Systemic Inflammatory Diseases in Children With Sickle Cell Disease: A French Multicenter Observational Study on Diagnostic and Therapeutic Issues

IF 2.4 3区医学 Q2 HEMATOLOGY Pediatric Blood & Cancer Pub Date : 2025-01-27 DOI:10.1002/pbc.31563

Caroline Vinit, Corinne Guitton, Mariane De Montalembert, Patricia Benhaim, Lahoueri Amor-Chelihi, Brigitte Bader-Meunier, Florence Missud, Isabelle Melki, Vincent Gajdos, Cécile Arnaud, Annie Kamden, Oussama Charara, Véronique Hentgen, Sylvie Nathanson, Coralie Bloch, Ulrich Meinzer, Pierre Quartier, Isabelle Kone-Paut, Loïc De Pontual, Luu-Ly Pham

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Abstract

Background

Systemic inflammatory diseases (SIDs) have been reported in patients with sickle cell disease (SCD), but clinical data in children are scarce.

Objectives

To identify clinical and laboratory features at diagnosis of SID in children with SCD and to describe their evolution.

Methods

Data from children with SCD and SIDs were retrospectively collected in a French multicenter study from 1991 to 2018. Information included clinical characteristics, inflammatory markers, autoantibodies patterns, treatments, and complications. Inflammatory marker levels were compared at SID diagnosis and at the last follow-up. Statistical analyses were performed using Cran R software.

Results

Among a cohort of 3800 children with SCD, 43 SIDs were identified in 35 study participants: autoimmune liver disease (AILD, n = 13), inflammatory bowel disease (IBD, n = 7), juvenile idiopathic arthritis (JIA, n = 6), systemic lupus erythematosus (n = 4), autoimmune hemolytic anemia (n = 3), Sjögren syndrome (n = 1), histiocytic necrotizing lymphadenitis (n = 2), vasculitis (n = 2), myasthenia gravis (n = 1), sarcoidosis (n = 1), idiopathic inflammatory granulomatous uveitis (n = 1), mixed connective tissue disease (n = 2). Prevalence of SID was 0.9% in our cohort of children with SCD. The median time between initial symptoms and SID diagnosis was 10 (3–20) months, notably longer in children with JIA, IBD, and Sjögren syndrome. Sixteen patients (46%) exhibited hypergammaglobulinemia (>20 g/L) at diagnosis. No significant differences were observed for other inflammatory parameters. Twenty-one children (60%) received systemic steroids and 13 (37%) biological therapies. Three patients (9%) underwent hematopoietic stem cell transplantation. Nine patients (26%) had severe infections; one died.

Conclusion

Delayed diagnosis was frequent due to overlapping clinical presentations between SCD and SID. Clinicians must be aware of warning signs associated with elevated inflammatory markers, hypergammaglobulinemia, or specific antibodies. Therapeutic strategies remain challenging.

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来源期刊

Pediatric Blood & Cancer 医学-小儿科

CiteScore

4.90

自引率

9.40%

发文量

546

审稿时长

1.5 months

期刊介绍： Pediatric Blood & Cancer publishes the highest quality manuscripts describing basic and clinical investigations of blood disorders and malignant diseases of childhood including diagnosis, treatment, epidemiology, etiology, biology, and molecular and clinical genetics of these diseases as they affect children, adolescents, and young adults. Pediatric Blood & Cancer will also include studies on such treatment options as hematopoietic stem cell transplantation, immunology, and gene therapy.