Anemia In Systemic Lupus Erythematosus Research Articles

Pancytopenia with aplastic anemia in systemic lupus erythematosus: case series and literature review.

Aplastic anemia (AA) is a rare, potentially catastrophic hematopoiesis failure manifested by pancytopenia and bone marrow aplasia. AA occurrence in Systemic Lupus Erythematosus (SLE) patients is extremely rare. The diagnosis may be delayed due to other possible pancytopenia etiologies. Confirmation of peripheral cytopenias diagnosis necessitates a bone marrow aspiration. The management of AA is challenging, and the literature reported using glucocorticoids, danazol, plasmapheresis, cyclophosphamide, intravenous immunoglobulin, and cyclosporine. We report two cases of SLE patients who presented with pancytopenia, with bone marrow biopsy confirmed AA. One case was treated with cyclophosphamide but unfortunately succumbed to Acute Respiratory Distress Syndrome (ARDS), while the other case was managed with rituximab with a good response. Interestingly, both patients were on azathioprine before the diagnosis of AA. A comprehensive search for reported cases of AA in PubMed, Scopus, and the Directory of Open Access Journals databases was performed to enhance the understanding of the diagnostic and management challenges associated with AA in SLE, facilitating ongoing exploration and research in this field. The decision to do a BM aspiration and biopsy is recommended for SLE patients with an abrupt decline in blood counts and previously stable blood counts.

Association of the premature immunosenescence with the presence and severity of anemia among patients with systemic lupus erythematosus.

One of the possible mechanisms that contribute to the development of anemia in systemic lupus erythematosus (SLE) is the presence of premature immunosenescence in SLE. This study aimed to observe the correlation between immunosenescence with anemia in SLE. This research was a cross-sectional study with the subject was 60 women with SLE aged 16-45years old. Subjects were recorded for the demographic and clinical data, complete blood counts, iron status (iron serum, total iron-binding capacity, and transferrin saturation), ferritin, inflammatory markers (erythrocyte sedimentation rate [ESR] and C-reactive protein [CRP]), and anti-dsDNA levels. Immunosenescence was observed by measuring the senescent T cells from peripheral blood mononuclear cells (PBMC) by flow cytometry, counted as CD4+CD57+ and CD8+CD57+ T cells. Serum IL-2 and IFNγ as the cytokines associated with immunosenescence were also measured from all subjects. Subjects were divided into anemic and non-anemic groups according to the classification of anemia from WHO (Hb < 12gr/dl). Anemic SLE patients had higher CD4+CD57+, CD8+CD57+, and IFNγ, while IL-2 was lower in SLE patients with anemia. Multivariate linear regression revealed that the decreasing levels of Hb were associated with the increase of CD8+CD57+ percentages and IFNγ levels. Anti-dsDNA, ESR, CRP, ferritin, iron serum, and transferrin saturation were correlated with CD8+CD57+. IFNγ level also correlated with the anti-dsDNA, iron serum, and ferritin levels. No correlation was found between the iron status and inflammatory markers with CD4+CD57+ percentages and IL-2 levels. Multivariate regression analysis showed that IFNγ was positively associated with anti-dsDNA and negatively associated with iron serum and transferrin saturation, while CD8+CD57+ percentages were positively associated with the ferritin levels. Immunosenescence is associated with anemia by modulating the inflammatory response and causing iron dysregulation in SLE.

Serum hepcidin and interleukin-6 in systemic lupus erythematosus patients: crucial factors for correction of anemia

