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Autoimmun Rev. 2015 Mar;14(3):246-53. doi: 10.1016/j.autrev.2014.11.006. Epub 2014 Nov 15.

Goodpasture's syndrome: a clinical update.

Author information

1
Department Organs of Sense, ENT Section, 'Sapienza' University of Rome, Viale del Policlinico 155, 00100 Roma, Italy.
2
Department of Surgical Science, 'Sapienza' University of Rome, Viale del Policlinico 155, 00100 Roma, Italy.
3
Department Organs of Sense, ENT Section, 'Sapienza' University of Rome, Viale del Policlinico 155, 00100 Roma, Italy; Department of Surgical Science, 'Sapienza' University of Rome, Viale del Policlinico 155, 00100 Roma, Italy. Electronic address: armando.devirgilio@gmail.com.
4
Department of Medico-Surgical Sciences and Biotechnologies, Otorhinolaryngology Section, 'Sapienza' University of Rome, Corso della Repubblica, 79, 04100 Latina, LT, Italy.
5
Department Organs of Sense, Audiology Section, 'Sapienza' University of Rome, Viale del Policlinico 155, 00100 Roma, Italy.

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.

KEYWORDS:

ANCAs; Anti-GBM antibodies; Anti-GBM disease; Crescentic glomerulonephritis; Goodpasture syndrome; Pulmonary-renal syndrome

PMID:
25462583
DOI:
10.1016/j.autrev.2014.11.006
[Indexed for MEDLINE]

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