood cells and antibodies (autoantibodies). Such conditions are called autoimmune diseases, and some may concurrently affect several organ systems.

Systemic lupus erythematosus (SLE) is probably the most familiar of these systemic autoimmune disorders. Nearly 2 million people in the U.S. are living with SLE, and the vast majority of these are women, especially of young age (20 to 30 years old). African Americans are three times more commonly affected than Caucasians.

What causes lupus is unknown. Particular immune-system-inherited features, hormones and environmental factors (like infections, drugs and sunlight exposure) are thought to play a role. The severity of SLE is highly variable; some patients may suffer only from skin rash or joint swelling, whereas others may experience heart, kidney, lung or brain problems, sometimes life-threatening.

Lupus is mainly characterized by formation of antinuclear autoantibodies, i.e., antibodies against the cell nucleus, particularly the DNA. These autoantibodies are deposited in various organs, where they trigger inflammation and injury. Other antibodies can destroy blood cells or induce blood clotting within vessels (antiphospholipid antibodies).

SLE may present with a multitude of symptoms, with sudden or gradual onset:

  • Fever and weight loss
  • Skin rash (particularly a butterfly-shaped facial rash, often induced by sunlight exposure) and hair loss
  • Joint inflammation (arthritis), with swelling, pain, redness and stiffness of fingers and hands, worsening during nighttime
  • Lung involvement, with pneumonia, pleural effusion and lung bleeding
  • Cardiovascular disease: heart inflammation (carditis), gangrene and phlebitis
  • Low blood cell count
  • Kidney disease (lupus nephritis), with abnormal protein and blood in the urine and progressive kidney failure
  • Nervous system involvement (neurolupus), with memory loss, headaches, mood disorders, paralysis, convulsions, confusion and meningitis.

Blood tests can detect inflammation and autoantibodies. Skin and kidney biopsies reveal specific changes, and brain imaging is usually needed to diagnose neurolupus.

The evolution of SLE is characterized by recurring flares. With each flare, additional organ damage occurs. In between flares, patients may be completely free of symptoms or may experience chronic organ disease (particularly kidney and brain disease). Flares may be triggered by infection, drugs and pregnancy. During pregnancy, female patients are also exposed to increased risk of miscarriage and stillbirth.

With currently available treatments, 95 percent of patients survive beyond 10 years.

The treatment of SLE includes:

  • General prevention methods: avoiding sunlight, oral contraceptives that contain estrogen, contagious diseases and certain drug exposures. Pregnancy is unwarranted in patients with recent or ongoing lupus flares, as well as in those with advanced nephritis or neurolupus.
  • NSAIDs (non-steroidal anti-inflammatory drugs), such as Aspirin or Advil, are helpful in cases of mild arthritis. Such drugs, however, may induce gastrointestinal, kidney and cardiovascular side effects.
  • Hydroxychloroquine (Plaquenil) is essential for treating moderate-to-severe arthritis, as well as skin troubles. Regular ophthalmology exams are needed to prevent possible eye toxicity from this drug.
  • Steroids (Medrol) and immunosuppressive agents like cyclophosphamide (Cytoxan) and azathioprine (Imuran) are required for severe organ-damaging and life-threatening SLE, as nephritis and neurolupus. Effective as they are, these drugs can also cause serious adverse effects, such as: infection, obesity, diabetes, high blood pressure and cholesterol, gastro-duodenal ulcer (with steroids), hair loss, cystitis, bone marrow damage, infertility, leukemia, infection and cancer (with immunosuppressors). Careful monitoring and using as small a dose as possible are mandatory to avoid these effects.

By Sal Levy

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