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Open Access 01-12-2024 | Mixed Connective Tissue Disease | Short Report

Longitudinal follow-up of mixed connective tissue disease and overlapping autoimmune diseases of childhood onset in the Afro-descendant population of the French West Indies

Authors: Arthur Felix, Lindsay Osei, Frederique Delion, Benoit Suzon, Aurore Abel, Moustapha Drame, Yves Hatchuel, Christophe Deligny, Fabienne Louis-Sidney

Published in: Pediatric Rheumatology | Issue 1/2024

Abstract

Introduction

Overlap autoimmune syndromes (OAS) and mixed connective tissue disease (MCTD) are rare in children. We performed a retrospective, longitudinal and descriptive study of Afro-Caribbean patients from the French West Indies followed for MCTD and OAS to describe their characteristics and outcomes during childhood.

Methods

Retrospective study from January 2000 to 2023. Listings of patients were obtained from multiple sources: computerized hospital archives and national hospital-based surveillance system, registry of pediatricians and adult specialists in internal medicine and the national registry for rare diseases. MCTD was defined according to Kasukawa’s criteria. OAS was defined as overlapping features of systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and dermatomyositis/autoimmune myositis (DM/AM).

Results

Sixteen patients were included over a 23-year period (10 MCTD and 6 OAS). The incidence was 0.23 per 100,000 children-years. The mean age at diagnosis was 11.9 years old (2.4–17) with median follow up of 7.9 years (2.1–19.6). SLE phenotype was present in the highest, followed by SSc and DM/AM. Patients had an average of three flares during childhood (1–7). A quarter (25%) had symptomatic pulmonary arterial hypertension (PAH). Ninety-four percent received steroids during follow-up and 88% required a corticosteroid-sparing therapy. Three patients (19%) developed SLE after more than 10y of follow-up. There were no death and no chronic organ failure.

Conclusion

This is the largest pediatric cohort of MCTD and OAS in Afro-descendant patients treated in a country with a high standard of care. The clinical evolution did not differ between MCTD and OAS. The main complication was PAH, more frequent in our cohort.

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Title: Longitudinal follow-up of mixed connective tissue disease and overlapping autoimmune diseases of childhood onset in the Afro-descendant population of the French West Indies
Authors: Arthur Felix
Lindsay Osei
Frederique Delion
Benoit Suzon
Aurore Abel
Moustapha Drame
Yves Hatchuel
Christophe Deligny
Fabienne Louis-Sidney
Publication date: 01-12-2024
Publisher: BioMed Central
Keywords: Mixed Connective Tissue Disease
Systemic Sclerosis
Systemic Lupus Erythematosus
Published in: Pediatric Rheumatology / Issue 1/2024
Electronic ISSN: 1546-0096
DOI: https://doi.org/10.1186/s12969-023-00951-3

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Longitudinal follow-up of mixed connective tissue disease and overlapping autoimmune diseases of childhood onset in the Afro-descendant population of the French West Indies

Abstract

Introduction

Methods

Results

Conclusion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Introduction

Methods

Results

Conclusion

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