The immune system is the body's defense system against infection, disease, and foreign substances. Once stimulated, the immune system automatically turns on to locate and fight invading cells.
An autoimmune disease develops when the body's immune system fails to recognize normal body tissues and attacks and destroys them as if they were foreign rather than attacking an outside organism. The cause is not fully understood, but in some cases it is thought that autoimmune diseases are triggered by exposure to microorganisms or other environmental causes, especially in people with a genetic predisposition to the disorder. A single organ or multiple organs and tissues may be affected.
There are nearly 150 autoimmune disorders. While there are no cures for these disorders, Seattle Cancer Care Alliance (SCCA) provides expert care for people to maintain a high quality of life.
If your condition requires a bone marrow transplant, you should know that we are one of 15 centers whose transplant patients achieved higher-than-expected survival rates, according to a multi-year study by the Center for International Blood and Marrow Transplant Research. The study compared patients at more than 165 bone marrow transplant centers in the United States. We’ve performed more bone marrow transplants than any other institution in the world. The Hutchinson Center pioneered the use of bone-marrow transplants as a treatment for blood and autoimmune diseases over 40 years ago. Since then, many patients with serious autoimmune diseases have come from around the world to receive bone marrow transplants at SCCA.
Patients with autoimmune diseases may be seen at SCCA , UW Medical Center, or Seattle Children’s, depending upon their age and diagnosis.
Each person's immune system is unique and treatment varies from person to person. Treatment usually includes medications to alleviate symptoms, whether it be simply non-steroidal anti-inflammatory medications, such as aspirin and ibuprofen, to reduce symptoms depending on the specific disease, or drugs like Cyclophosphamide (Cytoxan) or Azathioprine (Imuran) to suppress or slow down the immune system and thus slow down progression of the disease. Some may even be treated with a stem cell (bone marrow) transplant, which occurs at SCCA’s Transplant Clinic.
Fred Hutchinson Cancer Research Center (Hutchinson Center), an SCCA parent organization, is taking the lead on several fronts in treating autoimmune diseases and has developed clinical trials using stem cell transplantation for treating severe autoimmune diseases.
Test for Lupus
Hutchinson Center scientists have developed a test for lupus that will help physicians make more accurate diagnostic and prognostic decisions for patients and therefore help those patients get appropriate treatment. Diagnosis for this disease is complex due to the similarity of symptoms with other autoimmune diseases. In an experiment, Mark Roth, a basic science investigator at Hutchinson Center, and, Karla Neugebauer, a former post doctoral fellow in his lab, discovered that lupus patients make antibodies to SR proteins, a family of splicing proteins discovered by the lab. Hutchinson Center has filed for patent protection on this assay system. A diagnostic kit based on Roth's assay will identify a population of 50 to 70 percent of lupus patients who react positively to SR proteins. To bring this work to the benefit of the public, Hutchinson Center researchers spent two years improving the test kit using the rigorous guidelines established by the Food and Drug Administration, which gave its consent for U.S. distribution in 2002.
Hutchinson Center is also leading a global team to accelerate the investigation of immune-related genes. A cluster of nearly 220 genes known as the human leukocyte antigen (HLA) gene complex holds clues to these and other medical issues. In search of answers, the National Institutes of Health launched a $20 million initiative in 2001 to catalog the HLA gene complex. The effort among 200 laboratories in more than 70 countries is lead by Hutchinson Center's Dr. John Hansen. The group will set up a centralized HLA gene database. For patients, the benefits include finding better matches for bone-marrow transplant patients.
If your condition is one that requires a bone-marrow transplant, be sure to visit the Bone Marrow Transplant section.
Multiple Sclerosis is an autoimmune disease of the central nervous system with symptoms ranging from numbness and tingling to paralysis. In MS, the body attacks the myelin sheath, which coats nerves in the brain and spinal cord. As a result, the ability of the nerves to conduct electrical impulses to and from the brain is disrupted. An estimated 300,000 people in the United States have MS. More women are affected than men.
Risk factors: Scientists believe that several factors are involved in the development of MS: genetics, gender, and environmental factors (e.g., viruses, trauma and heavy metals). The incidence of MS is higher in more northern latitudes, suggesting an environmental component to the disease. Epidemiology surveys have determined that a person's risk of developing MS increases several-fold if a close family member has MS.
