Goodpastures Syndrome



Definition:

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 mabe 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

deteriorated 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

Expectations(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



CAUTION!!!!

This infomation is not a diagnosis. These symptoms should be made

aware to your doctor and then he will make a discision.



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