BackgroundThe incidence rate of anemia of chronic disease (ACD) in systemic lupus erythematosus (SLE) ranges between 30 and 80%. Serum iron is the main regulator of hepatic hepcidin production. Interleukin-6 (IL-6) upregulates hepcidin expression. The aim of this study is to compare between serum hepcidin and IL-6 in SLE patients and control subjects, and to find out if they are correlated with each other and with disease activity in order to find their role in treatment of anemia in SLE patients.The study was carried out on 50 SLE patients, suffering from anemia, diagnosed according to SLICC revision of the ACR classification criteria for SLE, and 50 healthy individuals, taken as control. Disease activity was assessed using the SLE disease activity index (SLEDAI-2 K). Serum hepcidin and IL-6 were measured by ELISA kit.ResultsThere was a highly statistically significant difference in serum hepcidin and IL-6 levels between patients and control subjects. There was a statistically significant correlation between serum hepcidin and IL-6 in SLE patients. Moreover, both of them were correlated with SLEDAI and ESR and negatively correlated with hemoglobin. The mean value of serum hepcidin in SLE patients with normocytic normochromic anemia was higher than that in patients with microcytic hypochromic anemia. However, this difference did not reach a statistically significant level.ConclusionHigh serum IL-6 and hepcidin levels are associated with anemia in SLE. They are correlated with each other and with disease activity. Although our study revealed serum hepcidin to be correlated with disease activity, it should not be used as a marker of disease activity in SLE patients as our patient’s group was SLE patients suffering from ACD. However, IL-6 inhibition should be considered in patients with SLE with anemia to guide the control of anemia of chronic diseases resulting from cytokine production as a result of high disease activity in SLE patients. It should be noted that the occurrence of ACD associated with IL-6 flare up could be a player in other systemic rheumatic diseases and is not specific to SLE patients.

Collagen triple helix repeat containing-1: a novel biomarker associated with disease activity in Systemic lupus erythematosus.

The objective of this article is to investigate whether the aberrant expression of collagen triple helix repeat containing-1 (CTHRC1) from patients with systemic lupus erythematosus (SLE) could contribute to the pathogenesis of lupus. We divided SLE patients into active groups (Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score ≥ 6) and inactive groups (SLEDAI score < 6). Serum concentrations of CTHRC1, interferon alpha, interleukin (IL)-28A and IL-28B were determined using an enzyme-linked immunosorbent assay in a group of 40 patients with SLE. Results were compared with those from 23 healthy controls. Serum CTHRC1 protein levels were higher in patients with SLE compared with healthy controls. Patients with active disease displayed higher CTHRC1 levels compared with those with inactive disease as well. There was a positive association between serum CTHRC1 levels and SLEDAI and erythrocyte sedimentation rate, and a negative correlation with complement 3 and 4. Moreover, serum CTHRC1 levels were higher in SLE patients with arthritis and anemia compared with patients without the above-mentioned manifestations. These findings indicate CTHRC1 probably plays an important part in the pathogenesis of SLE, and is positively associated with disease activity, while it also likely refers to the development of arthritis and anemia in SLE. Therefore, CTHRC1 may provide a novel research target and shed new light on the pathogenesis and therapy of SLE.

Aetiology and outcome of anemia in patients with systemic lupus erythematosus

Background: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder that is greatly subject to the combined effect of genetic, environmental, demographic and geographical factors. Hematological manifestations are very common in SLE, with many patients presenting with anemia. The cause of Anemia could be varied, with Autoimmune hemolytic anemia, anemia of chronic disease, iron deficiency being the common causes. The aim of the present study was to estimate the proportion of patients with prevalence of different causes of anemia in SLE and it‘s association between immunological and clinical parameters and to correlate the severity of anemia with SLEDAI score and SLICC/ACR score.Methods: This was an observational and prospective study conducted on 52 patients satisfying ACR criteria for SLE and WHO definition of anemia. All patients underwent baseline investigations for hematological, biochemical parameters and immunological investigations for C3 and C4. Other special investigations were done as per the treating rheumatologists’ opinion. Patients were followed up after three months to evaluate the response to therapy.Results: In this study, most of the patients were in the age group between 20-50 years (94.22%) and female:male ratio was 13:1. At presentation 55.76% patients had severe anemia, 38.46% had moderate anemia and 5.78% had mild anemia. After therapy (three months) only 3.84% patients had severe anemia. The most common cause of anemia was AIHA (38.46%). Mean SLEDAI score at presentation was &gt;20 but after three months therapy the score was reduced to 4. There was no correlation between aneamia and SLICC/ACR damage index.Conclusions: Anaemia usually occurs at the onset of SLE and its recurrence rate will become low after three months of therapy. SLEDAI scores, SLICC/ACR damage index and serum complement levels (C3 and C4) acts as good indices for assessment and follow up of SLE.

