Thrombocytopenia In Systemic Lupus Erythematosus Research Articles

Prognostic Factors for Chronic Thrombocytopenia in Systemic Lupus Erythematosus with Immune Thrombocytopenia.

We aimed to identify the clinical characteristics and risk factors for chronic immune thrombocytopenia (ITP) in patients with systemic lupus erythematosus (SLE). We retrospectively reviewed patients diagnosed with SLE-associated ITP between January 2000 and December 2021. Patient characteristics were analyzed according to the progression of chronic thrombocytopenia. No response was defined as a platelet count <30 × 109/L or less than double the baseline count after treatment. Factors associated with chronic ITP were evaluated by logistic regression analysis. Among the 121 patients with SLE-associated ITP, 27 progressed to chronic ITP lasting more than 1 year after initial diagnosis. The median initial platelet count was significantly lower in patients with chronic thrombocytopenia than in those without the disease (16 vs. 51 × 109/L). Patients who did not achieve a response within 1 month of treatment exhibited a high probability of progressing to chronic ITP (55.6 vs. 22.3%, p < 0.001). Multivariable analysis revealed that severe thrombocytopenia at baseline (<20 × 109/L) (adjusted odds ratio [aOR] = 13.628, 95% confidence interval [CI] = 3.976-46.791) and no response within 1 month (aOR = 9.171, 95% CI = 2.776-30.298) were significantly associated with the risk of progression to chronic ITP in patients with SLE. Approximately one-quarter of the patients with SLE-associated ITP progressed to chronic ITP. Severe thrombocytopenia and failure to achieve a response within 1 month were risk factors for the development of chronic ITP in those patients.

Immune thrombocytopenia in systemic lupus erythematosus: Prevalence, risk factors, and a novel predictive model for risk assessment

IntroductionImmune thrombocytopenia (ITP) is a potentially severe manifestation of systemic lupus erythematosus (SLE) reported in 7–40% of SLE patients. ITP has been associated with a higher risk of organ damage and mortality. ObjectivesTo describe which factors are associated with the presence of ITP in SLE patients. MethodsRetrospective case–control study. Cases were defined as SLE patients who had ever developed ITP and were sex- and age-matched with two controls. A predictive model was constructed to identify SLE patients who were at risk of developing ITP. ResultsITP prevalence in our SLE cohort was 8.35%. Cases had a higher frequency of hemolytic anemia, while controls had a higher prevalence of arthritis at SLE diagnosis. During SLE progression, cases tested positive for anticardiolipin, anti-β2-glycoprotein 1, and lupus anticoagulant antibodies more frequently. Cases received mycophenolic acid and azathioprine more often than controls and had a higher SLICC/ACR score. The model demonstrated a sensitivity of 87.53%, a positive predictive value of 81.92%, a specificity of 80.50%, area under the curve of 83.92%, a F1 of 83% and an overall accuracy of 83.68%. The variables that best explain the model were hemolytic anemia, arthritis, oral ulcers, Raynaud's phenomenon, low C4, low CH50, anticardiolipin and anti-β2GP1 antibodies. ConclusionSLE patients who develop ITP have a distinct phenotype characterized by more hemolytic anemia and less arthritis at SLE onset, and higher prevalence of antiphospholipid syndrome antibodies during SLE progression. This phenotype is associated with heightened organ damage and the need for more intensive therapies and stricter follow-up. Our predictive model has demonstrated an impressive ability to identify SLE patients at risk of developing ITP.

Autoimmune Thrombocytopenia in SLE and COVID-19.

