THE MORE YOU LOOK, THE MORE YOU MAY SEE.

  • atypical Haemolytic Uraemic Syndrome (aHUS) is a rare life-threatening, complement-mediated disease 1,2

  • aHUS is characterised by thrombotic microangiopathy (TMA) and its onset can be associated with complement-amplifying conditions 1,2

    aHUS poses a high mortality risk to patients

DIAGNOSIS IS URGENT 1

COMPLEMENT-AMPLIFYING CONDITIONS (CAC)
CAN UNMASK aHUS1

patients
7 out of 10 patients with aHUS presented their first clinical manifestation while experiencing a CAC 3
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Block_2_CACs_TMA
In aHUS, a complement system defect places patients at high risk of TMA1

IF TMA PERSISTS, LOOK FURTHER 2

PREGNANCY

CAN UNMASK aHUS 1,2

  • The complement system is amplified in pregnancy and postpartum settings 4

  • In a recent study, 16% of women diagnosed with aHUS experienced TMA in association with pregnancy 4

  • 51% of women with pregnancy-
    associated aHUS experienced CKD or
    ESRD within 3 months following TMA 4


    If TMA persists, look further.
    The diagnosis may be aHUS.
    1,2
Differentiation of aHUS from other pregnancy-associated conditions is essential for optimal management 5
Block_4_Table
aHUS = atypical Haemolytic Uraemic Syndrome; HELLP = Haemolysis, Elevated Liver enzymes, and Low Platelet count;
TTP = Thrombotic Thrombocytopenic Purpura. +/– Sometimes (0–20%); + moderately frequent (20–50 %); ++ frequent (50–80%);
+++ very frequent or constant (80–100%) Adapted from Machado et al, 2012; Bergmann and Rath, 2015; Laurence 2016.

TRANSPLANT

CAN UNMASK aHUS 1,2

  • De novo TMA represents a majority of post-transplant TMA cases 8

  • Acute graft rejection, immunosuppressive drugs such as calcineurin inhibitors and genetic mutations in complement regulatory proteins can lead to de novo TMA after kidney transplant 8,9

  • The underlying diagnosis may not be obvious in aHUS patients presenting with post- transplant TMA 8

    If TMA persists, look further.
    The diagnosis may be aHUS.
    1,2
De novo TMA after kidney transplantation is a severe condition with poor graft outcome 10
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MALIGNANT HYPERTENSION

CAN UNMASK aHUS 1,2

  • Up to (44%) of patients with malignant hypertension (MHT) present with TMA 11

  • Difficulty in achieving control of hypertension and progressive renal dysfunction in patients with MHT can be clues to underlying aHUS 12

    If TMA persists, look further.
    The diagnosis may be aHUS.
    1,2

INFECTION

CAN UNMASK aHUS
IN BOTH ADULTS
AND CHILDREN 1,2

  • Infections, particularly of the respiratory and gastrointestinal tract, precede aHUS in about half of cases 2

  • Common bacterial and viral infections associated with TMA can amplify the complement system 2

    If TMA persists, look further.
    The diagnosis may be aHUS.
    1,2

PERSISTENT TMA, DESPITE CONVENTIONAL TREATMENT
WARRANTS FURTHER INVESTIGATION 2

Management algorithm for patients with CACs and TMA 2

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If you are a healthcare professional wishing to learn
more about aHUS, please get in touch with us:

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For Medical Information, please email Alexion at medicalinformation.australasia@alexion.com
For Pharmacovigilance, please email Alexion at pharmacovigilance.australasia@alexion.com