Aplastic anemia in systemic lupus erythematosus: A better prognosis acquired aplastic anemia

Aplastic anemia (AA) is an uncommon hematologic manifestation of systemic Lupus erythematosus (SLE) with significant difference in the course and outcome from other forms of acquired AA due to underlying immune mediated mechanism of the disease. Here we report a case of SLE with thrombocytopenia at presentation and later evolved to develop pancytopenia with bone marrow aplasia. You can submit your Manuscripts at: https://symbiosisonlinepublishing.com/submitManuscript.php

Autoimmune hemolytic anemia in systemic lupus erythematosus at diagnosis: differences between pediatric and adult patients.

Objective To determine the overall prevalence of autoimmune hemolytic anemia (AIHA), and to compare clinical and laboratory features in a large population of children and adult lupus patients at diagnosis. Methods This retrospective study evaluated the medical charts of 336 childhood-onset systemic lupus erythematosus (cSLE) and 1830 adult SLE (aSLE) patients followed in the same tertiary hospital. Demographic data, clinical features and disease activity were recorded. AIHA was defined according to the presence of anemia (hemoglobin <10 g/dL) and evidence of hemolysis (reticulocytosis and positive direct antiglobulin test (DAT)/Coombs test) at SLE diagnosis. Evans syndrome (ES) was defined by the combination of immune thrombocytopenia (platelet count <100,000/mm3) and AIHA. Results The frequency of AIHA at diagnosis was significantly higher in cSLE patients compared to aSLE (49/336 (14%) vs 49/1830 (3%), p = 0.0001), with similar frequency of ES (3/336 (0.9%) vs 10/1830 (0.5%), p = 0.438). The median of hemoglobin levels was reduced in cSLE vs aSLE patients (8.3 (2.2-10) vs 9.5 (6.6-10) g/dL, p = 0.002) with a higher frequency of multiple hemorrhagic manifestations (41% vs 7%, p = 0.041) and erythrocyte transfusion due to bleeding (24% vs 5%, p = 0.025). cSLE patients also had more often constitutional involvement (84% vs 31%, p < 0.001), fever (65% vs 26%, p < 0.001), weight loss > 2 kg (39% vs 6%, p < 0.001), reticuloendothelial manifestations (48% vs 8%, p < 0.001), hepatomegaly (25% vs 2%, p < 0.001) and splenomegaly (21% vs 2%, p = 0.004). Other major organ involvements were common but with similar frequencies in cSLE and aSLE ( p > 0.05). Median systemic lupus erythematosus disease activity index 2000 (SLEDAI-2 K) was comparable in cSLE and aSLE (p = 0.161). Conclusions We identified that AIHA was not a common condition in cSLE and aSLE, with distinct features characterized by a higher prevalence/severity in children and concomitant constitutional symptoms in the majority of them.

Osteopontin circulating levels correlate with renal involvement in systemic lupus erythematosus and are lower in ACE inhibitor-treated patients.

Elevated serum levels of osteopontin have been associated with cardiovascular disease, diabetic nephropathy, and autoimmune disease activity. Aim of the study was to investigate the relationship between osteopontin serum levels and renal damage in a population of patients with systemic lupus erythematosus (SLE). Osteopontin serum levels were analyzed in 101 SLE patients and compared to those of 115 healthy controls. Associations between osteopontin levels and renal involvement, disease activity and damage index, biochemical parameters, and therapy were assessed. Overall osteopontin serum levels were higher in SLE patients (median, 17.93 ng/mL; interquartile range, 8.13-35.07 ng/mL) than in healthy controls (median, 5.62 ng/mL; interquartile range, 2.61-13.83 ng/mL). Univariate logistic analysis among cases showed that high osteopontin levels (higher vs medium-lower tertile) were associated with renal involvement (p = 0.012), renal function (p = 0.007), proteinuria (p = 0.011), anemia (p < 0.001), and SLICC/ACR Damage Index (p < 0.001). Multivariate analysis showed an independent association between high osteopontin serum levels (higher vs medium-lower tertile) and chronic kidney disease (OR = 4.89; 95 % CI, 1.24-19.24; p = 0.008), proteinuria (OR = 4.56; 95 % CI, 1.15-18.04; p = 0.027), anemia (OR = 4.66; 95 % CI, 1.25-17.43; p = 0.008), and use of renin-angiontensin system antagonists (OR = 0.234; 95 % CI, 0.06-0.98; p = 0.047). This study shows that elevated osteopontin serum levels significantly correlate with renal involvement and anemia in SLE. Moreover, it suggests that renin-angiontensin system antagonists decrease osteopontin levels-this effect is consistent with the inhibitory effect of these drugs on osteopontin renal expression, detected in animal models by other authors, and may provide a new rationale for their employment.

