A form of rapidly progressive glomerulonephritis(inflammation of the kidney glomeruli)involving progressive decrease in kidney function, accompanied by a cough with bloody sputum. Causes,incidence, and risk factors:

The disorder is charaterized by deposits of antibodies in the basement membranes of both the kidneys glomerulus and the lung alveoli, causing both glomerulonephritis and pulmonary(lung) bleeding. The exact cause is unknown. It is an autoimmune disorder(action of the immune system against normal body tissues)

Sometimes the disorder is triggered by a viral infection or by inhalation of gasoline and hydrocarbon solvents.

The disorder is rare,affecting approximately 1 out of 100,000 people. It is most often seen in men ages 16 to 61, usually around 20 years old. Smoking increase the risk of developing the disorder. It may develop after a recent viral respiratory infection. There is an inherited predisposition for Goodpasture's Syndrome.

The disease usually manifests with the excretion of protein and blood in the urine. It progresses rapidly to kidney failure. Antibody deposits in the lungs cause bleeding within the lung tissue, resulting in the bloody sputum, which maybe one of the first symptoms of the disorder. Iron deficiency anemia maybe present, as well as anemia associated with renal failure, and maybe worse than expected concidering the amount of kidney damage.



PREVENTION:

There is no known prevention for Goodpasture's Syndrome. Avoid glue sniffing and siphoning gasoline. Stop smoking to decrease risk in those with a family history of renal failure. Early diagnosis and treatment may slow the progression of the disorder.



SYMPTOMS:

bloody urine
dark colored urine
decreased urine output
foamy urine
cough with bloody sputum(coughing up blood)
difficulty breathing after exertion
weakness
general discomfort,uneasiness,or ill feeling
fatigue
nausea/vomiting
weight loss
nonspecific chest pain
pale skin



SIGNS AND TESTS:

Ausculation of the lungs may indicate fluid(from bleeding in the lungs). Occasionally, blood pressure maybe high. Edema(swelling) of the body may develop as kidney deteriorates. Skin rash maybe observed in some cases.

A CBC often indicates anemia Serum iron and ferritin maybe low
BUN and creatinine levels increase as kidney function decreases.
A urinalysis may show protein, blood, casts or other abnormality.
Surum antibody to normal human glomerular basment membrane is positive
Sputum stain may indicate macrophages(immune system cells that respond to the presence of antibodies) that contain iron pigmants
a chest x-ray shows fluid in the lung tissue
a lung needle biopsy shows immune system deposits
a kidney biopsy shows immune system deposits, with crescentic(shaped like a "new moon")
deposits indicating rapidly progressive glomerulonephritis



TREATMENT:

Treatment is focused on slowing progression of the disease. It is most effective when begun early, before kidney function has deterirated to the point of requiring dialysis. Corticosteroids or other antiflammatory agents maybe used to reduce the immune response, with variable results. Immune suppressants such as cyclophospamide(cytoxan) or azathioprine are used aggressively to reduce immune system effects. A procedure by which blood plasma, which contains antibodies, is removed from the body and replaced with fluids or donated plasma(plasmapheresis) maybe performed daily for 2 or more weeks to remove circulating antibodies. It is fairly effective in slowing or reversing the disorder

Dialysis maybe required if kidney function is poor Kidney transplant maybe quite successful, especially if performed after circulating antibodies have been absent for several months



PROGNOSIS:

The probable outcome is variable. Most cases progress to severe renal failure and end-stage renal disease within months. Early diagnosis with treatment makes the prognosis(probable outcome) more favorable



COMPLICATIONS:

rapidly progressive glomerulonephritis
chronic renal failure
end-stage renal failure








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