Retrospective Cohort Study
Copyright ©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Pediatr. Mar 9, 2024; 13(1): 88912
Published online Mar 9, 2024. doi: 10.5409/wjcp.v13.i1.88912
Systemic juvenile idiopathic arthritis–associated lung disease: A retrospective cohort study
Konstantin E Belozerov, Natalia M Solomatina, Eugenia A Isupova, Alla A Kuznetsova, Mikhail M Kostik
Konstantin E Belozerov, Natalia M Solomatina, Eugenia A Isupova, Alla A Kuznetsova, Mikhail M Kostik, Department of Pediatric, Saint-Petersburg State Pediatric Medical University, Saint-Petersburg 194100, Russia
Konstantin E Belozerov, St. Petersburg State Budgetary Institution of Health Care, Children's City Polyclinic No. 29 of the Kalininsky District of St. Petersburg, St. Petersburg 195274, Russia
Mikhail M Kostik, Research Laboratory of Autoimmune and Autoinflammatory Diseases, World-Class Research Centre for Personalized Medicine, Almazov National Medical Research Centre, St. Petersburg 197341, Russia
Author contributions: Kostik MM and Belozerov KE contributed to the conceptualization, writing, review, editing and original draft preparation; Kostik MM and Kuznetsova AA contributed to the methodology; Belozerov KE contributed to the software and formal analysis; Solomatina NM and Isupova EA contributed to the validation and resources; Belozerov KE, Solomatina NM, and Isupova EA contributed to the investigation; Kuznetsova AA contributed to data curation; Kostik MM contributed to funding, supervision and project administration; All authors read and approve the final manuscript, were involved in drafting the article or revising it critically for important intellectual content.
Supported by the Ministry of Science and Higher Education of the Russian Federation, No. 075-15-2022-301.
Institutional review board statement: The Ethics Committee of Saint Petersburg State Pediatric Medical University (18/01 from 27.10.2022) approved this retrospective study's protocol.
Informed consent statement: Written consent was obtained according to the Declaration of Helsinki. All patients or patient representatives (for patients under the age of 15 years) gave their consent in their case report forms authorizing the anonymous use of their medical information. All patients were appropriately anonymized.
Conflict-of-interest statement: All authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
Data sharing statement: The datasets generated during and/or analyzed during the current study are available from the corresponding author upon reasonable request.
STROBE statement: The authors have read the STROBE Statement—a checklist of items, and the manuscript was prepared and revised according to the STROBE Statement—a checklist of items.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Mikhail M Kostik, MD, PhD, Professor, Department of Pediatric, Saint-Petersburg State Pediatric Medical University, Lytovskaya 2, Saint-Petersburg 194100, Russia. kost-mikhail@yandex.ru
Received: October 14, 2023
Peer-review started: October 14, 2023
First decision: December 23, 2023
Revised: January 3, 2024
Accepted: February 18, 2024
Article in press: February 18, 2024
Published online: March 9, 2024
ARTICLE HIGHLIGHTS
Research background

Chronic lung involvement is an ultra-rare, unrecognized, poorly understood condition in children with systemic juvenile idiopathic arthritis.

Research motivation

To describe this ultra-rare complication and disease course in children with systemic juvenile idiopathic arthritis with interstitial lung involvement.

Research objectives

The clinical and laboratory data of these patients are not well-diagnosed. The number of patients is nearly a hundred.

Research methods

The clinical, radiological, and laboratory features were described in detail. The H score was applied to these patients for the first time.

Research results

The main clinical features of the disease are associated with early onset, chronic course of macrophage activation syndrome, pleuritis at onset, protracted lymphopenia, eosinophilia, and anaphylaxis drug-reaction on biologics.

Research conclusions

This life-threatening complication is associated with chronic, persistent macrophage activation syndrome, drug-associated anaphylaxis similar to DRESS syndrome.

Research perspectives

The future collection of information on these patients requires the following study of the features of the macrophage activation syndrome (cytokine profile, interferon signatures), and new target drugs are needed.