An uncommon cause of anemia in systemic lupus erythematosus

An uncommon cause of anemia in systemic lupus erythematosus

Protocolo diagnóstico de la anemia en el lupus eritematoso sistémico

In patients with SLE, it is common to observe blood disorders in the clinical course of the disease, and sometimes may be the initial presentation of the disease. Anemia is the most common, in relation to the disease itself or triggered by intercurrent unrelated to the autoimmune process.The anemia of chronic disorders (anemia inflammatory) is the most frequent type of anemia in systemic lupus erythematosus, and normochromic normocytic usually is related to the disease activity. Hemolytic anemia occurs in 10% of patients and is mediated by IgG antibodies hot (sometimes IgA) with positive Coombs test immunoglobulin and complement. Other types of anemia in SLE, are iron deficiency anemia, autoimmune hemolytic anemia, microangiopathic hemolytic anemia, anemia pure red cell aplasia and autoimmune anemias not secondary to intercurrent digestive origin, gynecological, deficiency states, drugs used in the treatment SLE, toxic exogenous infections and neoplasms.

Autoimmune hemolytic anemia in systemic lupus erythematosus: association with thrombocytopenia

Hematological disturbances are common in systemic lupus erythematous (SLE). Specifically, autoimmune hemolytic anemia (AHA) may manifest in SLE patients at the time of diagnosis or within the first year of the disease. AHA is often associated with thrombocytopenia, lupus nephritis, and central nervous system activity. In this study we investigated these associations in Brazilian patients with SLE. Forty-four consecutive SLE patients who had a history of AHA were age, gender, and disease duration matched with 318 SLE patients without AHA who formed the control group. All patients fulfilled the revised American College of Rheumatology criteria for SLE and were followed-up within our Service. Clinical and laboratorial manifestations were similar in both groups, except for the predominance of leukopenia, thrombocytopenia, and anti-dsDNA on univariate analysis in the AHA group. The multivariate logistic regression model revealed risk only for thrombocytopenia in the AHA group compared to the control group (odds ratio, 2.70; 95% confidence interval, 1.32-5.50). Our results corroborate previous data that AHA in SLE increases the risk of thrombocytopenia in individuals with SLE. This association suggests a common mechanism in AHA and SLE pathophysiologies.

Familiality and co-occurrence of clinical features of systemic lupus erythematosus.

To evaluate familiality of 15 clinical and laboratory features in systemic lupus erythematosus (SLE)-affected sibpairs, and to estimate correlations with the age at SLE diagnosis in affected sibpairs and parent-offspring pairs. Concordance rates and sibling risk ratios were used as indicators of familiality for 15 manifestations of SLE. Pearson's correlations and paired t-tests were used to compare the age at SLE diagnosis in affected sibpairs and in parent-offspring pairs. Increased sibling risk ratios (1.9-3.9) for thrombocytopenia, discoid rash, neurologic disorder (defined as seizure or psychosis), and hemolytic anemia were observed in 159 SLE-affected sibpairs. Among these clinical features, paired expression of hemolytic anemia plus thrombocytopenia and hemolytic anemia plus neurologic disorder appeared to be more frequent in 709 SLE patients than would be expected by chance (P < 0.00001 and P < 0.007, respectively). The ratio of the presence of both hemolytic anemia and neurologic disorder was approximately 13 times higher in the younger affected sib than in the older affected sib (P < 0.02). Familiality of patient age at SLE diagnosis, as observed by relative correlations, was greater in 125 affected sibpairs (r = 0.67, P < 0.0001) than in 37 affected parent-offspring pairs (r = 0.47, P = 0.003). The median +/- SD age at SLE diagnosis was significantly lower in offspring (21.5 +/- 10.1 years) than in their parents (41.6 +/- 15.8 years) (P < 0.0001) but was not different in sibpairs. The combined non-Caucasian sibpairs had a younger mean age at SLE diagnosis compared with Caucasian sibpairs (P = 0.014). Evidence for familiality of thrombocytopenia, discoid rash, neurologic disorder, hemolytic anemia, and co-occurring neurologic disorder plus hemolytic anemia in SLE was observed in 159 affected sibpairs. Familiality of the age at SLE diagnosis in relative pairs suggests that shared genes and/or shared environmental exposures impact disease susceptibility. Shared immediate environmental triggers appear less compelling, because the average time between dates of diagnosis was 11 years in parent-offspring pairs and 7.5 years in affected sibpairs. The significantly earlier age at disease diagnosis in offspring compared with their parents suggests that some forms of anticipation might play a role in susceptibility to SLE. Stratifying families by subphenotypes that are familial may reduce heterogeneity and facilitate identification of genetic risk factors for SLE.

