Antinuclear antibody–associated autoimmune cytopenia in childhood is a risk factor for systemic lupus erythematosus
Jérôme Granel
(1, 2)
,
Helder Fernandes
(1)
,
Brigitte Bader-Meunier
(3)
,
Amandine Guth
(4)
,
Olivier Richer
(1)
,
Pascal Pillet
(1)
,
Guy Leverger
(2)
,
Stéphane Ducassou
(1, 2)
,
Mony Fahd
(2)
,
Marlène Pasquet
(5)
,
Nathalie Garnier
(6)
,
Vincent Barlogis
(7)
,
Corinne Guitton
(8)
,
Eric Jeziorski
(9)
,
Caroline Thomas
(10)
,
Sophie Bayart
(11)
,
Nathalie Cheikh
(12)
,
Catherine Paillard
(13)
,
Wadih Abou Chahla
(14)
,
Pascal Chastagner
(15, 16)
,
Bénédicte Neven
(3)
,
Frédéric Millot
(17)
,
Julien Lejeune
(18)
,
Valérie Li-Thiao Te
(19)
,
Corinne Armari-Alla
(20)
,
Claire Briandet
(21)
,
Liana Carausu
(22)
,
Marianna Deparis
(23)
,
Christophe Piguet
(24)
,
Joy Benadiba
(25)
,
Aude Marie-Cardine
(26)
,
Jean-Louis Stephan
(27)
,
Isabelle Pellier
(28)
,
Claire Pluchart
(29)
,
Eric Doré
(30)
,
Katell Michaux
(31)
,
Sébastien Héritier
(2)
,
Thierry Leblanc
(2)
,
Nathalie Aladjidi
(1, 2)
1
CIC Bordeaux
2 Centre de Référence National des Cytopénies Auto-immunes de l'Enfant, Bordeaux
3 Paediatric Haematology-Immunology and Rheumatology Department, Hôpital Necker-Enfants Malades, AP-HP, Paris
4 Paediatric Department, Pontarlier Hospital, Pontarlier
5 Sercice Hématologie, immunologie et oncologie pédiatrique [CHU Toulouse]
6 Institute of Paediatric Haematology and Oncology, Hospices Civils de Lyon, Lyon
7 Department of Paediatric Haematology, La Timone Hospital, Marseille University Hospital, Marseille
8 Department of Paediatrics, Bicêtre University Hospital, Assistance Publique-Hôpitaux de Paris, Le Kremlin-Bicêtre
9 Paediatric Oncology Haematology Unit, Arnaud de Villeneuve University Hospital, Montpellier
10 Paediatric Haematology Unit, Nantes University Hospital, Nantes
11 Paediatric Haematology Unit, Rennes University Hospital, Rennes
12 Department of Paediatric Haematology-Oncology, Besançon University Hospital, Besançon
13 Department of Paediatric Haematology and Oncology, Hautepierre University Hospital, Strasbourg
14 Department of Paediatric Haematology, Jeanne de Flandre Hospital, Lille University Hospital, Lille
15 CRAN - Centre de Recherche en Automatique de Nancy
16 CHRU Nancy - Centre Hospitalier Régional Universitaire de Nancy
17 Department of Paediatric Haematology, Poitiers University Hospital, Poitiers
18 Department of Paediatric Haematology-Oncology, Clocheville Hospital, Tours University Hospital, Tours
19 Department of Paediatric Haematology/Oncology, Amiens University Hospital, Amiens
20 Paediatric Haematology-Oncology Department, Grenoble University Hospital, Grenoble
21 Department of Paediatrics, Dijon University Hospital, Dijon
22 Department of Paediatric Hematology, CHU de Brest, Brest
23 Paediatric Oncology- Haematology Unit Department, Caen University Hospital, Caen
24 Paediatric Oncology Hematology Unit, Limoges University Hospital, Limoges
25 Department of Haematology-Oncology Paediatrics, Nice University Hospital, Nice
26 Department of Paediatric Haematology and Oncology, Rouen University Hospital, Rouen
27 University Hospital of Saint Etienne, North Hospital, Department of Paediatric Oncology, Saint Etienne
28 Paediatric Unit, Angers University Hospital, Angers
29 Paediatric Haematology-Oncology Unit, Institut Jean Godinot, Reims University Hospital, Reims
30 Paediatric Unit, Clermont-Ferrand University Hospital, Clermont-Ferrand
31 Paediatric Unit, Martinique University Hospital, Fort-de-France
2 Centre de Référence National des Cytopénies Auto-immunes de l'Enfant, Bordeaux
3 Paediatric Haematology-Immunology and Rheumatology Department, Hôpital Necker-Enfants Malades, AP-HP, Paris
4 Paediatric Department, Pontarlier Hospital, Pontarlier
5 Sercice Hématologie, immunologie et oncologie pédiatrique [CHU Toulouse]
6 Institute of Paediatric Haematology and Oncology, Hospices Civils de Lyon, Lyon
7 Department of Paediatric Haematology, La Timone Hospital, Marseille University Hospital, Marseille
8 Department of Paediatrics, Bicêtre University Hospital, Assistance Publique-Hôpitaux de Paris, Le Kremlin-Bicêtre
9 Paediatric Oncology Haematology Unit, Arnaud de Villeneuve University Hospital, Montpellier
