Article No. 444
12 July 2021
Not unexpectedly, as aHUS Global Action had postulated it in March last year, a COVID 19 infection can trigger aHUS in those predisposed to it.
In a recent article in Kidney International Reports, doctors from Hanover in Germany presented two case studies of patients who both had COVID 19 infections and which subsequently led to an aHUS onset.
The full article can seen HERE.
Both patients were female, one 22 years old ,the other 56. The first presented to a primary care practitioner with diarrhoea and vomitting plus loss of taste and the second with flu-like symptoms, loss of taste and exhaustion.
After a further four days the 22 year old visited an emergency unit at a hospital with paleness and in acute distress. It was a further 10 days before the second patient returned to her doctor as the antibiotics that she had been prescribed had not worked.
In both patients blood tests revealed low haemoglobin, high LDH and low or lower platelets as well as acute kidney injury. Both had tested positive for a COVID 19 infection.
Both had kidney biopsies to reveal, or confirm, a suspected TMA. Both needed acute haemodialysis.
The younger patient was treated with plasma exchange until an ADAMTS 13 test showed a high level, at which point treatment switched to eculizumab with an aHUS. diagnosis . It took ten days for her blood tests to normalise and further time for her dialysis to be tapered and stopped. A genetic test revealed a likely causative novel mutation in her CFH.
In the older patient eculizumab treatment begun after TTP was ruled out and within fourteen days her blood results had returned to normal ( albeit usually she had higher than normal platelet levels) but her kidney function took longer, 2 months, to resolve. The patient was found to have a novel aHUS predisposing CFH variant, which results in a CFH deficiency.
Both remain on eculizumab treatment.
The diagnosis timeline for the younger patient was much less than the elder.
Complement activation is believed to be involved in the COVID 19 disease process. It can be seen in these two case studies it can also lead to an equally life threatening event, aHUS, even when only mildly symptomatic ,or even resolved, COVID 19 has been present.
The authors of the article conclude “Our cases suggest that patients with inherent complement defects may be at risk to relapse or present with a first episode of aHUS when infected withSARS-CoV-2.” The most emphatic statement yet that COVID 19 triggers aHUS.
aHUS patients in remission ( see Prof Fadi Fakhouri et al’s case study report paper HERE ) as well as aHUS families who know that a predisposing variant exists need to remain vigilant, and consider vaccination.
If anyone who is reading this has a story about their aHUS being triggered by COVID 19 , Global Action would like to hear about it.
