Outcomes of Purpura FULminans in Adults - The hOPeFUL Study

Study Overview
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Brief Summary
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Purpura fulminans (PF) is a rare life-threatening infectious disease characterized by the association of a sudden and extensive purpura together with acute circulatory failure. The mortality of PF has been reported to be as high as 50% in previous adult series. Additionally, patients surviving to the early phase of PF are exposed to a high risk of limb amputation. The hOPeFUL study aims at assessing the short and long term outcomes of adult patients admitted in the intensive care unit for a purpura fulminans.

Detailed Description
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Purpura fulminans (PF) is a rare life-threatening infectious disease characterized by the association of a sudden and extensive purpura together with acute circulatory failure. Neisseria meningitidis and Streptococcus pneumoniae are the most frequently involved microorganisms but other species (e.g., Staphylococcus aureus, Streptococcus pyogenes, Haemophilus influenzae…) have also been reported. Despite prompt antibiotics administration and intensive care management, the mortality of PF has been reported to be as high as 50% in previous adult series. Additionally, patients surviving to the early phase of PF are exposed to a high risk of serious sequelae related to extensive skin necrosis and acral symmetrical gangrene, which typically requires limb amputations, a potential source of severe handicap in these previously young and healthy patients. Although the clinical features and outcomes of PF have been well studied in the pediatric setting, the amount of available data for adult PF are scarce, often outdated and mainly limited to patients with meningococcal infections. The current study aims at assessing the short and long-term outcomes of adult patients admitted in the intensive care unit for a purpura fulminans. A large multicenter retrospective cohort will be built in order to assess hospital outcomes (i.e., mortality and amputations). Long-term outcomes, including health-related quality variables, will be prospectively assessed among survivors and compared to septic controls (i.e., patients having septic shock non-related to purpura fulminans).

Official Title
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Short and Long-term Outcomes of Purpura Fulminans in Adults

Conditions
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Purpura Fulminans

Intervention / Treatment
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* Other: Phone interview measuring health-related quality of life variables


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Patients admitted in the intensive care unit between 2010 and 2016 for an infectious purpura fulminans and discharged alive Exclusion Criteria: Age < 18 years Non infectious purpura Infective endocarditis

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Exposed to purpura fulminans<br>Patients who were admitted in the intensive care unit and survived a purpura fulminans episode | Other: Phone interview measuring health-related quality of life variables<br>* A phone interview will be conducted by a dedicated nurse who will be blinded to the exposed/non-exposed status of the patient.<br>|
| Non-exposed to purpura fulminans<br>Patients admitted in the intensive care unit for a septic shock unrelated to a purpura fulminans, and matched to exposed patients for age, gender, and severity of illness. | Other: Phone interview measuring health-related quality of life variables<br>* A phone interview will be conducted by a dedicated nurse who will be blinded to the exposed/non-exposed status of the patient.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Hospital mortality | factors associated with hospital mortality (and rate of amputation in the hospital) will be identified using a polytomous logistic regression model | day 60 | 
| Rate of amputation in the hospital; | factors associated with rate of amputation in the hospital (and hospital mortality) will be identified using a polytomous logistic regression model | day 60 | 
| Long-term outcome measure: Physical dimension scale of the SF-36 questionnaire | This variable will be compared between patients with purpura fulminans and patients with septic shock non-related to purpura fulminans | Within 6 years of hospital discharge | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Mental component score of the SF-36 score | This variable will be compared between patients with purpura fulminans and patients with septic shock non-related to purpura fulminans | Within 6 years of hospital discharge | 
| Activity of daily living (ADL score) | This variable will be compared between patients with purpura fulminans and patients with septic shock non-related to purpura fulminans | Within 6 years of hospital discharge | 
| Hospital anxiety and depression scale | This variable will be compared between patients with purpura fulminans and patients with septic shock non-related to purpura fulminans | Within 6 years of hospital discharge | 
| Impact of Event Scale | This variable will be compared between patients with purpura fulminans and patients with septic shock non-related to purpura fulminans | Within 6 years of hospital discharge | 
| Physical dimension scale of the SF-36 questionnaire | This variable will be compared between patients with purpura fulminans who were and those who were not amputated | Within 6 years of hospital discharge | 
| Mental component score of the SF-36 score | This variable will be compared between patients with purpura fulminans who were and those who were not amputated | Within 6 years of hospital discharge | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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health-related quality of life