Department of Medicine

University of Pittsburgh

Lupus Center of Excellence

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Definition

Lupus is a chronic, inflammatory disease, which can affect virtually any organ system of the body, but mostly involves the skin, joints, kidney, brain, blood, and heart. Normally, the body's immune system forms antibodies, which identify and act against 'foreign' cells, such as bacteria and other infections. In lupus, autoantibodies develop and attack the body's normal tissues and organs. The disease is characterized by periods of illness, known as 'flares' and periods of feeling well, known as 'remission' or inactivity.

Lupus tends to affect women more often than men. Nine of 10 patients with lupus are women. Women of African American, Hispanic and Asian descent are more likely to develop lupus than Caucasian women.

The exact cause of lupus is unknown. Experts believe that lupus may be caused by a combination of genetic and environmental factors, but sometimes it can be triggered by the use of certain medications such as hydralazine, isoniazid or minocycline. When it is medication-related, often discontinuation of the drug helps to resolve the symptoms. Sometimes, lupus may only affect the skin, in which there is no internal organ involvement. Examples of this include discoid lupus and subcutaneous cutaneous lupus erythematosus (SCLE).

Signs and symptoms of Lupus

Common symptoms of lupus include rashes, joint pains, muscle aches, fever, fatigue, chest pain with difficulty breathing and sun sensitivity. Many lupus patients have anemia and increased platelets on lab testing. Complications of lupus include kidney disease, nervous system involvement (seizures, memory loss, headaches) and heart disease (heart attacks, strokes).

Lupus is often considered the 'great imitator' since many diseases can mimic lupus symptoms. Examples include fibromyalgia, thyroid disease, infections, rheumatoid arthritis and a host of other conditions.

Diagnosis

Lupus is diagnosed based on the symptoms mentioned above and autoantibodies, which are tested by blood work.

  1. Antinuclear antibodies (ANA): Commonly done test to look for lupus. While most patients with lupus will test positive or have an abnormal test for ANA, sometimes an abnormal ANA is found in healthy people or in people with thyroid disease, psoriasis, inflammatory bowel disease and other conditions.
  2. Confirmatory antibodies for lupus: Several confirmatory antibodies that bind certain self-proteins or DNA (Sm and dsDNA) in patients with lupus are available. These tests are are done to confirm the presence of disease, once lupus is suspected.
  3. Complements: Such as C3, C4 and CH50 are done every few months to test for disease activity of lupus. The complement tests are lower in patients with active lupus.
  4. Inflammatory markers: Laboratory tests such as C-reactive protein (CRP) and sedimentation rate (ESR) are done to monitor inflammation in the blood.
  5. Urinalysis: Done every 3-6 months to check for red blood cells and protein in the urine, which may indicate lupus involving the kidney.
  6. Antiphospholipid antibodies: Patients with lupus may have an increased tendency to form blood clots and anti-phospholipid antibodies help to predict which patients are likely to have clots. If found, patients may need treatment with aspirin or blood thinners.
  7. Rheumatoid factor, Sjogren's antibodies: May be done if your physician suspects that you may have another disease with lupus, such as rheumatoid arthritis or Sicca syndrome (dry eyes and dry mouth).

Other tests commonly done in lupus patients:

  1. 2-D echocardiogram (ECHO) helps to look for elevated pressures in the pulmonary artery (a large vessel that carries blood from the heart to the lungs) or to look for fluid around the heart due to inflammation from lupus.
  2. Chest X-ray helps to look for fluid in the lungs due to inflammation from lupus.
  3. Pulmonary or lung function tests (PFTs) to look for lung disease.
  4. Kidney biopsy if there is suspicion for kidney disease.
  5. Cardiac stress test to look for early heart disease in women with lupus.
  6. Lipid panel for control of high cholesterol in lupus patients.
  7. MRI of the brain for evaluation of headaches, seizures or memory loss in lupus patients.

This is not a comprehensive list of testing available for patients with lupus and often the diagnostic testing is tailored to the patient's condition and needs.

Treatment

The medications listed below should only be used under the guidance of an experienced rheumatologist or lupus specialist.

Some of the common medications used to treat lupus include:

  1. Non-steroidal anti-inflammatory medications (NSAIDs): Agents such as naproxen, ibuprofen or meloxicam can be used for treatment of rashes and joint pains in lupus. These will help most patients, but need to be used cautiously because of risks of bleeding gastric ulcers and kidney disease.
  2. Prednisone (steroid/cortisone): One of the most commonly used medications for lupus, to be used in 'flares' of disease. Many patients need a low dose of prednisone to control their lupus over the long-term. Since steroids have many side effects, physicians often need to use other agents to help reduce the dose of daily prednisone. Dermatologists may prescribe steroid creams or ointments for lupus rashes.
  3. Hydroxychloroquine (Plaquenil): This is one of the better drugs for lupus. Most physicians will recommend indefinite or life long treatment with hydroxychloroquine to control symptoms of lupus, particularly involving the skin, hair, joints and fatigue. It is one of the safer drugs and has been proven to reduce complications of disease and to prevent flares of disease.
  4. Methotrexate (MTX): Often used in rheumatoid arthritis patients, this has been found to have a place in the management of lupus joint disease and rashes.
  5. Azathioprine (Imuran): Useful for blood involvement, serositis and kidney disease from lupus.
  6. Mycophenolate mofetil (Cellcept): Used primarily for kidney disease in lupus and for other more serious manifestations of lupus.
  7. Cyclosphosphamide (Cytoxan): Used primarily to treat central nervous system and kidney involvement and occasionally lung disease from lupus.
  8. High doses of intra-venous corticosteroids: May be used in severe 'flares' of disease.
  9. Rituximab: May be used 'off-label' for lupus kidney disease and brain disease and for severe systemic involvement in lupus.
  10. Belimumab (Benlysta): Newest drug approved for lupus, which will be used after other medications have been tried and failed to achieve success.
  11. Aspirin/Warfarin (Coumadin, blood thinners): May be used in patients with a tendency for blood clots to prevent strokes, lower extremity and lung blood clots.

As one can tell, these are complex treatments, all of which require regular blood work and disease monitoring to ensure their safety and efficacy for lupus patients. Lupus patients should be seen by a lupus expert or rheumatologist every 3-4 months to assess disease activity and to help control any ongoing symptoms and monitor for drug side-effects.

Although there is still a need to find a cure for many autoimmune diseases, the hope is to achieve the best control of the disease and strive for disease remission with a goal of helping reduce disability and improving quality of life for lupus patients.

Other important components of lupus therapy include supportive care and counseling, treatment for depression and anxiety, use of sunscreen to protect against flares, treatment for associated fibromyalgia, and pain management.

ABOUT THE UPMC LUPUS CENTER OF EXCELLENCE:

The UPMC Lupus Center of Excellence offers comprehensive medical care for lupus patients in the region, serving not only the Western Pennsylvania region, but also Ohio and West Virginia. The UPMC Lupus Center serves as an important referral center for many other states that need specialized care for complicated difficult-to-treat lupus patients. The physicians at the UPMC Lupus Center of Excellence perform a careful review of the patient's history and medical records, physical examination and lab tests to ensure a correct diagnosis and offer excellent diagnostic and medical care for lupus patients.