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PostPosted: 28 Feb 2020 22:39 
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OVERVIEW
• Goodpasture syndrome is also referred to as anti-glomerular basement membrane (anti-GBM) disease first identified by Dr Ernest Goodpasture in 1919
• Rare but potentially life threatening condition involving the lungs and kidneys; some forms of the disease may involve either the lungs or the kidneys
Autoimmune disease wherein the body produces antibodies that attack its own tissues, a protein called collagen in the tiny air sacs in the lungs and the filtering units (glomeruli) of the kidneys.
• Unless promptly diagnosed, it may result in severe hemorrhage into the lungs, kidney failure, and even death.
• More common in men
• Occurs more commonly between 20-30 years and persons over 60 years of age

ETIOLOGY /RISK FACTORS
• Not clear but following risk factors have been shown to increase the risk
• Exposure to tobacco smoke, hydrocarbon fumes, metallic dust, or certain drugs, such as cocaine
• Genetic susceptibility (persons with HLA-DR15)

PATHOLOGY OF GOODPASTURE SYNDROME
Persons at high risk like those mentioned above develop circulating antibodies that bind with antigenic proteins in the kidneys and lungs leading to an inflammatory response causing symptoms and signs of the disease

CLINICAL FEATURES OF GOODPASTURE SYNDROME
Non-specific symptoms
• fatigue, weakness, or lethargy
• nausea or vomiting
• loss of appetite
• unhealthy, pale appearance
Respiratory symptoms
• Dry cough
• Breathlessness
• Coughing up blood (hemoptysis)
Renal symptoms
• Blood in urine
• Burning micturition
• High blood pressure
• Swelling of hands and feet
• Pain in the loins

COMPLICATIONS OF GOODPASTURE SYNDROME
• Chronic kidney disease
• End stage renal disease
• Lung failure
• Fatal pulmonary hemorrhage

DIAGNOSIS OF GOODPASTURE SYNROME
• History of hemoptysis
• Physical examination – High blood pressure and swelling of hands and feet can raise suspicion of renal disease
• Blood tests to demonstrate anti-GBM antibodies
• Renal function tests – Elevated BUN and serum creatinine and reduced creatinine clearance
• Chest x-ray or CT scan may show evidence of bleeding and damage to lungs
Renal biopsy – If blood tests are negative, a renal biopsy may be done to confirm the diagnosis. The histological appearance is that of rapidly progressive focal segmental necrotizing glomerulonephritis with formation of crescents. Immunofluorescent staining of lung or kidney tissue demonstrates shows linear IgG deposition along the glomerular or alveolar capillaries

TREATMENT OF GOODPASTURE SYNDROME
The various forms of treatment include
• Plasmapheresis a procedure that removes harmful antibodies from the blood that are responsible for the symptoms of the disease
• Corticosteroid medicines (such as prednisone) and other medications to suppress the activity of the immune system
• Angiotensin-converting enzyme (ACE) inhibitors and angiotensin receptor blockers (ARBs) to control elevated blood pressure
• Dialysis in patients with chronic renal failure
• A kidney transplant for patients with end stage renal failure

PROGNOSIS
Early diagnosis is key. The prognosis is much poor diagnosis is delayed and there is severe damage to the lungs and kidneys. Most patients require dialysis or a renal transplant


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