Symptoms of Anxiety, PTSD Present Years After Acute iTTP, aHUS Episodes

New data underscore the long-term implications of immune-mediated thrombotic thrombocytopenic purpura (iTTP) and atypical hemolytic-uremic syndrome (aHUS), both rare diseases.

People who suffer from thrombotic microangiopathies (TMAs) often experience mental health challenges and lower quality of life, even years into remission, a new study has found.

The report, published in Journal of Critical Care, showed half of people with immune-mediated thrombotic thrombocytopenic purpura (iTTP) and atypical hemolytic-uremic syndrome (aHUS) recounted symptoms of anxiety, and a quarter of patients experienced symptoms of post-traumatic stress disorder (PTSD).

Historically, TMAs were most commonly fatal, but novel therapeutic options have created a new paradigm in which 80% to 90% of patients survive, noted the study authors.

“As a result, long-term data in survivors have emerged, showing a substantial burden of residual disease, including mental health impairments that may be difficult to detect,” they wrote.

However, although there is substantial evidence that mental health can be affected by the rare diseases, less is known about how prevalent mental health challenges are in this patient population or how such challenges affect patients’ quality of life (QOL).

To better understand the mental health implications of iTTP and aHUS, the investigators decided to conduct telephone interviews with patients 72 months after their discharge from the intensive care unit. The goal was to measure symptoms of anxiety, depression, and PTSD, and gauge participants’ QOL using the 36-item Short Form Questionnaire (SF-36).

The researchers were able to interview 103 patients, of whom 52 had iTTP and 51 had aHUS; 74% were female. The investigators found that 50% of the interviewees showed symptoms of anxiety, 27% had symptoms of PTSD, and 14% had symptoms of depression.

Those rates were generally similar between the 2 disease cohorts, the authors said. However, significant differences in outcomes were seen based on other factors.

For instance, people who reported PTSD symptoms were more likely to experience weight gain, report feeling less well both emotionally and physically, and to have anxiety and depression symptoms. People with aHUS with PTSD also had significantly greater QOL impairment compared with people with iTTP, regardless of whether they had PTSD, the authors found.

However, treatment also appeared to make a significant difference, the authors found. Half of the patients with aHUS were taking eculizumab (Soliris), and those patients had significantly better QOL than their peers who were not taking the therapy.

The investigators also found other predictive factors.

“Low platelet count during the index TMA episode and current TMA therapy showed independent and positive associations with having PTSD symptoms, whereas male sex was protective,” they wrote.

Other factors did not appear to be associated with mental health outcomes. For instance, the amount of time since the acute episode did not affect the prevalence or severity of mental health systems, according to the data, nor did the existence or number of relapses a patient experienced, the authors said. Finally, the presence of cardiac or neurologic signs during the acute episode did not appear to correlate with PTSD symptoms.

The authors said their study was subject to several limitations. For one, they excluded patients with iTTP who were taking caplacizumab (Cablivi), since the drug was not yet available at the start of the study period. The therapy has since become the first-line therapy for iTTP, and the authors said it has been associated with better QOL results.

In addition, the authors cautioned that their study was designed only to identify symptoms of mental health disorders, and thus does not reflect actual diagnoses. They said the prevalence of mental health symptoms, however, is enough to warrant further research and vigilant screening.

“In clinical practice, routine evaluations for mental health symptoms, including those suggesting PTSD, seem warranted in the hope that very early referral and treatment might improve patient outcomes,” they concluded.


Azoulay E, Souppart V, Kentish-Barnes N, et al. Post-traumatic stress disorder and quality of life alterations in survivors of immune-mediated thrombotic thrombocytopenic purpura and atypical hemolytic and uremic syndrome. Published online March 15, 2023. J Crit Care. doi:10.1016/j.jcrc.2023.154283

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