Autoimmune haemolytic anaemia and antiphospholipid antibodies in paediatrics: a systematic review and meta-analysis
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ORIGINAL ARTICLE
Autoimmune haemolytic anaemia and antiphospholipid antibodies in paediatrics: a systematic review and meta-analysis Mira Merashli 1 & Alessia Arcaro 2 & Maria Graf 3 & Fabrizio Gentile 2 & Paul R. J. Ames 4,5 Received: 17 August 2020 / Revised: 28 September 2020 / Accepted: 29 September 2020 # International League of Associations for Rheumatology (ILAR) 2020
Abstract 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.
Keywords Antiphospholipid syndrome . Autoimmune haemolytic anaemia . Lupus
Introduction Autoimmune haemolytic anaemia (AIHA) is one of the EULAR/ACR classification criteria for childhood systemic lupus erythematosus (SLE) [1]. AIHA ensues when autologous erythrocytes are attacked and destroyed by antibodies of the
* Paul R. J. Ames [email protected] 1
Department of Rheumatology, American University of Beirut, Beirut, Lebanon
2
Department of Medicine & Health Sciences ‘V.Tiberio’, Universita’ del Molise, Campobasso, Italy
3
Department of Laboratory and Transfusion Medicine, Federico II University Hospital, Naples, Italy
4
Immune Response & Vascular Disease Unit, Nova University, Lisbon, Portugal
5
Dumfries and Galloway Royal Infirmary, Dumfries, UK
host immune system demonstrated by a positive (+ve) direct antiglobulin test (DAT) leading to a shortened erythrocyte survival; AIHA is defined as warm or cold (reviewed in [2]) according to the temperature at which the antibody-erythrocyte reaction occurs. According to two recent meta-analyses, AIHA is more common in childhood than adult SLE (relative risk: 0.69, 95% CI: 0.58 to 0.81) [3], and in adults, AIHA relates to antiphospholipid antibodies (aPL) with a prevalence of up to 26%, particularly in patients with antiphospholipid syndrome (APS) [4]. The objective of our study was to investigate the extent of the aPL/AIHA relationship in childhood SLE.
Methods Search strategy and selection criteria The systematic rev
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