aHUS alliance Global Action came up with this table describing aHUS patients position within a matrix of triggers and mutations guiding treatment discontinuation. It was created more than 9 years ago!
It has now been revised to describe both aHUS onsetting or relapsing.
This is a less precise description of aHUS reflecting the often said ” no two aHUS patient are the same” cliche.
And this is two dimensional. There is variability in the “strength” of mutation and their locations in the complement system and elsewhere. As well as in the triggers. Some don’t apply to men and some not to children.,
But all aHUS patients are in the box.
Even those in remission or are predisposed – if only on the bottom line of the box so are currently uncertain of re-entry or entry..
Explaining incomplete penetrance.
But where do those with a no known predisposing mutation or have a secondary versions of aHUS without mutations reside ?
Well the mutations labels do not say “known” and triggers do not exclude other conditions which can cause an indirect complement TMA onset too .
Roughly 1.5 million pixels make up this image. Too many for each patient alive today but maybe not enough to include every aHUS patient that has ever been.
Whether in the box or on the side lines, all aHUS patients are in there somewhere.
Article No. 827