Thrombocytopenia and hypercoagulopathy are haematological abnormalities commonly seen in individuals with coronavirus disease 2019 (COVID-19) and systemic lupus erythematosus (SLE). The difficulty arises when the patient has both diseases concurrently. The clinician should be able to comprehend the pathophysiology of these patient abnormalities in order to provide the best treatment possible. We present a case of a 20-year-old female COVID-19 patient with a history of SLE who had thrombocytopenia but normal D-dimer results. Our analysis revealed that the thrombocytopenia may have been caused by a relapse of lupus, not by COVID-19 infection. In this case, glucocorticoids were the primary therapy and produced excellent results.LEARNING POINTSThe pathophysiology of thrombocytopenia in a patient with concurrent COVID-19 and SLE should not always be associated with platelet consumption.Low-dose glucocorticoids should be administered, with infection risk and comorbidities taken into consideration.Glucocorticoid therapy may result in a delay in viral clearance.

Refractory Thrombocytopenia in SLE with Evans Syndrome : A Case Report

Background: Evans syndrome (ES) is a rare autoimmune disorder of unknown etiology. It is characterized by the occurrence of two or more hematologic immune cytopenias, most often immune thrombocytopenia and autoimmune hemolytic anemia (AIHA). In this study we present a rare case of a 9-year-old girl, who was diagnosed with secondary ES associated with active systemic lupus erythematosus (SLE). Case presentation: On November 16th, 2018, a 9-year old girl, presented for the first time with recent onset of gum bleeding and decreased level of consciousness for two weeks. On examination, she was conscious but not oriented, unable to walk, showed scanty hair with oral and gum bleeding, and had multiple bruises and massive hepatosplenomegaly. No parental consanguinity, and no similar history in the family was noted. She had low platelet count (1×103 /μL), with high mean platelet volume (19 fl), anemia (HB 5.5 g/dL), with normal white blood cell count, positive direct Coombs test, and hemolysis with no malignant cells on a peripheral blood smear. Bone marrow evaluation showed hypercellularity, and increased number of megakaryocytes, with no other significant abnormalities. Her flowcytometry study was normal. Brain computed tomography (CT) revealed left subdural hematoma with elements of old brain atrophy (Photo 1), with significant dilatation of ventricular system. Chest X-ray showed mild right pleural effusion. Abdominal ultrasonography showed hepatosplenomegaly with no focal lesions, and mild-to-moderate ascites. Both antinuclear antibody and anti-phospholipid antibody tests showed positive results and C3 was low. Hepatitis and human immunodeficiency virus (HIV) serology were negative.Autoimmune lymphoproliferative syndrome gene sequence analysis was negative. The patient was diagnosed with SLE, after she fulfilled the American Rheumatology Association criteria, associated with Evans syndrome. Any planned surgery was postponed until thrombocytopenia correction, and their aim was to increase the platelet count to a level of above 100.The patient received packed red blood cells (RBCs) and platelets several times. To control her condition, several medications were tried. These medications included intravenous immunoglobulin (1 gm/kg) for two successive days, prednisolone (2 mg/kg/day); however, no improvement was observed after 10 days. Therefore, all of these medications were replaced with methylprednisolone pulse therapy (30 mg/kg for three days), with one dose of anti-D (75 mcg/kg IV). Our next choice was mycophenolate mofetil, which was stopped after finishing the course of five weeks, but the patient did not show any improvement. Moreover, platelets failed to respond to four doses of Rituximab (370 mg/m2/dose) for four weeks and to two cycles of high dose of dexamethasone 20mg/m2 for 4 days every 4 weeks. On 12/29/2018, chloroquine (4 mg/kg/day) was administered for two weeks in conjunction with cyclosporine (4 mg/kg/day). Splenectomy was an option but was not performed because her platelet levels never recovered. Her anemia started to improve on 1/9/2019 with combination of prednisolone and cyclosporine and Coombs test results were negative. On 1/23/2019, the patient was started on Eltrombopag, in a dose-escalated manner starting with 25 mg/kg/day up to 75 mg/kg/day in addition to corticosteroids. One week later, her platelet counts markedly improved (Photo 2). No major side effects were reported. On 2/12/2019, the patient underwent left side burr-hole evacuation of subdural hematoma, but her consciousness was never regained. She was maintained in intensive care unit and ventilated, but she developed multiple postoperative complication, including sepsis with high inflammatory markers and spiking fever. Her platelet count was continuously high, so the dose of Eltrombopag was decreased to 50 mg/kg/day to just keep the level between 50 and 100. Despite all of therapeutic interventions, she passed away one month later, on 3/13/2019, due to respiratory failure. Conclusion: The incidence of ES is quite rare, especially in children. Eltrombopag is a safe and effective drug for management of refractory thrombocytopenia in cases of ES associated with SLE. Figure Disclosures No relevant conflicts of interest to declare.

