Goodpasture's syndrome is a rare condition characterized by glomerulonephritis and bleeding in the lungs. Although many diseases can present with these symptoms, the name Goodpasture's syndrome is usually reserved for autoimmune disease triggered when the patient's immune system attacks the Goodpasture antigen (hypersensitivity reaction type II), which is found in the kidneys and lungs, and in time, causing damage to the organ these organs. This disease bears the name of the American pathologist Dr. Ernest Goodpasture, 1919 description of which is considered as the first report about the existence of the condition.
Primary abnormalities: diffuse alveolar haemorrhage (plus gremorulus) due to reaction with antigen in the basal membrane GMB antibody (anti-IgG).
I. DIAGNOSIS
A. Complaint Main
* Weak
* Nausea, vomiting
* hemoptysis, no massive
* Hematuri
* Chills
B. Signs Important
* Especially young men (30-40 yrs)
* wet rhonchi in both lungs.
C. Laboratory examination
* Radoimunoesai: there are anti-GMB
* pulmonary physiology: hipoksemi and hipokapni, PCO? down
* skin test
D. Radiology
* infiltrates or pulmonary nodule in the field below:
o Acute: shadow billion granular on the lower lung field
o Chronic: description vascularization / fibrosis in the lung lobes
Pleural effusion
* Classification
* cavity
* atelectasis
E. Special Investigation -
II. KOMPLIKASI -
III .MANAGEMENT
A. General Therapy
1.Break
Triple therapy: plasmapheresis, and corticosteroids sitotoksis
2.Diet
3.Medikamentosa
First drug: -
Alternative medicine:
* Cyclophosphamide 3 mg / kg 8 weeks
* Prednisone 60 mg / day, then the tapering 15 mg / day for 1-2 months
* plasmapheresis, a minimum of 14 days, plasma volume of 3-6 liters.
B. Treatment of complications -
C. Dialysis -
IV.PROGNOSIS
Triple therapy with mortality:
- Year I: mortality 40%
- Year II: mortality 50%
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