Outcomes of Purpura FULminans in Adults - The hOPeFUL Study (hOPeFUL)

Short and Long-term Outcomes of Purpura Fulminans in Adults

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.

Study Overview

• Status: Completed
• Conditions: Purpura Fulminans
• Intervention / Treatment: Other: Phone interview measuring health-related quality of life variables

Detailed Description

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of PF have been well studied in the pediatric setting, the amount of available data for adult PF are scarce, often outdated and mainly limited to patients with meningococcal infections.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).

• Study Type: Observational
• Enrollment (Actual): 74

Contacts and Locations

Study Locations

• France
  • Aulnay-sous-Bois, France, 86000
    • Aulnay sous Bois Hospital
  • Caen, France, 14118
    • CAEN University Hospital
  • Chartres, France, 28000
    • Chartres Hospital
  • Colombes, France, 92700
    • Louis Mourier Hospital
  • Créteil, France, 94010
    • Henri Mondor Hospital
  • Grenoble, France, 38100
    • Grenoble University Hospital
  • La Roche sur Yon, France, 85925
    • La Roche Sur Yon Hospital
  • Le Chesnay, France, 78150
    • Versailles Hospital
  • Le Havre, France, 76600
    • Le Havre Hospital
  • Lilles, France, 59000
    • Lilles University Hospital
  • Lyon, France, 69003
    • Edouard Herriot Hospital
  • Melun, France, 77000
    • Melun Hospital
  • Nice, France, 6003
    • Nice University Hospital
  • Orléans, France, 45000
    • Orléans Hospital
  • Paris, France, 75014
    • Cochin Hospital
  • Paris, France, 75013
    • Pitie-Salpêtrière Hospital
  • Paris, France, 75010
    • Saint Louis Hospital
  • Paris, France, 75012
    • Saint-Antoine Hospital
  • Paris, France, 75018
    • Bichat University Hospital
  • Poissy, France, 78300
    • Poissy Hospital
  • Poitiers, France, 86000
    • Poitiers university hospital
  • Quincy-sous-Sénart, France, 91480
    • Clinique Gallien
  • Reims, France, 51100
    • Reims university Hospital
  • Rennes, France, 35000
    • Rennes University Hospital
  • Roubaix, France, 59100
    • Roubaix hospital
  • Tours, France, 37000
    • Tours University Hospital

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Patients admitted in the intensive care unit between 2010 and 2016 for an infectious purpura fulminans (cases/exposed patients) and discharged alive will be matched (gender, age and SAPS II score) to patients admitted during the same time frame for a septic shock non-related to purpura fulminans (controls/non-exposed pâtients).

Description

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

Study Plan

How is the study designed?

Design Details

• Observational Models: Case-Control
• Time Perspectives: Prospective

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
Exposed to purpura fulminans; 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; A phone interview will be conducted by a dedicated nurse who will be blinded to the exposed/non-exposed status of the patient.
Non-exposed to purpura fulminans; 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; A phone interview will be conducted by a dedicated nurse who will be blinded to the exposed/non-exposed status of the patient.

What is the study measuring?

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

Collaborators and Investigators

• Sponsor: Henri Mondor University Hospital
• Collaborators: Fondation de France
• Investigators: Principal Investigator: Nicolas de Prost, MD, PhD, Henri Mondor University Hospital

Publications and helpful links

General Publications

• Giraud T, Dhainaut JF, Schremmer B, Regnier B, Desjars P, Loirat P, Journois D, Lanore JJ. Adult overwhelming meningococcal purpura. A study of 35 cases, 1977-1989. Arch Intern Med. 1991 Feb;151(2):310-6.
• Lerolle N, Carlotti A, Melican K, Aubey F, Pierrot M, Diehl JL, Caille V, Hekimian G, Gandrille S, Mandet C, Bruneval P, Dumenil G, Borgel D. Assessment of the interplay between blood and skin vascular abnormalities in adult purpura fulminans. Am J Respir Crit Care Med. 2013 Sep 15;188(6):684-92. doi: 10.1164/rccm.201302-0228OC.
• Contou D, Canoui-Poitrine F, Coudroy R, Preau S, Cour M, Barbier F, Terzi N, Schnell G, Galbois A, Zafrani L, Zuber B, Ehrmann S, Gelisse E, Colling D, Schmidt M, Jaber S, Conia A, Sonneville R, Colin G, Guerin L, Roux D, Jochmans S, Kentish-Barnes N, Audureau E, Layese R, Alves A, Ouedraogo R, Brun-Buisson C, Mekontso Dessap A, de Prost N; Hopeful Study Group. Long-term Quality of Life in Adult Patients Surviving Purpura Fulminans: An Exposed-Unexposed Multicenter Cohort Study. Clin Infect Dis. 2019 Jul 2;69(2):332-340. doi: 10.1093/cid/ciy901.
• Contou D, Sonneville R, Canoui-Poitrine F, Colin G, Coudroy R, Pene F, Tadie JM, Cour M, Beduneau G, Marchalot A, Guerin L, Jochmans S, Ehrmann S, Terzi N, Preau S, Barbier F, Schnell G, Roux D, Leroy O, Pichereau C, Gelisse E, Zafrani L, Layese R, Brun-Buisson C, Mekontso Dessap A, de Prost N; Hopeful Study Group. Clinical spectrum and short-term outcome of adult patients with purpura fulminans: a French multicenter retrospective cohort study. Intensive Care Med. 2018 Sep;44(9):1502-1511. doi: 10.1007/s00134-018-5341-3. Epub 2018 Aug 20.

Study record dates

Study Major Dates

• Study Start (Actual): July 27, 2017
• Primary Completion (Actual): March 8, 2018
• Study Completion (Actual): March 8, 2018

Study Registration Dates

• First Submitted: July 7, 2017
• First Submitted That Met QC Criteria: July 12, 2017
• First Posted (Actual): July 13, 2017

Study Record Updates

• Last Update Posted (Actual): March 9, 2018
• Last Update Submitted That Met QC Criteria: March 8, 2018
• Last Verified: March 1, 2018

More Information

Terms related to this study

• Keywords: health-related quality of life
• Additional Relevant MeSH Terms: Pathologic Processes; Hematologic Diseases; Hemorrhage; Blood Coagulation Disorders; Skin Manifestations; Purpura; Purpura Fulminans
• Other Study ID Numbers: hOPeFUL

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: Undecided

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No