Abstract
The relationship between antiphospholipid antibodies (aPL) and autoimmune haemolytic anaemia (AIHA) has never been systematically addressed. The aim of this study is to assess the link between aPL and AIHA in adult systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). This study performed an EMBASE/PubMed search from inception to June 2019 and meta-analysis using Peto’s odds ratios. The pooled prevalence (PP) of IgG/IgM anticardiolipin (aCL) and lupus anticoagulant (LA) was greater in AIHA +ve than AIHA −ve patients (34.7% vs. 27.6%, p = 0.03; 33.3% vs. 21.8%, p < 0.0001; 20.9% vs. 8.3%, p = 0.01). The PP of AIHA was greater in: (1) IgG and IgM aCL +ve than −ve patients (21.8% vs. 11.1%, p = 0.001 and 18.7% vs. 6.3%, p < 0.0001), (2) in SLE related APS than in primary APS patients (22.8% vs. 3.9% p < 0.0001), (3) in APS +ve than APS −ve SLE patients (23.2% vs. 8.4%, p = 0.01), and (4) in thrombotic APS than non-thrombotic APS/SLE patients (26.8% vs. 10%, p = 0.03). The PP of IgG/IgM aCL and LA was greater in DAT +ve than DAT −ve patients (42.4% vs. 12.8%, p < 0.0001; 26.2% vs. 12.8%, p = 0.03 and 29.2% vs. 15.7%, p = 0.004 respectively). It was found that AIHA prevalence is maximal in SLE with aPL/APS, low-moderate in SLE without aPL and minimal in PAPS. Moreover, AIHA is rightly included among the classification criteria for SLE but not for APS/aPL. The significance of an isolated DAT positivity remains unclear in this setting
Highlights
Recurrent arterial or venous thrombosis and/or obstetric morbidity in the presence and persistence of antiphospholipid antibodies detected via immunoassays or clotting tests characterise the antiphospholipid syndrome (APS): APS may be isolated or associated with other autoimmune disease, the most common being represented by systemic lupus erythematosus (SLE, lupus-related APS) [1]
Exploring the relationship between aPL and Autoimmune haemolytic anaemia (AIHA)/direct antiglobulin test (DAT) (Table 1) that were included in the qualitative
64] exploring the relationship between aPL and AIHA/DAT (Table 1) that were included in the survey
Summary
Recurrent arterial or venous thrombosis and/or obstetric morbidity in the presence and persistence of antiphospholipid antibodies (aPL) detected via immunoassays or clotting tests characterise the antiphospholipid syndrome (APS): APS may be isolated (primary APS, PAPS) or associated with other autoimmune disease (secondary APS), the most common being represented by systemic lupus erythematosus (SLE, lupus-related APS) [1]. 2020, 21, x FOR PEER REVIEW antibodies of the host immune system attack autologous red blood cells, usually demonstrated by a positive direct antiglobulin test (DAT), decreasing the patient’s lifedecreasing span and leading to haemolysis. Usually demonstrated by a positive direct antiglobulin test (DAT), the patient’s life span. Depending on the temperature at which theon antibody-red cell reaction occurs, antibodies can and leading to haemolysis. Depending the temperature at which the the antibody-red cell be defined as warm or cold (reviewed in [2]).
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