Aplastic Anemia in Systemic Lupus Erythematosus: A Distinct Presentation of Acquired Aplastic Anemia?

Aplastic anemia is a rare but serious complication of systemic lupus erythematosus (SLE) with an often dramatic and unanticipated onset. The peripheral destruction of formed blood elements, which frequently accompanies the syndrome, may obscure or delay the diagnosis of bone marrow suppression, and the number of published cases may be an underestimate of the actual incidence of the disease. Furthermore, the disease course may differ significantly from other forms of acquired aplastic anemia and seems to carry a more favorable prognosis once effectively diagnosed and treated. In addition, aplastic anemia may precede other manifestations of SLE. Therefore, the possibility of bone marrow aplasia should be excluded in all SLE patients with severe pancytopenia, and conversely, the diagnosis of SLE should be explored in cases of aplastic anemia. Two patients with aplastic anemia in SLE, one with aplastic anemia preceding the onset of SLE, are described along with 15 cases reviewed from the English language literature. The presentation, prognosis, treatment, and pathogenesis of aplastic anemia complicating SLE are discussed. Recognition that cytopenias, especially pancytopenia, may occur on the basis of inhibited myelopoesis rather than peripheral destruction as either a harbinger of SLE or as a manifestation of disease flare is important. This knowledge will prompt the astute clinician to obtain screening antinuclear antibodies in the setting of otherwise unexplained bone marrow acellularity or, given the prognosis of SLE associated aplastic anemia, give early consideration to more aggressive immunosuppression.

Thrombotic microangiopathic hemolytic anemia in systemic lupus erythematosus associated with antiphospholipid antibodies: usefulness of monitoring coagulation–fibrinolysis markers

Thrombotic microangiopathic hemolytic anemia in systemic lupus erythematosus associated with antiphospholipid antibodies: usefulness of monitoring coagulation–fibrinolysis markers

Thrombotic microangiopathic hemolytic anemia in systemic lupus erythematosus associated with antiphospholipid antibodies: usefulness of monitoring coagulation–fibrinolysis markers

Abstract We report on a 24-year-old woman with systemic lupus erythematosus and lupus anticoagulant who developed chronic thrombotic microangiopathic hemolytic anemia. The patient responded well to a combination of plasma exchange and anticoagulant therapy. Changes in the molecular markers for coagulation and fibrinolysis corresponded with the disease activity. We suggest that thrombotic microangiopathic hemolytic anemia should be suspected when anemia and thrombocytopenia of unknown etiologies occur in systemic lupus erythematosus. In such cases, the evaluation of molecular markers for coagulation and fibrinolysis might be helpful both for diagnosis and for assessing the response to therapy.

Une observation de rémission post-partum d'une dysérythropoïèse acquise, une cause rare d'anémie au cours du lupus érythémateux disséminé

Amongst the various causes of anemia in systemic lupus erythematosus, isolated and acquired erythrocyte dysplasia is rare and most often part of global dysmyelopoiesis. The authors report a case of acquired erythrocyte dysplastic syndrome that occurred in a 34-year-old woman in whom previous diagnosis had evidenced systemic lupus erythematosus of rather benign course. Other causes of dysmyelopoiesis were ruled out. Myeloid stem cell cultures showed selective inhibition of erythroid cells growing, with no particular effect of the patient's serum. While a corticosteroid treatment with prednisone (1 mg/kg/d) did not show any efficacy upon anemia, the patient's pregnancy was followed by prolonged correction of hemoglobin, making possible the tapering of prednisone down to 10 mg/d. Acquired erythrocyte dysplastic syndrome remains a rare cause of anemia in systemic lupus erythematosus. This case report suggests an immunological phenomenon, but the mechanisms underlying both the appearance and long-lasting remission after pregnancy remain unexplained.