Antinuclear antibodies associated auto-immune cytopenia in childhood is a risk factor for systemic lupus erythematosus

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 the subgroup of children who developed SLE were described. ANA were positive in 355/1803 (20%) children with AIC. With a median follow-up of 5.8 (0.1-29.6) years, 79/355 (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. No ANA-negative patients 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 = 0.024) and ANA titer > 1/160 (RR: 5.28, 95% CI 1.20; 23.17, P = 0.027) 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 ANA positive patients should be long-term monitored for SLE with a special attention on the transition period.