Symptoms: Symptoms are caused by the destruction of patches of myelin, a protective covering of nerve fibers in the brain and spinal cord. The symptoms can be mild, moderate or severe. 85 percent of the time, MS symptoms appear as a defined flare-up followed by long periods of remission. In more moderate cases, people with MS experience a progressive worsening of the disease with no distinct relapses or remissions. In the most severe cases, which are rare (5 percent), patients experience a progressive worsening of the disease and acute flare-ups. Symptoms of MS are unpredictable and vary greatly from person to person depending on the location of the nerve lesion. The symptoms include: abnormal fatigue, vision problems, dizziness, vertigo, bladder dysfunction, loss of balance and muscle coordination, slurred speech, tremors, stiffness, hearing loss, seizures, itching, numbness, sexual dysfunction, depression and pain.
Diagnosis: No single test is available to diagnose MS but several procedures are used. An MRI scan gives a view of the brain and makes it possible to visualize and count damaged areas or plaque in the brain and spinal cord. Evoked potential tests of electrical activity can detect a slowing in nerve impulses caused by demyelination. A spinal tap checks cerebrospinal fluid for signs of the disease. People with MS usually have elevated levels of IgG antibodies as well as oligoclonal bands in the cerebrospinal fluid. Sometimes, there are also certain proteins that break down products of myelin.
Systemic Lupus Erythematosus
Systemic Lupus Erythematosus is a chronic inflammatory disease characterized by inflammation in multiple organ systems, frequently involving skin, joints, and the kidneys. Lupus most commonly affects women between the ages of 20 and 40 years. The Lupus Foundation estimates that up to 1.5 million Americans have been diagnosed with lupus.
Symptoms: Lupus can affect any part of the body but most people experience symptoms in only a few organs. The most prevalent symptoms of lupus include achy joints, fever, arthritis, extreme fatigue, skin rashes and anemia. Symptoms also include, pain in the chest, butterfly-shaped rash across the cheeks and nose, sun or light sensitivity, hair loss, abnormal blood-clotting problems, Raynaud's Phenomenon (fingers turning white and/or blue in the cold), seizures and ulcers in the nose and mouth.
Diagnosis: A diagnosis may be difficult because many of the symptoms are similar to other diseases. To help doctors diagnose lupus, the American College of Rheumatology has issued a list of symptoms to distinguish lupus from other diseases. The individual should have four or more of the symptoms to confirm a diagnosis of lupus.
Risk factors: A number of environmental factors play a critical role in triggering lupus. Those include infection, antibiotics, ultraviolet light, extreme stress, certain drugs and hormones. While there is no known gene or genes thought to cause lupus, the disease is known to occur within families. Lupus can occur at any age and is 10-15 times more frequent among adult women. The disease shows an increase of symptoms before menstrual periods and during pregnancy, which supports the belief that the hormone estrogen may play a role in the disease.
The most common manifestation of systemic sclerosis is Scleroderma (hard skin). The thickening and hardening of the skin-and possibly of other organs, is caused by the production of too much collagen. Early indications of the disease include joint pain and swelling of the hands, tightening skin and sensitivity to cold. This reaction to temperature changes occurs when the blood vessels in the fingers and toes narrow and may spasm when exposed to extremes of cold or heat, resulting in color change (Raynaud's phenomenon). The fingers become swollen and small blood vessels may be prominent; gradually the skin over the face and fingers becomes thickened and tight. In severe cases the lungs, heart and kidneys can be affected.
Treatment of Scleroderma is difficult and the condition tends to progress gradually. However, rapid progression of the disease is seen in more severe cases.
There are two kinds of Scleroderma: The systemic form, which affects the internal organs as well as the skin, and the localized form, which affects a specific area of skin. Possibly as many as 300,000 people in the United States have Scleroderma with about four times as many women as men being affected.
Risk factors: In most cases, the cause of Systemic Sclerosis is unknown.
Symptoms: Generalized symptoms can include: Raynaud's phenomenon, swollen, painful and or stiff joints; thickening of skin, difficulty swallowing, weight loss, fatigue, muscle aches and joint weakness, shortness of breath.
Diagnosis: A diagnosis may be very difficult in the early stages because many of the symptoms overlap with other diseases such as Rheumatoid Arthritis, Lupus, and Polymyositis. Confirmation may depend on blood studies and specific organ function tests. Diagnosis is aided by the presence of auto-antibodies in the blood.