Prevalence and outcome of thrombocytopenia in systemic lupus erythematous: single-centre cohort analysis

We aimed to characterise the frequency of thrombocytopenia in systemic lupus erythematosus (SLE) and determine its time of onset during the course of the disease, and its severity and impact on mortality. This was a single-centre cohort analysis of 707 patients with SLE followed for up to 40 years. We reviewed the patients' clinical notes identifying the presence of thrombocytopenia, its time of onset and ascertained other clinical and serological features of the disease. Thrombocytopenia was classified as mild (100-149x109/L), moderate (31-99x109/L) or severe (≤30x109/L platelets). It was also classified as asymptomatic, with minor bleeding or with major bleeding. 22.9% of patients (n=162) had thrombocytopenia prior to or during the course of SLE. Twenty-three patients (14.2%) had isolated immune thrombocytopenia (ITP) before the diagnosis of SLE. Median follow-up time was 19 years (IQR=13). Most patients (n=67, 41.4%) had mild thrombocytopenia. More than half the patients (n=98, 60.5%) developed asymptomatic thrombocytopenia and only 6 patients (3.7%) had major bleeding events in the context of thrombocytopenia. The development of severe thrombocytopenia any time during the course of SLE was associated with an increased risk of death (HR=3.57, p=0.025). Anti-phospholipid syndrome was over twice as common in patients with thrombocytopenia in the cohort. There is an increased risk of death for male patients (HR=3.41, p=0.036) who develop thrombocytopenia and for those who present with concomitant haemolytic anaemia (HR=3.07, p=0.027). The presence of severe thrombocytopenia (platelets ≤30x109) in patients with SLE is associated with an increased risk of death, regardless of bleeding events. Male patients with SLE and thrombocytopenia have an increased mortality risk, as have those who develop concomitant thrombocytopenia and haemolytic anaemia.

Antiphospholipid antibodies and the risk of thrombocytopenia in patients with systemic lupus erythematosus: A systematic review and meta-analysis.

According to criteria for the classification of Systemic Lupus Erythematosus (SLE), thrombocytopenia is one of the disease-defining hematologic disorders. Since the recognition of Antiphospholipid Syndrome (APS), thrombocytopenia was frequently reported but several studies yielded contradictory results on the association between aPL-positivity and thrombocytopenia. We evaluated the role of antiphospholipid antibodies (aPL) and different aPL profiles on the risk of thrombocytopenia in SLE patients by conducting a systematic review and meta-analysis of available literature from 1987 to 2018. MEDLINE, EMBASE, Cochrane Library, congress abstracts, and reference lists of eligible studies were searched. Studies were selected if they included SLE patients with descriptions of the exposure to aPL and the outcomes (thrombocytopenia). Two reviewers extracted study characteristics and outcome data from published reports. Estimates were pooled using random effects models and sensitivity analyses. We followed the PRISMA guidelines for all stages of the meta-analysis. PROSPERO registration number: CRD42015027378. From 3278 articles identified, 53 studies met inclusion criteria amounting to 9019 SLE patients. Twenty-nine percent of aPL-positive SLE patients had thrombocytopenia compared to 15.1% in aPL-negative SLE patients. The overall pooled Odds Ratio (OR) for thrombocytopenia in aPL positive patients was 2.48 (95% CI; 2.10-2.93). Among aPL subtypes, the risk of thrombocytopenia was highest for lupus anticoagulant (OR = 3.56 [95% CI, 2.57-5.25]), IgM anti-β2-GP1(OR = 2.87 [95% CI; 2.57-5.25]), IgG and IgM anticardiolipin antibodies (OR = 1.87 [95% CI; 1.52-2.31] and OR = 1.73 [95% CI; 1.36-2.19] respectively). The occurrence of thrombocytopenia was strongly determined by various aPL profiles in SLE patients. While the association between IgM antibodies and other APS manifestations including thrombosis is debated, IgM isotypes are helpful in the risk stratification of thrombocytopenia in SLE.

