When your patient’s own complement
system turns against them1

SEE THE THREAT.
STAY AHEAD OF
THE STORM.

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You have the power to recognize atypical-HUS early.

Prompt differential diagnosis could impact the risk of organ damage, including kidney injury. 1-5

Know the cause

Atypical hemolytic uremic syndrome (atypical-HUS) is a life-threatening condition driven by terminal complement overactivation.2

  • In atypical-HUS, the risk of complement-mediated thrombotic microangiopathy (TMA) can be lifelong.1,2,6-8
  • Onset can be sudden or gradual, can occur at any age, and should be considered life-threatening.1,2,9-11

Understand the consequences

Patients with atypical-HUS may be at ongoing risk of sudden systemic, life-threatening complications.1,2

  • If undetected, atypical-HUS can progress toward irreversible tissue damage and progressive organ damage, such as end-stage renal disease.2
  • Nearly half of patients will require dialysis, suffer permanent kidney damage, or die within 1 year of first occurrence.1,9

Speak to your Alexion representative to learn more about managing patients with atypical-HUS and get answers to your questions.

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Identify the TMA

Look for this clinical triad of symptoms2

If appropriate, renal biopsy can reveal TMA3,11*

Recognize the triggers

Consider screening for TMA in your AKI patients, especially in the presence of one of the following triggers. If found during your differential diagnosis, consider atypical-HUS1-4*:

*Other triggers may include but are not limited to: pregnancy, infection, and other autoimmune disorders.2

Hypertensive emergency

TMA and atypical-HUS may be present and often overlooked in patients with malignant HTN (mHTN).15

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  • In a retrospective study of hospitalized patients with mHTN (N=199)† 20% (n=40/199) presented with TMA, and of them, 60% (n=24/40) had atypical-HUS at baseline.

†Retrospective cohort analysis of patients diagnosed with mHTN (N=199) from 2000-2020 in 8 nephrology departments in Spain.

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Systemic lupus erythematosus (SLE)

Atypical-HUS, which can be triggered by SLE, is associated with continuous risk of complement-mediated TMA and life-threatening consequences.1,2,16,17

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  • 17.5% of patients with lupus nephritis, a kidney disease caused by SLE, have been shown to develop TMA, with progressive, life-threatening thrombocytopenia, MAHA, and progressive renal failure.18,19‡

AKI=acute kidney injury; MAHA=microangiopathic hemolytic anemia; HTN=hypertension.

‡Based on a cohort study of clinical and renal histopathological data of patients with biopsy-proven lupus nephritis (N=341) diagnosed from 2000 to 2008 at a hospital in China.

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Conduct Prompt Differential Diagnosis of TMA

Urgently run key tests, including ADAMTS13, to differentiate between atypical-HUS, STEC-HUS, and TTP.2*

The following can aid your differential diagnosis of TMAs.1,2,6,20

Identification of TMA With Differential Diagnosis

Identification of TMA

Thrombocytopenia

Platelet count <150 × 109/L
or >25% decrease from baseline

AND

Microangiopathic hemolysis

Schistocytes and/or
Elevated LDH and/or
Decreased haptoglobin and/or
Decreased hemoglobin

PLUS 1 or more of the following symptoms:

Common Symptoms

Other Symptoms

Differential Diagnosis

Evaluate ADAMTS13 activity and Shiga toxin/EHEC test

≤5%-10%† ADAMTS13 activity

TTP

Shiga toxin/EHEC positive

STEC-HUS

>10% ADAMTS13 activity

Atypical-HUS

*Other tests may include, but are not limited to, a coagulation panel to rule out DIC and homocysteine, methionine, methylmalonic acid to rule out cobalamin C deficiency.1,2,21 TMA can also manifest in the presence of other clinical conditions such as HTN emergency, GN, and SLE.2

†5%-10% depending on the assay used.2

ADAMTS13=a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13; DIC=disseminated intravascular coagulation; GN=glomerulonephritis; LDH=lactate dehydrogenase; STEC-HUS=Shiga toxin-producing Escherichia coli-associated hemolytic uremic syndrome; TTP=thrombotic thrombocytopenic purpura.

Act fast: early clinical intervention is crucial to help patients with atypical-HUS improve their outcomes.1,2

Access additional highlights of the diagnostic pathway:

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Monitoring Your Patients Is Crucial for Managing TMA Recurrence in atypical-HUS

Some strategies for monitoring TMA Recurrence

LLN=lower limit of normal; sCr=serum creatinine; ULN=upper limit of normal.

Learn about the risks of TMA recurrence in this brochure.

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Stay Vigilant and Informed

You can play a key role in managing patients at risk for life-threatening complications of TMA in atypical-HUS. Doing so could impact the risks of organ damage, including kidney injury.1-5

Explore our website to learn more about atypical-HUS

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Access highlights of this page in a downloadable brochure

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Speak to your Alexion representative to learn more about managing patients with atypical-HUS. If you are unsure who your Alexion representative is, you can call 1-833-551-2539.

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