Goodpasture syndrome overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Goodpasture’s disease is a rare condition characterised by rapid destruction of the kidneys and hemorrhaging of the lungs.
Although many diseases can present with these symptoms, the name Goodpasture’s syndrome is usually reserved for the autoimmune disease produced when the patient’s immune system attacks cells presenting the Goodpasture antigen, which are found in the kidney and lung, causing damage to these organs.
Historical Perspective
Goodpasture syndrome was first discovered by Dr.Ernest William Goodpasture, an American pathologist and physician, who studied the influenza pandemic in 1919, described a fatal disease that was associated with glomerulonephritis and pulmonary hemorrhage.
Classification
There is no established system for the classification of Goodpasture syndrome.
Pathophysiology
The pathogenesis of Goodpasture syndrome includes the presence of auto-antibodies directed against the glomerular or alveolar basement membranes. As with many autoimmune conditions, the precise cause of Goodpasture’s Syndrome is not yet known. It is believed to be a type II hypersensitivity reaction to Goodpasture’s antigens on the cells of the glomeruli of the kidneys and the pulmonary alveoli, specifically the basement membrane's (including a-3 chain of type IV collagen), whereby the immune system wrongly recognizes these cells as foreign and attacks and destroys them, as it would an invading pathogen. The target antigen that has the strongest pathogenic effect on anti-GBM disease is the non-collagenous 1 domain of alpha-3 type IV collagen. There is strong correlation of anti-glomerular basement membrane disease with allele HLA DRB1-1501.This allele is associated in causing renal injury. On gross pathology, Goddpasture syndrome with lung involvement may present with diffuse pulmonary hemorrhage. On microscopic histopathological analysis, early focal proliferative changes that display necrosis and crescent formation with an inflamed interstitial are seen. Under direct immunofluorescence, linear immunoglobulin G deposits are found encompassing the glomerular basement membrane and at times the distal tubular portion of the basement membrane.
Causes
There are no known direct causes for Goodpasture syndrome. Common risk factors for Goodpasture syndrome are viral or bacterial infections and certain environmental and behavioral risk factors such as smoking, hydrocarbons, formaldehyde and cocaine use.
Differentiating Goodpasture syndrome from Other Diseases
Goodpasture syndrome must be differentiated from other diseases that cause rapid progressive glomerulonephritis and pulmonary hemorrhage. ANCA associated vasculitis, are disorders that affect the renal and pulmonary system, must be differentiated from Goodpasture syndrome.
Epidemiology and Demographics
The prevalence of Goodpasture syndrome worldwide affects is an estimated 1 per million individuals, with high prevalence in Caucasians. The peak incidence of the disease occurs between the ages of 20 and 30 and again at 60 and 70. Goodpasture syndrome affects men and women equally.
Risk Factors
Common risk factors in the development of Goodpasture syndrome may be occupational, environmental, genetic, and viral. However, we don't known what causes the antibodies to form. However, we don't known what causes the antibodies to form.
Screening
There is insufficient evidence to recommend routine screening for Goodpasture syndrome.
Natural History, Complications, and Prognosis
If left untreated, Goodpasture syndrome can progress to end stage renal disease and pulmonary failure. Complications of Goodpasture syndrome include, infections, alveolar hemorrhage, end stage renal disease, and pulmonary failure. The prognosis of Goodpasture syndrome is variable, as it depends upon the diagnosis, start of treatment and the level of serum creatinine.