The monogenic autoinflammatory diseases define new pathways in human innate immunity and inflammation

K Manthiram, Q Zhou, I Aksentijevich, DL Kastner - Nature immunology, 2017 - nature.com
Autoinflammatory diseases were first recognized nearly 20 years ago as distinct clinical and
immunological entities caused by dysregulation in the innate immune system. Since then …

Thrombotic microangiopathy in aHUS and beyond: clinical clues from complement genetics

F Fakhouri, V Frémeaux-Bacchi - Nature Reviews Nephrology, 2021 - nature.com
Studies of complement genetics have changed the landscape of thrombotic
microangiopathies (TMAs), particularly atypical haemolytic uraemic syndrome (aHUS) …

[HTML][HTML] Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a “Kidney Disease: Improving Global Outcomes”(KDIGO) Controversies …

THJ Goodship, HT Cook, F Fakhouri, FC Fervenza… - Kidney international, 2017 - Elsevier
In both atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G)
complement plays a primary role in disease pathogenesis. Herein we report the outcome of …

Exploring the genetic basis of early-onset chronic kidney disease

A Vivante, F Hildebrandt - Nature Reviews Nephrology, 2016 - nature.com
The primary causes of chronic kidney disease (CKD) in children differ from those of CKD in
adults. In the USA the most common diagnostic groups of renal disease that manifest before …

Atypical hemolytic–uremic syndrome

M Noris, G Remuzzi - New England Journal of Medicine, 2009 - Mass Medical Soc
The hemolytic–uremic syndrome, which is characterized by nonimmune hemolytic anemia,
thrombocytopenia, and renal impairment, occurs most frequently in young children. Most …

Complement system and the kidney: its role in renal diseases, kidney transplantation and renal cell carcinoma

F Lasorsa, M Rutigliano, M Milella, M Ferro… - International Journal of …, 2023 - mdpi.com
The crosstalk among the complement system, immune cells, and mediators of inflammation
provides an efficient mechanism to protect the organism against infections and support the …

The Factor H protein family: The switchers of the complement alternative pathway

L Lucientes‐Continente… - Immunological …, 2023 - Wiley Online Library
The factor H (FH) protein family is emerging as a complex network of proteins controlling the
fate of the complement alternative pathway (AP) and dictating susceptibility to a wide range …

Atypical aHUS: state of the art

CM Nester, T Barbour, SR de Cordoba… - Molecular …, 2015 - Elsevier
Tremendous advances in our understanding of the thrombotic microangiopathies (TMAs)
have revealed distinct disease mechanisms within this heterogeneous group of diseases. As …

Horror Autoinflammaticus: The Molecular Pathophysiology of Autoinflammatory Disease

SL Masters, A Simon, I Aksentijevich… - Annual review of …, 2009 - annualreviews.org
The autoinflammatory diseases are characterized by seemingly unprovoked episodes of
inflammation, without high-titer autoantibodies or antigen-specific T cells. The concept was …

STEC-HUS, atypical HUS and TTP are all diseases of complement activation

M Noris, F Mescia, G Remuzzi - Nature Reviews Nephrology, 2012 - nature.com
Haemolytic uraemic syndrome (HUS) and thrombotic thrombocytopaenic purpura (TTP) are
diseases characterized by microvascular thrombosis, with consequent thrombocytopaenia …