Introduction/objective: The relationship between autoimmune haemolytic anaemia (AIHA) and antiphospholipid antibodies (aPL) has never been addressed via a meta-analysis in the paediatric age group. We evaluated the link between AIHA and aPL in childhood systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). Methods: EMBASE and PubMed were screened from inception to May 2020 and Peto’s odds ratio for rare events was employed for the between group comparisons. Results: The meta-analysis included 11 articles for a total of 575 children: the pooled prevalence of AIHA was greater in (1) IgG aCL–positive than IgG aCL–negative children (39.7% vs 20.9%, p = 0.005); (2) in APS-positive than APS-negative SLE children (36.8% vs 13.2%, p = 0.01); and (3) in SLE-related APS than in primary APS children (53% vs 16.2%, p = 0.008). Conclusions: The pooled prevalence of AIHA is greatest in SLE with aPL/APS, low-moderate in SLE without aPL/APS, and lowest in primary APS.Key Points• Antiphospholipid antibodies strongly relate to autoimmune haemolytic anaemia.• Autoimmune haemolytic anaemia is more common in systemic lupus erythematosus with antiphospholipid antibodies.
- Antiphospholipid syndrome
- Autoimmune haemolytic anaemia