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Goodpasture's syndrome: A clinical update
- Source :
- Autoimmunity Reviews. 14:246-253
- Publication Year :
- 2015
- Publisher :
- Elsevier BV, 2015.
-
Abstract
- Goodpasture's syndrome (GS) is a rare and organ-specific autoimmune disease that is mediated by anti-glomerular basement membrane (anti-GBM) antibodies and has pathology characterized by crescentic glomerulonephritis with linear immunofluorescent staining for IgG on the GBM. It typically presents as acute renal failure caused by a rapidly progressive glomerulonephritis, accompanied by pulmonary hemorrhage that may be life-threatening. It was first described as a distinctive syndrome by Pasture in 1919. Autoimmune Inner Ear Disease (AIED) may be associated. The etiology of GS is unknown. Researchers hypothesized a genetic predisposition HLA-associated. Complex immunological mechanisms are in the pathogenesis. The disease is caused by autoantibodies against the NC1 domain of the alpha 3 chain of type IV collagen. The limited presence of this molecule in the body explains the interest confined to specific target organs, such as the lung and kidney. It occurs when the immune system attacks the walls of the lungs and the tiny filtering units in the kidneys. Without prompt diagnosis and treatment, the disease can lead to bleeding in the lungs, kidney failure, and even death.
- Subjects :
- Pathology
medicine.medical_specialty
Anti-Glomerular Basement Membrane Disease
Immunology
Autoantigens
Antibodies
Epitopes
Type IV collagen
Pulmonary-renal syndrome
anti-GBM antibodies
medicine
Goodpasture's syndrome
Humans
Immunology and Allergy
Goodpasture syndrome
Rapidly progressive glomerulonephritis
anti-GBM disease
goodpasture syndrome
Autoimmune disease
pulmonary-renal syndrome
business.industry
Autoimmune inner ear disease
T-Lymphocytes, Helper-Inducer
medicine.disease
crescentic glomerulonephritis
ANCAs
Pulmonary hemorrhage
business
Subjects
Details
- ISSN :
- 15689972
- Volume :
- 14
- Database :
- OpenAIRE
- Journal :
- Autoimmunity Reviews
- Accession number :
- edsair.doi.dedup.....7539f4fc18c85bbd428de4d0309951f3
- Full Text :
- https://doi.org/10.1016/j.autrev.2014.11.006