GOODPASTU

RE SYNDRO

MEPresented By:

Bijay Mandal

DEFINITION OF GOODPASTURE

SYNDROME

Goodpasture syndrome is an

uncommon autoimmune disease in

which kidney and lung injury are

caused by circulating

autoantibodies against the

noncollagenous domain of the α3

chain of collagen IV

CAUSES:The antibodies initiate

inflammatory destruction of

the basement membrane in

renal glomeruli and pulmonary

alveoli, giving rise to

proliferative, usually rapidly

progressive

glomerulonephritis and a

necrotizing hemorrhagic

interstitial pneumonitis.

A U TO A N T I B O D I E S A G A I N S T C O L L A G E N I V I N T H E B A S E M E N T  

Most cases occur in the teens or

20s, and in contrast to many other

autoimmune diseases, there is a

male preponderance. In one study

89% of patients were active

smokers

PATHOGENESIS:

The trigger that initiates the anti–

basement membrane antibodies is still

unknown. Since the epitopes that evoke

anti-collagen antibodies are normally

hidden within the molecule, it is presumed

that some environmental insult such as

viral infection, exposure to hydrocarbon

solvents (used in the dry cleaning

industry), or smoking is required to

unmask the cryptic epitopes

MORPHOLOGY:In the classic case, the lungs are heavy, with

areas of red-brown consolidation. Histologically,

there is focal necrosis of alveolar walls

associated with intra-alveolar hemorrhages.

Often the alveoli contain hemosiderin-laden

macrophages.

In later stages there may be fibrous thickening

of the septae, hypertrophy of type II

pneumocytes, and organization of blood in

alveolar spaces. In many cases

immunofluorescence studies reveal linear

deposits of immunoglobulins along the

basement membranes of the septal walls

DIAGNOSIS: A sputum test will be done to look for specific

antibodies.

A chest x raywill be done to assess the amount of fluid

in the lung tissues.

A lung needle biopsy and a kidney biopsy will show

immune system deposits.

The kidney biopsy can also show the presence of the

harmful antibodies that attack the lungs and kidneys.

  P U L M O N A R Y H E M O R R H A G E S I N A C H I L D W I T H   G O O D PA S T U R E ' S S Y N D R O M E .

CLINICAL FEATURES:

Most cases begin clinically with respiratory

symptoms, principally hemoptysis, and

radiographic evidence of focal pulmonary

consolidations. Soon, manifestations of

glomerulonephritis appear, leading to

rapidly progressive renal failure.

The most common cause of death is

uremia.

TREATMENTS:A procedure whereby blood plasma, which

contains antibodies, is removed from the body

and replaced with fluids or donated plasma (

plasmapheresis) may be performed daily for two

or more weeks to remove circulating antibodies.

It is fairly effective in slowing or reversing the

disorder.

Dialysis to clean the blood of wastes may be

required if kidney function is poor. A kidney

transplant may be successful, especially if

performed after circulating antibodies have

been absent for several months

TREATMENTS:The once dismal prognosis for this disease has

been markedly improved by intensive

plasmapheresis.

This procedure is thought to be beneficial by

removing circulating anti–basement membrane

antibodies as well as chemical mediators of

immunological injury.

Simultaneous immunosuppressive therapy

inhibits further antibody production, ameliorating

both lung hemorrhage and glomerulonephritis.

PROGNOSIS:

The probable outcome is variable. Most

cases progress to severe renal failure and

end-stage renal disease within months. Early

diagnosis and treatment makes the probable

outcome more favorable

PREVENTION:

No known prevention of Goodpasture's syndrome

exists.

People should avoid glue sniffing and the siphoning

gasoline. Stopping smoking, if a family history of renal

failure exists, may prevent some cases.

Early diagnosis and treatment may slow progression

of the disorder

ID IOPATHIC PULMONARY HEMOSIDEROSIS :

Idiopathic pulmonary hemosiderosis is

a rare disorder characterized by

intermittent, diffuse alveolar

hemorrhage.

Most cases occur in young children,

although the disease has been reported

in adults as well.

PATHOGENESIS:The cause and pathogenesis are unknown, and no

anti–basement membrane antibodies are detectable

in serum or tissues.

However, favorable response to long-term

immunosuppression with prednisone and/or

azathioprine indicates that an immunological

mechanism could be involved in the pulmonary

capillary damage underlying alveolar bleeding.

In addition, long-term follow-up of patients shows

that some of them develop other immune disorders

SYMPTOMS:

It usually presents with an

insidious onset of productive cough,

hemoptysis, anemia, and weight

loss associated with diffuse

pulmonary infiltrations similar to

Goodpasture syndrome.

WEGENER GRANULOMATOSIS:

This autoimmune disease most

often involves the upper

respiratory tract and/or the

lungs, with hemoptysis being the

common presenting symptom

DIAGNOSIS:

it is enough to emphasize that a

transbronchial lung biopsy might

provide the only tissue available

for diagnosis.

Since the amount of tissue is

small, necrosis and

granulomatous vasculitis might

not be present

Rather, the diagnostically important

features are capillaritis and

scattered, poorly formed granulomas

(unlike those of sarcoidosis, which

are rounded and well-defined).