ACR Meeting Abstracts

ACR Meeting Abstracts

  • Meetings
    • ACR Convergence 2024
    • ACR Convergence 2023
    • 2023 ACR/ARP PRSYM
    • ACR Convergence 2022
    • ACR Convergence 2021
    • ACR Convergence 2020
    • 2020 ACR/ARP PRSYM
    • 2019 ACR/ARP Annual Meeting
    • 2018-2009 Meetings
    • Download Abstracts
  • Keyword Index
  • Advanced Search
  • Your Favorites
    • Favorites
    • Login
    • View and print all favorites
    • Clear all your favorites
  • ACR Meetings

Abstract Number: 104

Patients Perspectives on Living with a Systemic Autoinflammatory Disease: Impact on Quality of Life

Mariana Correia Marques1, Nicole Tennermann 2, Sivia Lapidus 3, Grant Schulert 4, Jennifer Tousseau 2, Rashmi Sinha 5, Karen Durrant 6, Saskya Angevare 7 and Fatma Dedeoglu 8, 1Boston Children's Hospital, Boston, Massachusetts, 2, 3The Joseph M. Sanzari Children's Hospital, Hackensack Meridian Health, Maplewood, 4Cincinnati Children's Hospital Medical Center and University of Cincinnati College of Medicine, Cincinnati, 5Systemic JIA Foundation, Cincinnati, 6Autoinflammatory Alliance, San Francisco, 7Amersfoort, Netherlands, 8Boston Children's Hospital, Boston

Meeting: 2020 Pediatric Rheumatology Symposium

Keywords: Autoinflammation, Autoinflammatory Disease, patient-reported outcome measures, quality of life

  • Tweet
  • Click to email a link to a friend (Opens in new window) Email
  • Click to print (Opens in new window) Print
Session Information

The 2020 Pediatric Rheumatology Symposium, originally scheduled for April 29 – May 2, was postponed due to COVID-19; therefore, abstracts were not presented as scheduled.

Date: Friday, May 1, 2020

Title: Poster Session 2

Session Type: ACR Abstract Session

Session Time: 5:00PM-6:00PM

Background/Purpose: Systemic autoinflammatory diseases (SAIDs) encompass multiple clinical entities in which spontaneous inflammation occurs due to dysregulation of the innate immune response.  The variability in presentation, rarity in occurrence, and mimicking features to other conditions can frequently lead to a diagnostic delay with potentially, uncontrolled inflammation. Due to their unpredictable course, we hypothesized that SAIDs have a negative impact on quality of life (QOL).
We aimed to investigate the patient-reported factors underlying this negative impact.

Methods: A self-reported 25 question online survey on QOL of patients with SAIDs was developed by the non-profit organizations, the Autoinflammatory Alliance, KAISZ/VAISZ, ENCA and SJIA Foundation in English. Respondents were recruited by convenience sampling through online social media posts. Data on triggers, medications, family history, and correlation of symptoms with labs were collected in addition to detailed information on QOL both during and in-between flares.

Results: Between 2017 and 2019, there were 347 responses. Demographics are in table 1. The most commonly reported diagnosis was undifferentiated SAID. Seventy percent of patients were diagnosed by a rheumatologist. Delay in diagnosis was common, at up to 5-20 years for 40% of patients. More than half of the respondents saw at least 3-8 specialists before receiving their final diagnosis. In addition to common SAID features occurring in SAID patients, such as  fever (79%), rash (60%), abdominal pain (70%), oral ulcers (50%);  pain and fatigue affected 80% and 87%, respectively. Half of the patients rated being “severely limited” during flares and “somewhat limited” in-between flares. 80% felt their condition affected their studies, job, and career.

After an analysis of open-ended responses, we categorized the impact into different domains of: Physical Impact (lack of understanding of the disease amongst the medical community and community at large, delays in diagnosis, unpredictable symptomatology, and unknown long term side effects of medications), Emotional Impact (feelings of anxiety, hopelessness and frustration, feeling doubted about disease severity, constant worry about flares), Social Impact (inability to make plans for vacations/social events because of the unpredictability of symptoms, which leads to isolation, dependence on others), Financial Impact (insurance not covering specialists/medications, inability to work).

Conclusion: Our study emphasizes the importance of patient engagement in designing survey questions to better capture the impact of a disease on all aspects of life. In addition to the well-known negative impact of chronic diseases on QOL, the unpredictable nature of the course of SAIDs magnifies the stress of daily living for patients and caretakers. Further studies with more granular questionnaires paired with clinical and biomarker analyses are needed to identify specific vulnerabilities and risk factors so that preventive measures can be implemented to improve QOL of patients with SAIDs.


Disclosure: M. Correia Marques, None; N. Tennermann, None; S. Lapidus, None; G. Schulert, Novartis, 1, SOBI, 1; J. Tousseau, None; R. Sinha, None; K. Durrant, None; S. Angevare, None; F. Dedeoglu, Novartis, 1, UptoDate, 1.

To cite this abstract in AMA style:

Correia Marques M, Tennermann N, Lapidus S, Schulert G, Tousseau J, Sinha R, Durrant K, Angevare S, Dedeoglu F. Patients Perspectives on Living with a Systemic Autoinflammatory Disease: Impact on Quality of Life [abstract]. Arthritis Rheumatol. 2020; 72 (suppl 4). https://acrabstracts.org/abstract/patients-perspectives-on-living-with-a-systemic-autoinflammatory-disease-impact-on-quality-of-life/. Accessed .
  • Tweet
  • Click to email a link to a friend (Opens in new window) Email
  • Click to print (Opens in new window) Print

« Back to 2020 Pediatric Rheumatology Symposium

ACR Meeting Abstracts - https://acrabstracts.org/abstract/patients-perspectives-on-living-with-a-systemic-autoinflammatory-disease-impact-on-quality-of-life/

Advanced Search

Your Favorites

You can save and print a list of your favorite abstracts during your browser session by clicking the “Favorite” button at the bottom of any abstract. View your favorites »

All abstracts accepted to ACR Convergence are under media embargo once the ACR has notified presenters of their abstract’s acceptance. They may be presented at other meetings or published as manuscripts after this time but should not be discussed in non-scholarly venues or outlets. The following embargo policies are strictly enforced by the ACR.

Accepted abstracts are made available to the public online in advance of the meeting and are published in a special online supplement of our scientific journal, Arthritis & Rheumatology. Information contained in those abstracts may not be released until the abstracts appear online. In an exception to the media embargo, academic institutions, private organizations, and companies with products whose value may be influenced by information contained in an abstract may issue a press release to coincide with the availability of an ACR abstract on the ACR website. However, the ACR continues to require that information that goes beyond that contained in the abstract (e.g., discussion of the abstract done as part of editorial news coverage) is under media embargo until 10:00 AM ET on November 14, 2024. Journalists with access to embargoed information cannot release articles or editorial news coverage before this time. Editorial news coverage is considered original articles/videos developed by employed journalists to report facts, commentary, and subject matter expert quotes in a narrative form using a variety of sources (e.g., research, announcements, press releases, events, etc.).

Violation of this policy may result in the abstract being withdrawn from the meeting and other measures deemed appropriate. Authors are responsible for notifying colleagues, institutions, communications firms, and all other stakeholders related to the development or promotion of the abstract about this policy. If you have questions about the ACR abstract embargo policy, please contact ACR abstracts staff at [email protected].

Wiley

  • Online Journal
  • Privacy Policy
  • Permissions Policies
  • Cookie Preferences

© Copyright 2025 American College of Rheumatology