ACR Meeting Abstracts

ACR Meeting Abstracts

  • Home
  • Meetings Archive
    • 2023 ACR/ARP PRSYM
    • ACR Convergence 2022
    • ACR Convergence 2021
    • ACR Convergence 2020
    • 2020 ACR/ARP PRSYM
    • 2019 ACR/ARP Annual Meeting
    • 2018 ACR/ARHP Annual Meeting
    • 2017-2009 Meetings
    • Download Abstracts
  • Keyword Index
  • Advanced Search
  • Your Favorites
    • Favorites
    • Login
    • View and print all favorites
    • Clear all your favorites
  • Meeting Resource Center

Abstract Number: 108

A Multinational Study of Thrombotic Microangiopathy in Macrophage Activation Syndrome: A Dreadful Condition Which Is Likely Underrecognized

Francesca Minoia1, Jessica Tibaldi 2, Valentina Muratore 3, Romina Gallizzi 4, Claudia Bracaglia 5, Alessia Arduini 6, Elif Comak 7, Olga Vougiouka 8, Ralf Trauzeddel 9, Giovanni Filocamo 10, Concetta Micalizzi 2, Ozgur Kasapcopur 11, Erbil Unsal 12, Toshiyuki Kitoh 13, Elena Tsitsamis 14, Mikhail Kostik 15, Randy Cron 16, Jana Pachlopnik 17, Despoina Maritsi 18, Marija Jelusic 19, Susan Shenoi 20 and Angelo Ravelli 2, 1Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy, 2Istituto Giannina Gaslini, Genoa, Italy, 3IRCCS Policlinico San Matteo, Pavia, Italy, 4Università degli Studi di Messina, Messina, Italy, 5Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Italy, 6IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, 7Akdeniz University, Antalya, Turkey, 8Second Department of Paediatrics, P. & A. Kyriakou Children’s Hospital, Athens, Greece, 9Helios Klinikum, Berlin, Germany, 10Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy, 11Istanbul University-Cerrahpasa, Cerrahpasa Medical School, Cerrahpasa, Turkey, 12Dokuz Eylul University, Izmir, Turkey, 13Aichi Medical University, Nagakute, Japan, 14Children's Hospital Aghia Sophia, Athens, Greece, 15State Pediatric Medical Academy, Saint Petersburg, Russia, 16University of Alabama at Birmingham, Birmingham, 17Universitats-Kinderspital, Zurich, Switzerland, 18National and Kopdistrian University of Athens, Athens, Greece, 19Division of Paediatric Rheumatology and Immunology, University of Zagreb, Zagreb, Croatia, 20Seattle Children's Hospital, Seattle

Meeting: 2020 Pediatric Rheumatology Symposium

Keywords: macrophage activation syndrome

  • Tweet
  • Email
  • 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

Session Title: Poster Session 2

Session Type: ACR Abstract Session

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

Background/Purpose: Macrophage activation syndrome (MAS) is a severe complication of rheumatologic conditions, mainly systemic juvenile idiopathic arthritis (sJIA), and is classified among the secondary forms of hemophagocytic lymphohistiocytosis (HLH). Thrombotic microangiopathy (TMA) is a heterogeneous group of life-threating diseases characterized by microangiopathic hemolytic anemia, thrombocytopenia and organ injury. The association between TMA and HLH has been described only in single reports in adult renal transplant recipients and in two pediatric cases of virus-induced HLH. The aim of our study is to present the preliminary data from a multinational cohort of pediatric patients with MAS and TMA

Methods: The clinical charts of patients with MAS were retrospectively reviewed to identify the instances that were associated with TMA. Demographic, clinical and laboratory features at MAS and TMA onset, therapeutic interventions and outcome were collected

Results: A total of 27 patients, 66.7% females, with MAS and TMA were collected. An underlying rheumatologic disease was reported in 19/27 (14 sJIA or a sJIA-like illness, 2 systemic lupus erythematosus, 1 Kawasaki disease, 1 autoimmune hepatitis, 2 connective tissue diseases). The median age at MAS onset was 10.9 years. In 10 patients MAS and TMA occurred simultaneously, whereas in 4 TMA preceded and in 13 followed MAS. The main features at MAS and TMA onset are presented in Table 1. Low complement levels and reduced ADAMTS13 activity were observed in 70.6% and 50% of patients respectively. The parameters which significatively change in MAS patients complicated by TMA were platelet count, fibrinogen, LDH and AST. For MAS management, 92.6% of patients received high-dose corticosteroids and 59.3% cyclosporine; in 8 cases anakinra was added. TMA episodes were treated with plasma-exchange in 63% of patients; 18 patients were given biologics (6 rituximab and 12 eculizumab). Admission to the Intensive Care Unit was required in 92.6% of cases. Mortality rate was 15%.

Conclusion: The association of MAS and TMA is a dreadful complication, likely under-recognized. Clues to suspect TMA in a patient with MAS are the increase in LDH and AST and the decrease in platelet count and fibrinogen out of proportion of other laboratory abnormalities, the drop in haptoglobin level, the presence of schistocytes in blood smear and the new onset of kidney failure or hematuria. Biologics, particularly rituximab and eculizumab, may offer an adjunctive therapeutic option for refractory cases


Disclosure: F. Minoia, None; J. Tibaldi, None; V. Muratore, None; R. Gallizzi, None; C. Bracaglia, None; A. Arduini, None; E. Comak, None; O. Vougiouka, None; R. Trauzeddel, None; G. Filocamo, None; C. Micalizzi, None; O. Kasapcopur, None; E. Unsal, None; T. Kitoh, None; E. Tsitsamis, None; M. Kostik, None; R. Cron, SOBI, 1, Novartis, 1, SOBI, 1, Pfizer, 1; J. Pachlopnik, None; D. Maritsi, None; M. Jelusic, None; S. Shenoi, None; A. Ravelli, None.

To cite this abstract in AMA style:

Minoia F, Tibaldi J, Muratore V, Gallizzi R, Bracaglia C, Arduini A, Comak E, Vougiouka O, Trauzeddel R, Filocamo G, Micalizzi C, Kasapcopur O, Unsal E, Kitoh T, Tsitsamis E, Kostik M, Cron R, Pachlopnik J, Maritsi D, Jelusic M, Shenoi S, Ravelli A. A Multinational Study of Thrombotic Microangiopathy in Macrophage Activation Syndrome: A Dreadful Condition Which Is Likely Underrecognized [abstract]. Arthritis Rheumatol. 2020; 72 (suppl 4). https://acrabstracts.org/abstract/a-multinational-study-of-thrombotic-microangiopathy-in-macrophage-activation-syndrome-a-dreadful-condition-which-is-likely-underrecognized/. Accessed March 28, 2023.
  • Tweet
  • Email
  • Print

« Back to 2020 Pediatric Rheumatology Symposium

ACR Meeting Abstracts - https://acrabstracts.org/abstract/a-multinational-study-of-thrombotic-microangiopathy-in-macrophage-activation-syndrome-a-dreadful-condition-which-is-likely-underrecognized/

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 »

© COPYRIGHT 2023 AMERICAN COLLEGE OF RHEUMATOLOGY

Wiley

  • Home
  • Meetings Archive
  • Advanced Search
  • Meeting Resource Center
  • Online Journal
  • Privacy Policy
  • Permissions Policies
  • Cookie Preferences