ABSTRACT
Atypical hemolytic uremic syndrome (aHUS) an important form of a thrombotic microangiopathy (TMA) that can frequently lead to acute kidney injury (AKI). An important subset of aHUS is the anti-factor H associated aHUS. This variant of aHUS can occur due to deletion of the complement factor H genes, CFHR1 and CFHR3, along with the presence of anti-factor H antibodies. However, it is a point of interest to note that not all patients with anti-factor H associated aHUS have a CFHR1/R3 deletion. Factor-H has a vital role in the regulation of the complement system, specifically the alternate pathway. Therefore, dysregulation of the complement system can lead to inflammatory or autoimmune diseases. Patients with this disease respond well to treatment with plasma exchange therapy along with Eculizumab and immunosuppressant therapy. Anti-factor H antibody associated aHUS has a certain genetic predilection therefore there is focus on further advancements in the diagnosis and management of this disease. In this article we discuss the baseline characteristics of patients with anti-factor H associated aHUS, their triggers, various treatment modalities and future perspectives.
Subject(s)
Acute Kidney Injury , Atypical Hemolytic Uremic Syndrome , Complement System Proteins , Acute Kidney Injury/genetics , Acute Kidney Injury/immunology , Acute Kidney Injury/therapy , Antibodies/genetics , Antibodies/immunology , Atypical Hemolytic Uremic Syndrome/complications , Atypical Hemolytic Uremic Syndrome/genetics , Atypical Hemolytic Uremic Syndrome/immunology , Atypical Hemolytic Uremic Syndrome/therapy , Blood Proteins/genetics , Complement C3b Inactivator Proteins/genetics , Complement Factor H/antagonists & inhibitors , Complement Factor H/genetics , Complement Factor H/immunology , Complement System Proteins/genetics , Complement System Proteins/immunology , Humans , Plasma ExchangeABSTRACT
Increased mortality has been observed in patients who develop acute kidney injury (AKI) in the setting of coronavirus disease 2019 (COVID-19), which has led to the approval of extracorporeal kidney support by the FDA. We analyzed the existing literature to compare the efficacy and therapeutic benefits of various extracorporeal modalities for the oXiris membranes and CytoSorb cartridge in high-flow continuous kidney replacement therapy (HFCKRT). AKI due to COVID-19 is mediated by a state of systemic inflammation (cytokine storm syndrome), leading to multiple organ dysfunction. Although there is no consensus on a protocol for providing kidney support therapy, clinically oriented studies have shown the capacities of oXiris and CytoSorb filters to effectively filter out pro-inflammatory components, leading to improved clinical outcomes in critically ill patients. In this review, we study the development of cytokine storm syndrome, important clinical evidence regarding the roles of various adsorption techniques in kidney support therapy in this setting, and a protocol influenced by FDA recommendations for oXiris and CytoSorb membranes.