Goodpasture's Syndrome
Other names: Goodpasture’s Disease, Anti-Glomerular
Basement Membrane Disease, Anti-glomerular Basement
Membrane Antibody Disease, Rapidly Progressive Glomerulonephritis
with Pulmonary Hemorrhage, Pulmonary Renal Syndrome,
Glomerulonephritis - Pulmonary Hemorrhage
Goodpasture's Syndrome is a rare
autoimmune that can cause hemorrhaging in the lungs
and a renal disease called glomerulonephritis where
the small blood vessels in the kidneys become inflamed
Some forms of the disease involve just the lung or
just the kidneys, not both. The initial onset of the
disease can be subtle and the first symptoms might
include fatigue, nausea, shortness of breath and paleness.
As the disease progresses, more symptoms such as coughing
up blood or a burning sensation when urinating may
develop.
When the lungs are involved and the disease is untreated
or not treated quickly enough, it can cause permanent
damage to the lungs. Goodpasture's may present mildly
at first, with maybe a dry cough or minor difficulties
breathing, and may stay pretty mild for many years
before more serious symptoms develop.
The kidney involvement usually causes a form of nephritis
where the glomeruli (the small blood vessels in the
kidneys) and can rapidly progress to complete kidney
function loss. When there is rapid kidney decline,
it's called Rapidly Progressive (Crescentic) Glomerulonephritis,
or RPGN. Acute renal failure is a risk with Goodpasture's
Syndrome, as the kidney function declines and blood
spills into the urine. Typically by the time a patient
experiences symptoms they've already lost about 80%
of their kidney function. Some of the symptoms of
renal failure may include loss of appetite and a general
ill feeling (malaise) at first, then it may progress
to shortness of breath, edema and high blood pressure.
The cause of Goodpasture's is not known, but as with
any autoimmune disease the immune system mistakes
healthy cells for bad cells and responds with an attack
on the healthy tissues and cells. With this disease,
people develop substances that attack a protein called
collagen in the tiny air sacs of the lungs and the
filtering units (glomureli) of the kidney. Other triggers
thought to cause the immune response may be a viral
respiratory infection or hydrocarbon solvents. There
may also be a genetic link or predisposition to Goodpasture's.
Goodpasture's is a rare condition that affects most
commonly between the ages of 18 and 30 and is more
common in males.
Symptoms:
Blood in the urine
Burning sensation when urinating
Coughing up blood
Difficulty breathing
Fatigue
Nausea
Diagnosis:
Because Goodpasture's is a rare condition and the
symptoms are pretty vague until the disease has progressed
to late stages, diagnosis is hard. Some of the common
tests physicians may run include Chest X-rays or CT
Scans. Kidney biopsies are usually the most conclusive
and fastest way to diagnose the disease correctly.
Blood tests may also show a Positive Anti-Glomerular
Basement Membrane Antibody.
Treatment:
Goodpasture's is usually treated with immunosuppressant
therapy such as oral Cyclophosphamide to lower the
body's immune response to the antigens. Corticosteroids
may also be used as immunosuppressant and anti-inflammatory
therapies. A process called plasmapheresis may be
helpful and necessary to remove the harmful antibodies
from the blood. Unfortunately, when patients already
have kidney damage, the damage is permanent. In some
cases, dialysis may be required or a kidney transplant
may be necessary.
Prognosis:
If Goodpasture's is left undiagnosed and untreated,
lung hemorrhaging is very possible and can even cause
death, as could acute renal failure. It is very important
that a diagnosis be made as soon as possible and treatment
started before late stages of Goodpasture's develop.
With proper treatment, lung damage can usually be
reversed, but serious kidney damage may require dialysis
or eventual kidney transplant.
If the disease is diagnosed and treated early enough,
the prognosis is a little better, but in some cases,
the immunosuppressant therapy causes other complications
such as infections or secondary diseases (such as
liver damage and other organ involvement).
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