The KDIGO “aHUS” Guidelines have arrived

The long anticipated KDIGO Guidelines from the Controversies Conference held in Florence in September 2022 are now published.

The report can be seen in full in Kidney International at this LINK. It is lengthy at 70 pages, mainly due to references section including almost 200 articles!

To say these are aHUS guidelines understates the way in which complement is being seen these days as contributing to the cause and progress of many more kidney diseases.

These guidelines address the following kidney diseases:

  • Diabetic Nephropathy
  • Focal Segmental Glomerulosclerosis
  • IgA Nephropathy
  • IgA associated vasculitis with nephritis
  • Membranous nephropathy
  • Systemic Lupus Erythematosus (SLE)
  • Anti-phospholipid Antibody Syndrome
  • ANCA-Associated Vasculitis
  • Thrombotic microangiopathies (TMAs) Complement -mediated forms of HUS
  • C3 Glomerulopathy
  • Immune Complex Membranoproliferative Glomerulonephritis (IC-MPGN

So the complement system may be implicated in either the cause of more kidney diseases or TMA occurrence in those diseases. The controversy is how many and how much.

Controversy conferences are set up for experts to agree or disagree on what is known , what the current knowledge gaps are, and what answers need to be found. Groups were set up to do that for each of the above kidney disorders.

aHUS , if mentioned was considered in the TMAs and CM -HUS section of the Report. “If mentioned” because one of the first controversies the group addressed was the name aHUS. Pragmatically ,because another international group is determining nomenclature, the KDIGO group chose the term Complement Mediated Kidney TMA to replace primary aHUS and secondary HUS or TMAs.

The KDIGO publication is a large document. The C-M Kidney TMA ,that was aHUS, along with typical HUS and secondary versions , features from page 15 to 21 and covers the following topics:

The remit of the group and participants signatures

  • Terminology
  • Complement involvement and associated pathogenicity
  • Biomarkers
  • Genetics
  • Treatment
  • Discontinuation of treatment
  • Complement involvement in other forms of HUS

Table 1 of the Report gives a comprehensive list of current and emerging complement inhibitors and their respective trials.

Table 2 lists collective disease’s patients and caregiver concerns, unmet needs, genetic testing and screening, and views on repeat biopsies in the setting of clinical trials.

Table 3 addresses questions and research needs regarding complement involvement in kidney diseases.

What is included in the tables as far as aHUS is concerned has been featured on this website as far as treatment horizon scanning and trial watch, articulating patient and care giver concerns and a research agenda for aHUS. Global Action will look at what KDIGO now reports in further website articles.

Unless there is a major significant development there will not be another KDIGO guideline published until the 2030s so these guidelines will set the scene for current and future aHUSs (or whatever they get called ) patients health and care outcomes.

Article No 667

Previous articles on KDIGO

Improving outcomes for “aHUS” patients, I was there.

As an aHUS advocate you can often be surprised by events. Nearly three months ago I received an e mail inviting me to attend a conference about Complement Renal Disease.…


KDIGO and aHUS treatment guidelines

KDIGO and aHUS treatment guidelines

KDIGO stands for Kidney Disease: Improving Global Outcomes. It is a global nonprofit organization that develops and publishes clinical practice guidelines for the evaluation and management of kidney diseases. KDIGO…

CONTINUE READINGKDIGO and aHUS treatment guidelines

KDIGO – aHUS controversies and consensus

KDIGO – aHUS controversies and consensus

Six years ago, aHUS featured, along with another renal disease MPGN/C3G, in a controversies conference held by KDIGO (Kidney Disease Improving Global Outcomes, ) in Barcelona , Spain. Complement…


aHUS & C3G:  KDIGO Physician Reference Guide (Oct 2017)

aHUS & C3G: KDIGO Physician Reference Guide (Oct 2017)

  Among rare kidney diseases, there are common clinical and patient issues. Meeting at the KDIGO Controversies Conference Common Elements in Rare Kidney Diseases, a multidisciplinary panel of clinicians joined…


aHUS Diagnosis & Treatment – KDIGO Controversy Conference

aHUS Diagnosis & Treatment – KDIGO Controversy Conference

  The KDIGO Controversies Conference on Complement-Mediated Kidney Disease was held November 19-21 2015 in Barcelona Spain.   KDIGO’s mission is to improve global outcomes for kidney disease, to include this…


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