Guillain-Barré syndrome: causes, immunopathogenic mechanisms and treatment.
Guillain-Barré syndrome is a rare disease representing the most frequent cause of acute flaccid symmetrical weakness of the limbs and areflexia usually reaching its peak within a month. The etiology and pathogenesis remain largely enigmatic and the syndrome results in death or severe disability in 9-17% of cases despite immunotherapy. Areas covered: In terms of etiology, Guillain-Barré syndrome is linked to Campylobacter infection but less than 0.1% of infections result in the syndrome. In terms of pathogenesis, activated macrophages and T cells and serum antibodies against gangliosides are observed but their significance is unclear. Expert commentary: Guillain-Barré syndrome is a heterogeneous condition with numerous subtypes and recent data point towards the role of ganglioside epitopes by immunohistochemical methods. Ultimately, the syndrome results from a permissive genetic background on which environmental factors, including infections, vaccination and the influence of aging, lead to disease.
a Division of Rheumatology, Allergy, and Clinical Immunology , University of California Davis , Davis , CA , USA.
Anil K
AK
Jasti
b Rheumatology and Clinical Immunology , Humanitas Research Hospital , Rozzano , Milan , Italy.|c BIOMETRA Department , University of Milan , Milan , Italy.
Carlo
C
Selmi
d Center for Autoimmune Diseases Research (CREA) , Universidad del Rosario , Bogota , Colombia.
Juan C
JC
Sarmiento-Monroy
e Intensive Care Unit, Mederi, Hospital Universitario Mayor , Universidad del Rosario , Bogotá , Colombia.
Daniel A
DA
Vega
d Center for Autoimmune Diseases Research (CREA) , Universidad del Rosario , Bogota , Colombia.
Juan-Manuel
JM
Anaya
a Division of Rheumatology, Allergy, and Clinical Immunology , University of California Davis , Davis , CA , USA.
M Eric
ME
Gershwin
D000375:Aging; D001323:Autoantibodies / Q000378:metabolism; D002169:Campylobacter Infections / Q000276:immunology* / Q000628:therapy; D016123:Campylobacter jejuni / Q000276:immunology*; D003429:Cross Reactions; D005732:Gangliosides / Q000276:immunology; D059647:Gene-Environment Interaction; D020275:Guillain-Barre Syndrome / Q000276:immunology* / Q000628:therapy; D006801:Humans; D007167:Immunotherapy / Q000379:methods*
D001323:Autoantibodies; D005732:Gangliosides
Guillain-Barré; Molecular mimicry; autoimmune neuropathy; immune tolerance; infection and immunity