aHUS or TMA syndromes consensus

Article No. 316

1 February 2020

The alliance has featured what are known as aHUS consensus or status publications about the state of play aHUS diagnosis and treatment. The alliance’s own Director Linda Burke has contributed to two such publications:

KDIGO Controversies Conference on Complement-Mediated Kidney Disease:  Conference Report & Conclusions:   “Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a “Kidney Disease: Improving Global Outcomes” (KDIGO) Controversies Conference.”   
Raina et al. Optimal management of atypical hemolytic uremic disease: challenges and solutions.
So there may be little novel about a recently released  publication by a group of Korean aHUS researcher with a title:
Consensus regarding the diagnosis and management of atypical haemolytic uremic syndrome.” 
But on opening its pages, it is different from others.
The lead author Prof. Doyeun Oh and his group have approached their analysis using the up and coming reclassification of aHUS into its TMA component diseases. The reclassification movement intensifies as the alliance noted it would in its series of three articles in December last year , “aHUS is its end really nigh?
A multi disciplinary team of expert Korean research clinicians, including haematologists, adult and paediatric Nephrologists, transplant surgeons, and geneticists met between March and November 2019 to get consensus on a definition of TMA and the differential diagnosis of TMA syndromes.
The study group set out each TMA type and discussed the complexity and the challenge of diagnosis. Differentiation between aHUS TMAs which are primary or secondary Is vital because of the different ways they are treated. Secondary TMA syndromes can be managed by removal of the triggering factors, whereas aHUS requires correction of complement dysregulation and removal of the triggering factors.
The triggering factors looked at included, pregnancy, malignant hypertension, kidney transplant, other solid organ and bone marrow transplants, drugs, autoimmune disorders and vasculitides, fibrinous thrombotic diseases, infections and sepsis. In each of the these resolving the trigger resolved the TMA unless the patient had complement predisposing genetic variants hampering complement control, in which case complement inhibition was also needed.
Throughout the article a set of “key messages” appear to summarise the sections. For example for this section.

Key message
• A variety of medications and clinical conditions (e.g., infection, pregnancy, malignancy, and autoimmune disease) are associated with TMA syndromes.
• TMA syndromes may coexist with aHUS and may contribute to complement system activation in patients genetically predisposed to aHUS.”
The article continues to cover genetic testing, the treatment options and challenges of cost of complement inhibitor treatment.
As with all “state of play” articles addressing a subject with the scope of aHUS requires brevity to be comprehensive. It is lengthy but simply written, and so it is understandable. It is worth looking at in order to understand the different perspectives raised by seeing things as TMA Syndromes , rather than just aHUS
Between them aHUS patient viewers of this website have had a spectrum of different experiences, TMA Syndromes allows them to identify with something specific to them, whilst still feeling a part of the collective aHUS group.


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