From the Guidelines
The treatment of Hemolytic Uremic Syndrome (HUS) primarily involves supportive care, with specific interventions depending on the type and severity, and should include aggressive intravenous fluid rehydration, careful electrolyte management, and monitoring of kidney function, as recommended by the most recent guidelines 1. The management of HUS is crucial to prevent morbidity, mortality, and to improve the quality of life. For typical HUS (Shiga toxin-associated), treatment includes:
- Aggressive intravenous fluid rehydration to prevent dehydration and maintain blood pressure
- Careful electrolyte management to prevent electrolyte imbalances
- Monitoring of kidney function to detect early signs of renal injury Dialysis may be necessary for severe kidney injury, typically required in about 50-70% of cases. Blood transfusions are often needed to address anemia, with packed red blood cells given when hemoglobin drops below 7 g/dL. For atypical HUS, eculizumab (Soliris) is the standard treatment, administered intravenously at 900 mg weekly for four weeks, then 1200 mg every two weeks thereafter, as it blocks the abnormal complement activation that causes vascular damage 1. It is essential to monitor hemoglobin and platelet counts, electrolytes, and blood urea nitrogen and creatinine frequently to detect hematologic and renal function abnormalities that are early manifestations of HUS, as recommended by the 2017 Infectious Diseases Society of America clinical practice guidelines 1. Antibiotics are generally avoided in Shiga toxin-associated HUS as they may increase toxin release, and nutritional support is essential, sometimes requiring temporary parenteral nutrition. Close monitoring of neurological status, fluid balance, and organ function is crucial throughout treatment. Most children with typical HUS recover with appropriate supportive care, while atypical HUS often requires long-term complement inhibitor therapy to prevent recurrence.
From the FDA Drug Label
SOLIRIS is indicated for the treatment of patients with atypical hemolytic uremic syndrome (aHUS) to inhibit complement-mediated thrombotic microangiopathy. Limitation of Use SOLIRIS is not indicated for the treatment of patients with Shiga toxin E. coli related hemolytic uremic syndrome (STEC-HUS).
The treatment for atypical Hemolytic Uremic Syndrome (aHUS) is eculizumab (SOLIRIS).
- The dosage regimen for aHUS is as follows:
- For patients 18 years of age and older: 900 mg weekly for the first 4 weeks, followed by 1200 mg for the fifth dose 1 week later, then 1200 mg every 2 weeks thereafter.
- For patients less than 18 years of age, the dosage is based on body weight. However, SOLIRIS is not indicated for the treatment of patients with Shiga toxin E. coli related hemolytic uremic syndrome (STEC-HUS), which is another type of Hemolytic Uremic Syndrome (HUS) 2 2.
From the Research
Treatment Options for Hemolytic Uremic Syndrome (HUS)
- The treatment for HUS depends on the underlying cause, with different approaches for typical and atypical HUS 3.
- For atypical HUS, treatment with eculizumab, a terminal complement inhibitor, has shown promising results in improving kidney function and reducing the risk of end-stage renal disease 4, 5, 6.
- Plasma exchange or plasma infusion (PE/PI) is also used as a treatment for aHUS, but eculizumab has been shown to be more effective in some cases 4, 6.
- Supportive care, including dialysis, is still the main treatment for typical HUS, which is often caused by Shiga toxin-producing E. coli infection 7, 3.
Eculizumab Treatment
- Eculizumab has been shown to improve kidney function and reduce the risk of end-stage renal disease in patients with aHUS 4, 6.
- The treatment should be started immediately after diagnosis, and the duration of treatment is still being investigated 5.
- Eculizumab has been shown to be effective in patients with aHUS who are resistant to PE/PI treatment 6.
Plasma Exchange and Plasma Infusion
- PE/PI is still used as a treatment for aHUS, but its effectiveness is limited compared to eculizumab 4, 6.
- PE/PI should be performed daily until the platelet count, lactate dehydrogenase (LDH), and hemoglobin levels are substantially improved, or until an alternate treatment strategy has been decided upon 5.