Goodpasture's Syndrome Information: Autoimmune Disease

Goodpasture's syndrome (anti - glomerular basement membrane antibody disease or Anti - GBM disease) is a rare autoimmune disease, affecting the lungs and kidneys. In a healthy person, the immune system functions to create antibodies to fight off germs. 1 Goodpasture's syndrome condition antibodies trigger inflammation that interferes with lung and kidney function: Damaging structures in the walls of the tiny sacs (alveoli) and capillaries (blood vessels) of the lungs and in the filtering process of the kidneys. 2 In 1919, Ernest Goodpasture first discovered a type of autoimmune disease: Described an 18 year old man with symptoms, known today as Goodpasture's syndrome. Predominately Goodpasture's syndrome affects mostly racial groups of white people. 8 "This syndrome may occur at any age but is most common in men between ages 20 and 30" (Six times more likely for males than females. 12). 3 Men and women 50 - 70 years equally affected or diagnosed with this type of autoimmune disease. 8 Also, when Anti - GBM disease is diagnosed later in life, tends to affect just the kidneys. 10 However, Goodpasture's syndrome is rarely diagnosed in children. According to 1999 United States Renal Data Systems (USRDS) Annual Data Report, from 1993 - 1997, Anti - glomerular basement antibody disease diagnosed in thirty-seven pediatric patients (younger than 20 years). 8

Researchers are not exactly certain as to the cause of Goodspasture's syndrome, but narrowed their focus to include presence of an inherited gene, viral infections, exposure to certain chemicals, and hydrocarbon solvents and the weed killer Paraquat. 4 Glue sniffing and siphoning gasoline may cause this autoimmune disease. Genetically renal failure exists among family members who are smokers. 5 Some cases of Goodspasture's syndrome linked to type Two Influenza (Flu). 3 Also, Anti - GBM disease suspected link to exposure of metal dusts, heavy smoking crack cocaine, and Alport's syndrome (renal failure). 10

Anti - glomerular basement membrane antibody disease attributed to a variety of symptoms: First signs may be fatigue, nausea, shortness of breath, nausea, or paleness. 1 These symptoms are followed by Foamy, bloody or dark colored urine, decrease urine production, (Also, feel burning sensation when urinating. 1) iron deficiency, coughing up blood, vomiting, weight loss, and nonspecific chest pain and/ or pale skin. 5 Also, common symptom of Anti - GBM disease nodular rashes (may appear on the elbows). 11 Diagnosis for Goodpasture's syndrome confirmed by clinical test results. These tests include blood urea nitrogen (BUN) and creatinine levels, test the kidney function including urinalysis, check blood for iron level deficiency (anemia), chest X ray assess for fluid in the lung tissues, and biopsy of the kidney and lung confirm immune system deposits. "The kidney biopsy can also show the presence of the harmful antibodies that attack the lungs and kidneys". 5

Goodpasture's syndrome most effectively treated when the disease is diagnosed early and before kidney is permanently damaged and dialysis (Method removing impurities or wastes from the blood when kidneys fail to function. 7) is necessary. 5 Goodpasture's syndrome is treated with oral immunosuppresive drugs keep immune system from making antibodies, such as cyclophosphamide (or azathioprine 5) (Usually given for three months 10) and corticosteriods (Such as prednisone 5) (control bleeding in the lungs). Usually in combination of immunosuppresive drug treatment process called plasmapheresis recommended (1): "Plasmapheresis is used to remove antibodies from the bloodstream, thereby preventing them from attacking their targets". 6 During this process about 300 ml of blood is drawn at a time, and placed in a centrifuge to separate the red and white blood cells from plasma. Afterwards, cells are then placed in a plasma substitute and returned to the body.1 Goodpasture's syndrome patients may require supportive care such as supplemental oxygen or may need to be placed on ventilator for a period of time, until the diseases runs its course. 2 Mortality rate for Goodpastures' syndrome about ten percent compared to previously higher- rate, when treatment and diagnostic options were not available.10

Goodpasture's autoimmune disease syndrome may last a few weeks or as long as two years, depending upon how a patient reacts to treatment. If kidney dialysis fails to remove waste products or extra fluid from the blood, a kidney transplant may be required. The disease normally does not lead to permanent lung damage. 4 During a pregnancy Goodpasture's syndrome both mother and baby risk serious health concerns. It may produce hypertension, and associated intrauterine growth retardation ("Intra-uterine growth restriction (IUGR) is a condition when a baby's growth slows or ceases when in it is in the uterus".) requiring premature delivery. 10 Goodpasture's syndrome occurs rarely in domestic animals: Diagnosed in horses and suspected to occur in dogs. 13 At least one case reported, 17 year old boy diagnosed with Goodpasture's syndrome successfully treated with prednissone, cyclophosphamide and courses of plasmapheresis. However, three years later this autoimmune disease recurred and again successfully treated. 9

For more information regarding Goodpasture's Syndrome contact: American Kidney Fund (helpline@kidneyfund.org) and National Kidney Foundation, Inc (www.kidney.org).)

References:

1.) Goodpasture's syndrome - http://www.cidpusa.org/Goodpasture%92s%20syndrome.html

2.) Goodpasture's Syndrome - http://www.merck.com/mmhe/sec04/ch051/ch051e.html

3.) What is Goodpasture's Syndrome - http://www.articlesbase.com/health-articles/what-is-goodpastures-syndrome-307224.html

4.) Goodpasture's Syndrome - http://kidney.niddk.nih.gov/kudiseases/pubs/goodpasture/

5.) Goodpasture's syndrome - http://www.lifesteps.com/gm/Atoz/ency/goodpastures_syndrome_pr.jsp

6.) Plasmapheresis - http://www.healthatoz.com/healthatoz/Atoz/common/standard/transform.jsp?requestURI=/healthatoz/Atoz/ency/plasmapheresis.jsp

7.) Dialysis - http://www.umm.edu/ency/article/003421.htm

8.) Goodpasture Syndrome: Article Excerpt by Rudolph P. Valentini - http://emedicine.com/ped/byname/goodpasture-syndrome.htm

9.) Recurrent Goodpasture's syndrome - http://www.cmaj.ca/cgi/content/abstract/129/9/978

10.) Goodpasture's Syndrome - http://www.patient.co.uk/showdoc/40001240/

11.) Hoarsness and Hemoptysis in a 28 - Year - Old Pregnant Woman - http://www.thoracic.org/sections/clinical-information/ats-clinical-cases/pages/hoarseness-and-hemoptysis-in-a-28-year-old-pregnant-woman.html

12.) Goodpasture's syndrome - http://www.reference.com/browse/wiki/Goodpasture's_syndrome

13.) Goodpasture's syndrome - http://www.answers.com/topic/goodpasture-s-syndrome?cat=health