Systemic lupus erythematosus and neutropaenia: a hallmark of haematological manifestations

Abstract

<jats:sec><jats:title>Objective</jats:title><jats:p>Systemic lupus is a chronic autoimmune disease characterised by its phenotypic heterogeneity. Neutropaenia is a frequent event in SLE occurring in 20%–40% of patients depending on the threshold value of neutrophil count. On a daily basis, the management of neutropaenia in SLE is difficult with several possible causes. Moreover, the infectious consequences of neutropaenia in SLE remain not well defined.</jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p>998 patients from the Lupus BioBank of the upper Rhein (LBBR), a large German and French cohort of patients with SLE, mostly of Caucasian origin (83%), were included in this study. Neutropaenia was considered when neutrophil count was below 1800×10<jats:sup>6</jats:sup>/L. An additional analysis of detailed medical records was done for 65 LBBR patients with neutropaenia.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>208 patients with neutropaenia (21%) were compared with 779 SLE patients without neutropaenia. Neutropaenia in SLE was significantly associated with thrombocytopaenia (OR 4.11 (2.57–10.3)), lymphopaenia (OR 4.41 (2.51–11.5)) and low C3 (OR 1.91 (1.03–4.37)) in multivariate analysis. 65 representative patients with neutropaenia were analysed. Neutropaenia was moderate to severe in 38%, chronic in 31%, and both severe and chronic in 23% of cases. Moderate to severe and chronic neutropaenia were both associated with lymphopaenia and thrombopaenia. Chronic neutropaenia was also associated anti-Ro/SSA antibodies and moderate to severe neutropaenia with oral ulcers.</jats:p></jats:sec><jats:sec><jats:title>Conclusion</jats:title><jats:p>This study is to date the largest cohort to describe neutropaenia in SLE. Neutropaenia displays a strong association with other cytopaenias, suggesting a common mechanism. Chronic neutropaenia is associated with anti-Ro/SSA antibodies with or without identified Sjögren’s disease.</jats:p></jats:sec>

The Clinical Characteristics of Leukopenia in Patients with Systemic Lupus Erythematosus of Han Ethnicity in China: A Cross-Sectional Study

“…There was also no significant association between any autoantibody against nuclear antigens and neutropenia in a study including 82 lupus erythematosus patients [ 18 ]. A study including 208 SLE patients with neutropenia and 779 SLE patients without neutropenia showed that neutropenia was significantly associated with thrombocytopenia, lymphopenia, and low levels of complement C3 as determined in a multivariate analysis, which was similar to our results [ 19 ].…”

Independent associations of lymphopenia and neutropenia in patients with systemic lupus erythematosus: a longitudinal, multinational study

“…Most leucopenia episodes were driven by lymphopenia, which is recognized as the most common white blood cell abnormality among lupus patients [ 1 ]. Neutropenia was observed at least once in 12.5% of patients, lower than a recently reported European cohort [ 15 ], and occurred in 3.4% of visits. Severe neutropenia was rare (0.95%).…”

“…The study was done across multiple Asia Pacific lupus centres and is therefore a predominantly Asian cohort (89%). Genetic factors relating to ethnicity can certainly influence the severity and frequency of leucopenia; for example, we have already seen that our cohort had a lower frequency of neutropenia compared with another recent report of lupus patients in Europe [ 15 ]. Our findings, however, are still largely generalizable, as the overall prevalence of lymphopenia (which made up the majority of leucopenia), the patterns of disease activity and medication use were all similar to other cohorts.…”

Hematological Manifestations among Patients with Rheumatic Diseases

“…Neutropenia was reported to occur in 20-47% of the patients with SLE [2,6,11,12]. A recent large multicentric registry (Lupus BioBank of the upper Rhein) found a prevalence of 21% of neutropenia in Caucasian SLE that was significantly associated with thrombocytopenia (OR 3.68 [2.58-5.25 [1.96-4.32], p < 0.0001) [13]. Among other causes of neutropenia in SLE patients, benign ethnic (familial) neutropenia, which is one of the most common causes of chronic neutropenia seen in individuals of African, Middle Eastern, and west Indian descent [14], should be excluded before attributing lupus as the cause of neutropenia.…”