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Children with autoimmune cytopenia (AIC) and positive antinuclear antibodies (ANA) have a 22% risk of progressing to systemic lupus erythematosus (SLE), emphasizing the need for ANA screening and long-term monitoring for SLE in this population, especially for those with an initial ANA titer > 1/160 and age > 10 years at AIC diagnosis.

The following is a summary of “Antinuclear antibodies associated auto-immune cytopenia in childhood is a risk factor for systemic lupus erythematosus,” published in the January 2024 issue of Hematology by Granel et al.

In this study, the researchers investigated the risk of progression to systemic lupus erythematosus (SLE) in children with autoimmune cytopenia (AIC) associated with positive antinuclear antibodies (ANA). Utilizing data from the French national prospective OBS’CEREVANCE cohort, the study group examined the long-term outcomes of children with ANA-associated AIC (ANA titer ≥ 1/160) and specifically focused on those who later developed SLE.

Among 1,803 children with AIC, 20% (355/1803) exhibited positive ANA. Over a median follow-up of 5.8 years, 22% (79/355) of these ANA-positive patients developed SLE, with a median age of onset at 14.5 years. Notably, SLE incidence varied among AIC subtypes, including 20% for chronic immune thrombocytopenic purpura, 19% for autoimmune hemolytic anemia, and 45% for Evans syndrome. No patients negative for ANA developed SLE. Severe SLE manifestations were observed in 21 patients, leading to 2 fatalities. A multivariate analysis, incorporating patients with positive ANA within the initial 3 months after AIC diagnosis, identified age > 10 years at AIC diagnosis (relative risk (RR): 3.67, 95% confidence interval (CI) 1.18–11.4, P = 0.024) and ANA titer > 1/160 (RR: 5.28, 95% CI 1.20–23.17, P = 0.027) as significant factors associated with the occurrence of SLE post-AIC diagnosis.

This study underscores the role of ANA-associated AIC as a risk factor for SLE progression in children, particularly in those with an initial ANA titer > 1/160 and an age > 10 years at AIC diagnosis. Their findings advocate for ANA screening in children with AIC, emphasizing the importance of long-term monitoring for SLE, with a heightened focus on the transitional period. These insights contribute to a better understanding of AIC and SLE development dynamics in pediatric populations, facilitating informed clinical strategies and interventions.

Source: sciencedirect.com/science/article/abs/pii/S000649712400185X