10 Paediatric Haematology Unit, Nantes University Hospital, Nantes
11 Paediatric Haematology Unit, Rennes University Hospital, Rennes
12 Department of Paediatric Haematology-Oncology, Besançon University Hospital, Besançon
13 Department of Paediatric Haematology and Oncology, Hautepierre University Hospital, Strasbourg
14 Department of Paediatric Haematology, Jeanne de Flandre Hospital, Lille University Hospital, Lille
15 CRAN - Centre de Recherche en Automatique de Nancy
16 CHRU Nancy - Centre Hospitalier Régional Universitaire de Nancy
17 Department of Paediatric Haematology, Poitiers University Hospital, Poitiers
18 Department of Paediatric Haematology-Oncology, Clocheville Hospital, Tours University Hospital, Tours
19 Department of Paediatric Haematology/Oncology, Amiens University Hospital, Amiens
20 Paediatric Haematology-Oncology Department, Grenoble University Hospital, Grenoble
21 Department of Paediatrics, Dijon University Hospital, Dijon
22 Department of Paediatric Hematology, CHU de Brest, Brest
23 Paediatric Oncology- Haematology Unit Department, Caen University Hospital, Caen
24 Paediatric Oncology Hematology Unit, Limoges University Hospital, Limoges
25 Department of Haematology-Oncology Paediatrics, Nice University Hospital, Nice
26 Department of Paediatric Haematology and Oncology, Rouen University Hospital, Rouen
27 University Hospital of Saint Etienne, North Hospital, Department of Paediatric Oncology, Saint Etienne
28 Paediatric Unit, Angers University Hospital, Angers
29 Paediatric Haematology-Oncology Unit, Institut Jean Godinot, Reims University Hospital, Reims
30 Paediatric Unit, Clermont-Ferrand University Hospital, Clermont-Ferrand
31 Paediatric Unit, Martinique University Hospital, Fort-de-France
Marlène Pasquet
- Fonction : Auteur
- PersonId : 1166707
- ORCID : 0000-0001-6919-8383
Vincent Barlogis
- Fonction : Auteur
- PersonId : 762453
- ORCID : 0000-0001-9645-3968
- IdRef : 108920046
Corinne Guitton
- Fonction : Auteur
- PersonId : 1403211
- ORCID : 0000-0001-5945-0469
Eric Jeziorski
- Fonction : Auteur
- PersonId : 1166461
- ORCID : 0000-0003-0318-3044
Nathalie Cheikh
- Fonction : Auteur
- PersonId : 792716
- ORCID : 0000-0002-3403-3842
Isabelle Pellier
- Fonction : Auteur
- PersonId : 1184769
- ORCID : 0000-0002-7988-4712
Sébastien Héritier
- Fonction : Auteur
- PersonId : 1166706
- ORCID : 0000-0003-0384-6370
- IdRef : 144258587
Thierry Leblanc
- Fonction : Auteur
- PersonId : 953414
Nathalie Aladjidi
- Fonction : Auteur
- PersonId : 1166705
- ORCID : 0000-0003-0231-4460
- IdRef : 091935172
Résumé
Autoimmune cytopenia (AIC) in children may be associated with positive antinuclear antibodies (ANA) and may progress to systemic lupus erythematosus (SLE). We evaluated the risk of progression to SLE of childhood-onset ANA-associated AIC. In the French national prospective OBS’CEREVANCE cohort, the long-term outcome of children with ANA-associated AIC (ANA titer ≥1/160) and a subgroup of children who developed SLE were described. ANA were positive in 355 of 1803 (20%) children with AIC. With a median follow-up of 5.8 (range, 0.1-29.6) years, 79 of 355 (22%) patients developed SLE at a median age of 14.5 (1.1-21.4) years; 20% of chronic immune thrombocytopenic purpura, 19% of autoimmune hemolytic anemia, and 45% of Evans syndrome. None of the patients with ANA-negative test developed SLE. Severe manifestations of SLE were observed in 21 patients, and 2 patients died. In multivariate analysis including patients with positive ANA within the first 3 months after AIC diagnosis, age >10 years at AIC diagnosis (relative risk [RR], 3.67; 95% confidence interval [CI], 1.18-11.4; P = .024) and ANA titer >1/160 (RR, 5.28; 95% CI, 1.20-23.17; P = .027) were associated with the occurrence of SLE after AIC diagnosis. ANA-associated AIC is a risk factor for progression to SLE, especially in children with an initial ANA titer >1/160 and an age >10 years at AIC diagnosis. ANA screening should be recommended in children with AIC, and patients with ANA should be monitored long-term for SLE, with special attention to the transition period. This trial was registered at www.ClinicalTrials.gov as #NCT05937828.