A study on hematological and immunological profile of patients with systemic Lupus Erythematosus

Background: Systemic lupus erythematosus (SLE) is a chronic, relapsing, inflammatory and often febrile multisystem disorder of the connective tissue, characterized principally by involvement of the skin, joints, kidneys, and serosal membranes. Hematological findings may be present at the onset of the disease or can develop during the course of the disease. Since blood and blood vessels together contain more diverse number of antigens than any other organ in the body, it is only natural to expect hematological manifestations more often than the other systems. Objectives: To study the haematological and immunological Profile of Patients with Systemic Lupus Erythematosis. Methodology: An Ambispective study was conducted at Kasturba Medical College and hospital, Manipal from January 2011 to December 2013. A total of One hundred twenty seven cases of SLE were classified as having SLE according to the revised American College of Rheumatology (ACR) classification criteria (1997). Results: Hematological manifestations were frequently seen in the cases studied, which included anemia, neutropenia, lymphopenia, thrombocytopenia, pancytopenia, increased Erythrocyte sedimentation rate (ESR), presence of spherocytes, DCT positivity etc.Anemia was the most common hematological abnormality observed in the patients accounting to 87.4% (111/127) of cases. Hemoglobin of patients varied from a minimum of 3.8g/dl up to 13.5g/dl (Median = 9.6±2.2). The erythrocyte sedimentation rate (ESR) was assessed in all except one.The PCNA (Anti proliferating cell nuclear antigen) antibodies were positive in 44% of the cases. Conclusion: Hematological abnormalities are the commonest among all other anifestations in SLE, and their treatment is challenging. Bone marrow examination should be considered in all cases of severe or persistent leukopenia or thrombocytopenia in SLE, to exclude dug-induced myelotoxicity in susceptible patients.

Retrospective analysis of the risk of hemorrhage associated with moderate and severe thrombocytopenia of 173 patients with systemic lupus erythematosus.

The aim of the study was to observe the risk of hemorrhage from moderate and severe thrombocytopenia in systemic lupus erythematosus (SLE).A retrospective analysis was undertaken of cases admitted to Qilu Hospital, China. Blood platelet counts (BPCs) of ≤20 × 109/L represent severe thrombocytopenia, and a BPC of 21 to 50 × 109/L indicates moderate thrombocytopenia. A comparison was made from the perspective of severity with a view to determine the influence of thrombocytopenia on the risk of hemorrhage and the results.Moderate and severe thrombocytopenia occurred in 173 cases, accounting for 15.2% of the total hospitalized patients with SLE with a male to female ratio of 1:23.7. The average age of those patients was 34.8 ± 14.6 years. In the group of severe thrombocytopenia, patients without visceral involvement had a mean age of onset of 31.4 ± 14.2 years with a median of 28.0 years compared with 37.8 ± 14.8 years with a median of 38.5 years for patients with visceral involvement; this difference was statistically significant (P = .034). Seventy-one (76.3%) of 93 patients with severe thrombocytopenia and 20 (25.0%) of 80 patients with moderate thrombocytopenia developed hemorrhagic conditions of various grades, the difference between both were markedly statistically significant. Twenty-three patients with SLE died. Nine deaths were due to a hemorrhage caused by thrombocytopenia, while more were caused by infection.Severe thrombocytopenia is a significant adverse prognostic factor of SLE. SLE with the main manifestation of thrombocytopenia tends to make younger visceral organ owners suffer.

Thrombocytopenia in Systemic Lupus Erythematosus: Clinical Manifestations, Treatment, and Prognosis in 230 Patients.

