Lupus

Background

Systemic lupus erythematosus (SLE) is an inflammatory connective tissue disease with variable manifestations that range from mild to severe.  The symptoms can affect many organ systems.  SLE is often a chronic, life-long illness.

Pathophysiology – What causes SLE?

No single cause of SLE has been identified.  Complex relationships between environmental factors, genetically predetermined host immune complexes, and hormonal influences are significant in the development and manifestation of the disease.

Incidence

SLE is a fairly common disease in the United States, with approximately 15-50 cases per 100,000 people in the United States.

About 90% of lupus patients are women, with a female-to-male ratio of 8:1-13:1 in adults.   Most women are diagnosed during the childbearing age.  This is believed to be secondary to hormonal changes.

The highest incidence of SLE is among Asians in Hawaii, African Americans, and certain groups of Native Americans.  African American women are 3 times more likely than Caucasian women to be affected by SLE.

Mortality/Morbidity

The 10-year survival rate is 70-90% for individuals with SLE.   Female patients with onset of the disease after age 60 years have the most favorable prognosis while children with SLE have a less favorable prognosis.

Symptoms (in decreasing frequency):

Achy joints (arthralgia)*

Fever over 100 degrees F

Prolonged or extreme fatigue

Swollen joints*

Skin rashes 

Anemia

Kidney involvement

Pain in the chest on deep breathing (pleurisy)

Butterfly-shaped rash across the cheeks and nose

Sun or light sensitivity (photosensitivity)

Hair loss

Raynaud's phenomenon (fingers turning white and/or blue in the cold)

Seizures

Mouth or nose ulcers

*Arthralgia and symmetrical arthritis often are features of acute SLE.  Patients occasionally can get joint deformities that occur secondary to tendon and ligament laxity.  Individuals with SLE can also suffer from muscle pain secondary to muscle inflammation

Diagnosis Criteria

1983 American Rheumatism Association Revised Criteria for Classification of Systemic Lupus Erythematosus (SLE)

Treatment

Diet:  An anti-arthritis diet under the supervision of a physician and/or nutritionist can be a very helpful adjunctive treatment for SLE as well as other forms of arthritis.

Medications: 

            All of the following medications, including nonsteroidal anti-inflammatory drugs) carry potentially significant side effects. Always discuss with your physician all potential benefits and side effects of medications you are considering so that you can make an informed decision in conjunction with your physician.

Nonsteroidal anti-inflammatory drugs: Ibuprofen and naproxen are among many examples of NSAIDs that are being rapidly added to the list of available options. These medications are used in full anti-inflammatory doses for fever, joint pain, and serositis.  Side effects may include stomach ulcers, stomach bleeding, other gastrointestinal problems, high blood pressure, and kidney damage. 

Corticosteroids: Have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.  Side effects may include lowered resistance to infection, increased thirst, irritability, gastrointestinal disorders, and insomnia as well as others.

Immunosuppressive agents: Used in patients with the most severe disease; immunosuppressive agents reduce humoral and cellular responses; azathioprine and cyclophosphamide are the most commonly used drugs.  Side effects may include increased cancer risk (in azathioprine when taken long term), increased risk of infection, nausea, vomiting, decreased liver function, decreased appetite, hair loss, jaundice, mouth sores, and decreased fertility as well as others. 

Antimalarial drugs: Often used to treat fatigue, skin disease and arthritis; hydroxychloroquine and chloroquine are the most commonly used drugs.  Side effects may include stomach upset, nausea, vomiting, headache, and kidney problems.

Physical therapy (PT)/Occupational Therapy (OT):  Physical therapy can reduce pain, stiffness, and inflammation, as well as improve joint range of motion (ROM) and functional mobility. Occupational therapy helps people with severe SLE restore their functional independence to the extent possible in spite of the problems caused by the disease.  Becoming enrolled in a well structured therapy program under the direction of a qualified physician can be invaluable to a patient with SLE.  After several sessions with a physical and/or occupational therapist, the patient can be instructed on a home exercise program. 

Authors:  Brett Gertsman, BA, UMDNJ-Robert Wood Johnson Medical School, Piscataway, NJ

              Ana Bracilovic, M.D., New York-Presbyterian Hospital, The University Hospital of Columbia and Cornell, New York NY