The National Kidney Foundation (NKF) has convened a multidisciplinary group of experts to reevaluate the current classification of atypical hemolytic uremic syndrome (aHUS) and propose a roadmap to prepare the field for revising the nomenclature. Drs. Carla Nester and Marina Vivarelli, leaders of the initiative, invited Anuja Java, MD, Assistant Professor of Medicine, Washington University Division of Nephrology, to co-chair one of the working groups of the committee.
Dr. Java explains why the need to update aHUS nomenclature is mandatory: “The term aHUS is misleading. It is a condition that causes a thrombotic microangiopathy (TMA) – an umbrella term used to describe vascular damage and thrombosis. TMA can occur in several conditions. The underlying cause for TMA in aHUS is a defect in one of the components of our immune system called the complement system. The term ‘aHUS’ was coined when the etiology of the disease had not been identified and because it was not ‘typical’ HUS, which was commonly known to be associated with certain infections. Moreover, patients with aHUS do not always have hemolysis and almost never uremic.”
Revising the aHUS nomenclature, in which etiology-based approaches to diagnosis are implemented, will facilitate earlier diagnosis, management, and improved outcomes for aHUS. Diagnosis based on underlying disease mechanisms will facilitate the timely use of targeted therapeutic approaches and be both clinically and fiscally beneficial.
Eliminating the bundling of aHUS syndromes with disparate etiologies will facilitate research efforts by separating diseases into targetable mechanisms. This, in turn, will pave the way for a strategic approach to develop disease-modifying treatments.
While there is a strong need and rationale to change the nomenclature around aHUS, it is acknowledged that the change must proceed incrementally. The Expert Opinion Paper put together by this group will prepare the field for the long-term goal of changing the aHUS nomenclature and will determine the mechanisms by which such a change can be achieved.
In addition to helping update the nomenclature for aHUS, Java has been invited to join the Show Me MO CKDIntercept™ Stakeholders Meeting Dissemination Working Group. This is an extremely important initiative investigating how to improve early diagnosis and management of chronic kidney disease (CKD). High-risk populations in Missouri will be a priority focus. Nine out of ten of the estimated 635,000 adults in Missouri with CKD are not aware that their kidneys are impaired. Communities of color are disproportionately impacted by CKD.
As part of the committee, Java will help review and analyze data, develop a strategy, and provide an outline and methods for Show Me MO CKDIntercept. The goal of the initiative is to ensure that everyone living with CKD receives a timely diagnosis, high quality care, and the information necessary to make informed healthcare choices.
“I am thrilled and honored to be part of these initiative,” says Java. “Working and learning from other experts who share the same passion for the field has been an incredible experience. Revising the aHUS nomenclature is a huge undertaking but one that is necessary and has been long overdue. With the insightful discussions ongoing in the committee, we have taken the first step towards that goal.
“As for the CKD committee, our work has just begun. This is a unique initiative that is going to have an enormous impact on the care of our kidney patients, and we hope that other states will use our example and follow suit.”