The aim of the study was to examine the clinical characteristics and prognosis according to severity of thrombocytopenia and response to treatment for thrombocytopenia in patients with systemic lupus erythematosus (SLE).We retrospectively evaluated 230 SLE patients with thrombocytopenia, and reviewed their clinical data and laboratory findings. Thrombocytopenia was defined as platelet counts under 100,000/mm3, and patients were divided into 3 thrombocytopenia groups according to severity: mild (platelet counts >50,000/mm3), moderate (>20,000/mm3, ≤50,000/mm3), and severe (≤20,000/mm3). Clinical characteristics, treatments, and prognoses were compared among the groups. Furthermore, complete remission of thrombocytopenia was defined as platelet counts >100,000/mm3 after treatment.There was no significant difference in clinical or laboratory findings among the groups according to severity of thrombocytopenia. However, hemorrhagic complications were more frequent in severe thrombocytopenia (P < 0.001) and mortality was also higher (P = 0.001). Complete remission was achieved in 85.2% of patients. The clinical characteristics and modality of treatment did not differ between the patients with and without complete remission. Mortality in patients with complete remission (1.5%) was significantly lower than in those without complete remission (29.4%, P < 0.001). Survival was significantly higher in patients with complete remission from thrombocytopenia (odds ratio = 0.049, 95% confidence interval: 0.013–0.191, P < 0.001).The severity of thrombocytopenia in SLE patients can be a useful independent prognostic factor to predict survival. Moreover, complete remission of thrombocytopenia after treatment is an important prognostic factor. The severity of thrombocytopenia and response to treatment should be closely monitored to predict prognosis in SLE patients.

Clinical features and outcome of pregnancy with SLE-associated thrombocytopenia

Objective: To analyze the course of maternal diseases and compare pregnancy outcomes in patients with systemic lupus erythematosus (SLE)-associated thrombocytopenia to patients without.Methods: Medical charts of 77 pregnancies in 73 SLE patients were systematically reviewed. Patients were divided into two groups according to the presence or absence of thrombocytopenia. Patients who are new onset SLE during pregnancy were also been studied.Result: Thrombocytopenia was found in 18 (23.3%) of the pregnancies. SLE patients with thrombocytopenia during pregnancy had higher percentage of disease flaring (11/18 versus 14/59, p = 0.003) and SLE-Pregnancy Disease Activity Index (7.89 ± 6.192 versus 2.41 ± 3.3.89, p = 0.001) compared to patients without. Also, patients with thrombocytopenia had a higher percentage of pulmonary, cardiac and multiple organ system involvement. There was a statistically significant difference in preeclampsia and early onset hypertensive disorder induced before 34 weeks as well as the rate of live birth less than 34 weeks (33.3% versus 6.8%, p = 0.003 & 38.9% versus 13.6%, p = 0.018 & 16.7% versus 1.7%, p = 0.038). Patients with thrombocytopenia suffered from higher rate of pregnancy loss (22.2% versus 3.4%, p = 0.024) and neonatal death (33.3% versus 1.7%, p = 0.000). In our study there were 17 patients with new-onset of SLE during pregnancy. The hematological system manifestation occurred in all of them and there was a significant increase in the incidence of thrombocytopenia (n = 12, 70.6%).Conclusion: Thrombocytopenia in SLE during pregnancy indicates higher disease activity, severe organ damage, early onset preeclampsia and higher pregnancy loss.

Steroid-resistant autoimmune thrombocytopenia in systemic lupus erythematosus treated with rituximab.

Systemic Lupus Erythematosus (SLE) is a multisystem disorder characterized by production of numerous autoantibodies, some of which have pathogenic consequences and result in considerable morbidity. Herein, we present a case of 48-year-old female with SLE having autoimmune hemolytic anemia, autoimmune thrombocytopenia, renal involvement, and recurrent flares of skin manifestations. She did not respond to the conventional therapy and was controlled and treated with Rituximab, a chimeric, monoclonal antiCD20 antibody, which specifically depletes B lymphocytes.

