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Paediatric Rheumatology

 

Insights into the clinical presentation of juvenile systemic lupus erythematosus: the PRAGMA monocentric cohort of 177 patients


1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16

 

  1. Department of Clinical Sciences and Community Health, University of Milan, and Paediatric Rheumatology Unit, ASST G. Pini-CTO, Milan, Italy. cecilia.chighizola@unimi.it
  2. Fondazione IRCCS Ca’ Granda-Ospedale Maggiore Policlinico, Milan, Italy.
  3. Paediatric Rheumatology Unit, ASST G. Pini-CTO, Milan, Italy.
  4. Fondazione IRCCS Ca’ Granda-Ospedale Maggiore Policlinico, Milan, Italy.
  5. Paediatric Rheumatology Unit, ASST G. Pini-CTO, Milan, Italy.
  6. Fondazione IRCCS Ca’ Granda-Ospedale Maggiore Policlinico, Milan, Italy.
  7. Clinical Rheumatology Unit, ASST G. Pini-CTO, Milan, Italy.
  8. Fondazione IRCCS Ca’ Granda-Ospedale Maggiore Policlinico, Milan, Italy.
  9. Department of Clinical Sciences and Community Health, University of Milan, and Clinical Rheumatology Unit, ASST G. Pini-CTO, Milan, Italy.
  10. Paediatric Rheumatology Unit, ASST G. Pini-CTO, Milan, Italy.
  11. Fondazione IRCCS Ca’ Granda-Ospedale Maggiore Policlinico, Milan, Italy.
  12. Fondazione IRCCS Ca’ Granda-Ospedale Maggiore Policlinico, Milan, Italy.
  13. Fondazione IRCCS Ca’ Granda-Ospedale Maggiore Policlinico, Milan, Italy.
  14. Department of Clinical Sciences and Community Health, University of Milan, and Clinical Rheumatology Unit, ASST G. Pini-CTO, Milan, Italy.
  15. Department of Clinical Sciences and Community Health, University of Milan, and Fondazione IRCCS Ca’ Granda-Ospedale Maggiore Policlinico, Milan, Italy.
  16. Department of Clinical Sciences and Community Health, University of Milan, and Clinical Rheumatology Unit, ASST G. Pini-CTO, Milan, Italy.

on behalf of the Pediatric Rheumatology Associated Group of the Milan Area (PRAGMA)

CER16288
2023 Vol.41, N°9
PI 1926, PF 1933
Paediatric Rheumatology

purchase article

PMID: 37223914 [PubMed]

Received: 17/10/2022
Accepted : 16/01/2023
In Press: 18/05/2023
Published: 17/08/2023

Abstract

OBJECTIVES:
The aim of this work is to describe the clinical manifestations at onset and during follow-up in a monocentric cohort of patients with juvenile systemic lupus erythematosus (jSLE) from the Paediatric Rheumatology group of the Milan area (PRAGMA).
METHODS:
Patients were retrospectively included in case of i) SLE diagnosis according to the 1997 American College of Rheumatology or the 2012 SLICC classification criteria and ii) disease onset before 18 years.
RESULTS:
Among the 177 recruited patients (155 females), haematologic involvement was the most common disease manifestation (75%), followed by joint and cutaneous involvements (70% and 57%, respectively). Renal disease was observed in 58 patients (32.8%), neurological complications in 26 cases (14.7%). Patients presented most commonly 3 clinical manifestations (32.8%), while 2 organ involvements were identified in 54 patients (30.5%) and 4 in 25 subjects (14.1%). The 49 patients with disease onset <10 years had less commonly articular involvement (p=0.02), while patients aged >14.8 years displayed less neurological manifestations (p=0.02). At a median follow-up of 118 month, the disease progressed in 93 patients, with a median of 2 new manifestations per patient. Low complement at diagnosis predicted new clinical manifestations (p=0.013 for C3 and p=0.0004 for C4). The median SLEDAI at diagnosis was 13; SLEDAI was substantially similar at 6 months, decreased at 12 months to remain stable at 18 months and further reduce at 24 months (p<0.0001).
CONCLUSIONS:
These data from a large jSLE monocentric cohort allow gaining further insights into a rare disease with a still high morbidity burden.

DOI: https://doi.org/10.55563/clinexprheumatol/rp916a

Rheumatology Article