Publications by authors named "Peter J Spaeth"
Hereditary Angioedema (HAE) is a rare and disabling disease for which early diagnosis and effective therapy are critical. This revision and update of the global WAO/EAACI guideline on the diagnosis and management of HAE provides up-to-date guidance for the management of HAE. For this update and revision of the guideline, an international panel of experts reviewed the existing evidence, developed 28 recommendations, and established consensus by an online DELPHI process.
View Article and Find Full Text PDFHereditary angioedema (HAE) is a rare and disabling disease for which early diagnosis and effective therapy are critical. This revision and update of the global WAO/EAACI guideline on the diagnosis and management of HAE provides up-to-date guidance for the management of HAE. For this update and revision of the guideline, an international panel of experts reviewed the existing evidence, developed 28 recommendations, and established consensus by an online DELPHI process.
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- The neonatal Fc receptor (FcRn) helps extend the lifespan of IgG antibodies by transporting them back to the cell surface to avoid degradation, but high levels from IVIg therapy can affect this process.
- Recent findings suggest that specific genetic variations (VNTR3/2 polymorphisms) in the FcRn gene may influence how long infused IgG remains in circulation and its overall effectiveness, particularly in certain autoimmune neurological diseases.
- The review also explores the potential of new monoclonal antibodies that inhibit FcRn in treating neurological conditions, signifying a shift in treatment strategies for patients who don't respond well to traditional IVIg therapies.
The complement system consists of a network of plasma and membrane proteins that modulate tissue homeostasis and contribute to immune surveillance by interacting with the innate and adaptive immune systems. Dysregulation, impairment or inadvertent activation of complement components contribute to the pathogenesis of some autoimmune neurological disorders and could even contribute to neurodegenerative diseases. In this Review, we summarize current knowledge about the main functions of the complement pathways and the involvement of complement in neurological disorders.
View Article and Find Full Text PDFAdhesion-induced eosinophil cytolysis requires the receptor-interacting protein kinase 3 (RIPK3)-mixed lineage kinase-like (MLKL) signaling pathway, which is counterregulated by autophagy.
J Allergy Clin Immunol
December 2017
Background: Eosinophils are a subset of granulocytes that can be involved in the pathogenesis of different diseases, including allergy. Their effector functions are closely linked to their cytotoxic granule proteins. Release takes place through several different mechanisms, one of which is cytolysis, which is associated with release of intact granules, so-called clusters of free eosinophil granules.
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- * Inappropriate activation of the complement system leads to autoantibodies attacking self-tissue, suggesting that targeting this activation could provide new treatment avenues for immune-mediated inflammatory diseases.
- * Current treatments for Guillain-Barré syndrome and neuromyelitis optica can have broad immunosuppressive effects; using complement inhibitors may offer a more specific and less disruptive therapeutic strategy.