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A new study suggests a close examination of the medical histories of patients with generalized pustular psoriasis can make clear which are most likely to die or be hospitalized in the ICU.
Investigators have developed a prediction model they say may help clinicians better understand which patients with generalized pustular psoriasis (GPP) are most likely to deteriorate or die from the condition.
The research identified several risk factors—particularly a history of cardiac injury—that were linked with adverse outcomes in patients with GPP.
Corresponding author Seong Jin Jo, MD, PhD, of the Seoul National University Hospital, in South Korea, and colleagues, explained that GPP presents with a mix of relapsing sterile pustules and systemic symptoms, such as fatigue.
The disease is a rare subtype of psoriasis, but it can be severe, both in terms of its impact on patient quality of life and in terms of mortality. An estimated 3-7% of patients with GPP will die from the disease, they noted.
Despite the risk associated with the disease, the authors said clinicians have very little data by which to determine which patients are at highest risk of adverse outcomes.
“Little is known about the clinical characteristics of potentially fatal GPP; only the GPP-affected elderly patients are empirically regarded as a high-risk population for mortality,” they wrote.
Jo and colleagues used a database of patients with GPP in order to find out which demographic and clinical features might be predictive of adverse outcomes.
The team used a national health insurance database to create a population-based retrospective cohort of 800 patients who were hospitalized in South Korea with GPP between January 2007 and December 2020.
The patients had a median age of 51.0 years, and a majority (56.5%) were women. About two-thirds (67.9%) of patients had previously been diagnosed with psoriasis, the most common previous diagnoses were plaque psoriasis and GPP.
The authors defined “deteriorating GPP” as cases in which a patient required intensive care unit (ICU) admission or died from their disease. Of the 800 people in the cohort, 21 required ICU admission. Seventeen patients with a median age of 75 years died from GPP.
When investigators compared the characteristics of patients with adverse outcomes to patients without deteriorating GPP, they found medical history was a significant predictor. The data showed that patients who ended up in the ICU were more likely to have renal disease, myocardial infarction history, liver disease, and diabetes.
Predictors of mortality in patients with GPP were myocardial infarction history and psoriasis history.
“Hypertension and congestive heart failure were not designated as predictors, although they were more common in deteriorating GPP cases,” Jo and colleagues said. “The prevalence of hypertension and congestive heart failure increases with age; therefore, it can be assumed that these factors were insignificant after adjusting for age.”
The investigators used these data to create a prediction model for deteriorating GPP, scoring patients 0 through 11 based on their medical history and age, with an extra point for each decade increase in age after the age of 50.
The authors said the model provides a more specific determination of patients’ risk factors, beyond simply looking at age or severity of their GPP alone.
“With no established standard treatment, supportive care is important for treating patients with GPP,” the authors concluded. “Therefore, it is crucial to predict and identify deteriorating GPP cases and intervene at an early time point, so that physicians can manage such patients as an indolent course.”
The study, "Identifying patients with deteriorating generalized pustular psoriasis: Development of a prediction model," was published online in The Journal of Dermatology.