scholarly journals Epidemiology of Secondary Warm Autoimmune Haemolytic Anaemia—A Systematic Review and Meta-Analysis

2021 ◽  
Vol 10 (6) ◽  
pp. 1244
Author(s):  
Stinne Tranekær ◽  
Dennis Lund Hansen ◽  
Henrik Frederiksen

Background: Warm autoimmune haemolytic anaemia (wAIHA) is a haemolytic disorder, most commonly seen among adults and is classified as either primary or secondary to an underlying disease. We describe the age and sex distribution and the proportion of secondary wAIHA. Method: We retrieved 2635 published articles, screened abstracts and titles, and identified 27 articles eligible for full-text review. From these studies, we extracted data regarding number of patients, sex distribution, age at diagnosis, number of patients with secondary wAIHA, and whether the patients were diagnosed through local or referral centres. All data were weighted according to the number of included patients in each study. Results: 27 studies including a total of 4311 patients with wAIHA, of which 66% were females, were included. The median age at diagnosis was 68.7 years, however, wAIHA affected all ages. The mean proportion of secondary wAIHA was 49%, most frequently secondary to systemic lupus erythematosus. The proportions of secondary wAIHA reported from primary vs. referral centres were 35% vs. 59%, respectively. Conclusion: This review consolidates previously reported gender distribution. The higher proportion of secondary wAIHA in referral centres suggests that the most severely affected patients are disproportionally more frequent in such facilities.

2020 ◽  
Vol 21 (11) ◽  
pp. 4120 ◽  
Author(s):  
Paul R.J. Ames ◽  
Mira Merashli ◽  
Tommaso Bucci ◽  
Daniele Pastori ◽  
Pasquale Pignatelli ◽  
...  

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


Lupus ◽  
2019 ◽  
Vol 28 (12) ◽  
pp. 1480-1487 ◽  
Author(s):  
B Artım-Esen ◽  
E Çene ◽  
Y Şahinkaya ◽  
M Erdugan ◽  
E Oğuz ◽  
...  

Introduction Thrombocytopaenia and autoimmune haemolytic anaemia (AIHA) have considerable impact on prognosis in systemic lupus erythematosus (SLE). We investigated the frequencies of these haemocytopaenias, along with their associations and effect on outcome in a single-centre cohort of patients with SLE. Methods Demographic characteristics, clinical features, autoantibody profiles, damage and mortality data were compared between patients with and without each haematological abnormality. Variables displaying significant differences between the groups were entered into logistic regression. Results Ninety-three patients had AIHA and 215 had thrombocytopaenia. Both were associated with neuropsychiatric (NP) involvement, with each other, leucopaenia, antiphospholipid syndrome (APS) and antiphospholipid antibodies. More patients in both groups had organ damage, and their damage scores were higher. Association to NP damage was discernible. In addition, cardiovascular and renal damage and diabetes were more pronounced in patients with thrombocytopaenia. At logistic regression analysis, younger age, anticardiolipin antibody IgM positivity, leucopaenia and thrombocytopaenia were associated with AIHA whilst lupus anticoagulant activity, AIHA, leucopaenia, APS and NP involvement were associated with thrombocytopaenia. Among damage items, peripheral vascular damage, diabetes, NP damage, renal and ocular damage displayed significant associations with thrombocytopaenia, whereas none of the items did with AIHA. Patients with AIHA had significantly reduced survival rates at 10 and 20 years. Conclusions We observed that AIHA and thrombocytopaenia were associated with severe lupus, affecting major organs and causing end organ damage. Thus, they may be considered as prognostic markers. Furthermore, AIHA and especially thrombocytopaenia may also be a marker for a subgroup of lupus patients who have or may develop APS.


2021 ◽  
Vol 14 (12) ◽  
pp. e243051
Author(s):  
Archita Makharia ◽  
Manoj Lakhotia ◽  
Brateen Roy

Evans syndrome (ES) is a simultaneous or subsequent development of two haematological disorders, autoimmune haemolytic anaemia (AIHA) and immune thrombocytopenia (ITP). It can be primary (idiopathic) or secondary (associated with an underlying disease). Primary Evans is a diagnosis of exclusion and has a poorer prognosis than AIHA or ITP alone. We present a 55-year-old man who presented with weakness and lethargy and was diagnosed to be suffering from primary ES.


BMJ Open ◽  
2021 ◽  
Vol 11 (9) ◽  
pp. e047081
Author(s):  
Lixiang Wang ◽  
Yan Xie ◽  
Yingping Deng

ObjectivesTo investigate dry eye disease (DED) in patients affected by systemic lupus erythematosus (SLE).MethodsWe conducted a systematic search of the literature on PubMed, EMBASE and the Cochrane Library databases from conception to 30 April 2020 for studies related to dry eye, secondary Sjögren’s syndrome (sSS) and SLE. Original full-text articles with the number of patients diagnosed with SLE of over 15 were included. The risk of bias was evaluated with a validated critical appraisal tool which assessed study quality based on confounding factors, selection bias, bias related to measurement and bias related to data analysis. Data were extracted and pooled to evaluate the overall prevalence of DED with the random-effect model and sSS with the fixed effect model.ResultsA total of 29 studies were included and 18 273 participants were involved. The pooled data showed that the overall prevalence of DED was 16% (95% CI 10% to 21%, p<0.001) in patients of SLE. Dry eye symptoms and abnormal Schirmer’s test were found in 26% (95% CI 20% to 32%, p<0.001) and 24% (95% CI 14% to 34%, p<0.001) of patients with SLE, respectively. 12% (95% CI 9% to 15%, p<0.001) of patients also met the criteria of sSS. The OR of DED in patients with SLE was 4.26 (95% CI 3.47 to 5.05, p<0.001) compared with healthy controls. The meta-regression analysis showed that the sample size (p=0.004) and study location (p=0.022) could be the source of heterogeneity.ConclusionsDED and sSS are both common in patients with SLE.


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