aHUS patient, 25, makes full recovery with Ultomiris after virus: Report
Timely therapy resolved symptoms for man with kidney transplant, lung infection
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Starting treatment with Ultomiris (ravulizumab) led to rapid clinical improvement — with recovery of kidney function — for a young man with a history of kidney transplant who developed atypical hemolytic uremic syndrome (aHUS) after a severe viral lung infection.
According to a report detailing the case, the man’s symptoms had worsened despite being given antibiotics and antiviral medications, and clinicians were concerned about possible life-threatening complications. The care team turned to Ultomiris after noting the man’s symptoms and saw his condition improve significantly within the first 24 hours of treatment with the approved aHUS medication.
The researchers believe that an adenovirus infection — typically mild — triggered aHUS for this patient, and noted that “early recognition [of the rare condition] is essential for prompt targeted therapy.” The man ultimately made a full recovery, the team noted.
“The case contributes to the understanding of aHUS diagnosis and management, as well as raising awareness for the need for early identification of triggers, potential genetic screening, and considering the administration of complement inhibitors, as a potentially life-saving treatment,” the researchers wrote.
The case was described in “Possible Adenovirus Triggered Case of Atypical Hemolytic Uremic Syndrome in a Kidney Transplant Recipient: A Case Report and A Review of the Literature,” published in the journal Transplantation Proceedings.
aHUS is caused by abnormal activation of the complement cascade, a part of the immune system, which leads to blood clotting in small blood vessels. Such clotting is known as thrombotic microangiopathy (TMA), and is marked by red blood cell destruction, called hemolysis, low platelet levels, and organ damage. Platelets are the tiny cell fragments in blood that help it to clot.
Triggering event usually needed to develop aHUS
While most people with aHUS have inherited mutations in genes that encode proteins of the complement cascade, a triggering event such as an infection is typically necessary for developing the disease.
Here, researchers in Croatia described the case of a 25-year-old man with such mutations who developed aHUS after a severe viral infection. The man, who had a history of chronic kidney failure and had undergone a kidney transplant, was admitted to the hospital with respiratory symptoms, fever, and generalized weakness.
At the time, he was taking several immunosuppressant drugs and medications to lower blood pressure and prevent kidney complications, the researchers noted.
At the first evaluation, the man had high blood pressure and normal breathing rates and blood oxygen levels. Laboratory findings indicated low hemoglobin, the oxygen-carrying protein in the blood, and elevated lactate dehydrogenase levels consistent with hemolysis.
The patient also had low platelet counts and low levels of white blood cells. Levels of creatinine, urea nitrogen and estimated glomerular filtration rate (eGFR) indicated low kidney function.
C-reactive protein, a marker of inflammation, was also markedly elevated. A chest X-ray led to a diagnosis of pneumonia in the right lung.
Treatment was started with antibiotics delivered intravenously, or directly into the bloodstream, together with an adjustment of the man’s immunosuppressant therapy. However, the patient still had a fever, his markers of inflammation increased further, the function of his kidney graft was worsening, and his blood cell levels continued to drop.
Antibiotics, antifungals, and antivirals were added to the treatment regimen. Further tests to examine the airways identified an infection by adenovirus, a virus that usually causes mild infections but can sometimes lead to serious disease.
Man discharged from hospital after 2 Ultomiris treatments
Considering the patient’s condition and symptoms, the research team concluded he likely had TMA leading to acute kidney failure, aggravated by the lung infection. The team started treatment with Ultomiris, an inhibitor of the complement pathway, together with ciprofloxacin to prevent meningococcal meningitis that may occur in people receiving the therapy.
Further results confirmed an increased activity of the complement pathway and several mutations in the CFH gene, which provides instructions for producing complement factor H, thus confirming the aHUS diagnosis.
On the first day after Ultomiris, the patient’s clinical condition improved significantly, with a drop in inflammatory markers alongside improved kidney function and gradual recovery of blood cell levels.
In the following weeks, signs of lung infection and low platelet counts reappeared, however. A second Ultomiris dose was then administered, which again improved his condition, the team noted.
Timely targeted treatment with [Ultomiris] led to the patient’s full recovery.
The man was discharged home in good general condition 34 days, or slightly longer than one month, after admission and continued taking Ultomiris every eight weeks, together with ciprofloxacin. He maintained stable kidney function, the team noted.
In the case highlights, the researchers said the adenovirus was “identified as a trigger of aHUS in [this] kidney transplant recipient,” adding in the report that “the clinical course, response to treatment, and previous medical history suggest that the [development] of aHUS in this patient was multifactorial.”
According to the team, “genetic variants and kidney transplantation with subsequent immunosuppressive therapy made the patient susceptible to the development of the disease, which was exacerbated by acute adenovirus infection.”
The researchers say this case “highlights the importance of considering this rare diagnosis in predisposed patients,” noting that “early recognition enables timely intervention, which is essential for preventing irreversible kidney damage that could be fatal.”
Here, “timely targeted treatment with [Ultomiris] led to the patient’s full recovery,” the team concluded.
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