Impact of the leucocyte immunoglobulin-like receptor A3 (LILRA3) on susceptibility and subphenotypes of systemic lupus erythematosus and Sjögren's syndrome

BackgroundRecently, our research group identified the non-deleted (functional) leucocyte immunoglobulin-like receptor A3 (LILRA3) as a new genetic risk for rheumatoid arthritis.ObjectivesTo further investigate whether the functional LILRA3 is a new...

AB0500 B-Cell Depleting Therapy for SLE Related Refractory Thrombocytopenia

BackgroundAutoimmune thrombocytopenia is a common manifestation of systemic lupus erythematosus (SLE) which may accompany bleeding risk and complications. B cell depleting therapy has been tried experimentally in SLE related thrombocytopenia...

OP0216 The Functional Rather than Nonfunctional LILRA3 Contributes to Susceptibility and Subphenotypes of Systemic Lupus Erythematosus and SjÖGren's Syndrome

BackgroundSeveral studies have reported that the 6.7-kb LILRA3 deletion was associated with multiple sclerosis (MS) and SS in Caucasians [1-3]. However, whether the 6.7-kb LILRA3 deletion also contributes to autoimmune...

Evaluation of Thrombocytopenia in Systemic Lupus Erythematosus and Correlation with Different Organs Damages

Introduction:Thrombocytopenia is highly prevalent among patients with Systemic Lupus Erythematous(SLE) and at the same time it has been reported that a correlation exists between Thrombocytopenia and organ damage. The aim of this study is to highlight the correlation between Thrombocytopenia and the clinical manifestations of SLE.Objectives:The objective is to show the clinical manifestations and organ damage of Systemic Lupus Erythematous (SLE) patients who have been found to have Thrombocytopenia.Methods:A retrospective study was conducted examining all patient charts diagnosed and treated for SLE at the Rheumatology Service of Mother Teresa Hospital Centre. All the data were collected from discharged patient charts. The data included were Anti DNA,AAN,C3 , thrombocytopenia, leucopenia, and organ damage. Data were taken from 2009 to 2013. The classification criteria of the American College of Rheumatology was used for all patients regarding the diagnosis.Results:Out of 330 patients, 12 (3.64%) are men and 318 (96.3%) women. 73 of all patients have thrombocytopenia as cases and 257 patients had SLE without thrombocytopenia, which was considered as the control group. AAN 68(93.1%), Anti DNA 50 (64.3%) , low value of C3 46 (63%), and leucopenia were higher in thrombocytopenic patients compared with control group (p<0.05) 48 (65.7%) of thrombocytopenic patients develop lupus nephritis, 10 (13.6%) were with pulmonary involvement, and 42 (57.5%) had leukopenia.Conclusion:Thrombocytopenia is not directly associated with any disease activity, organ damage and mortality, but it should be considered as a prognostic factor which may help identifying a category of patients whose disease course can be aggravated.

Splenectomy in Systemic Lupus Erythematosus with Refractory Thrombocytopenia: Efficacy for Thrombocytopenia and Effects on Lupus Disease Activities

The precise role of splenectomy in the treatment of refractory thrombocytopenia associated with systemic lupus erythematosus (SLE) remains a matter of debate. We retrospectively investigated the efficacy of splenectomy in 10 SLE patients who received splenectomy for thrombocytopenia and compared their lupus disease activity before and after operation. Our study showed that the response of thrombocytopenia to splenectomy achieved complete remission in 5 (50%) patients and partial improvement in 3 (30%) patients and the response sustained in the long-term follow-up period. However, compared with disease activity prior to splenectomy, we observed that lupus flare occurred in 5 (50%) patients at 6 months, and in another 4 (40%) patients at 12 months after operation. Kidney was the most frequently affect organ in flare. Surprisingly, among the 10 patients, two developed typical rheumatoid arthritis later. Thus we concluded that splenectomy was safe and effective in the treatment of refractory thrombocytopenia in SLE. Physician should be alert to disease flare and the development of rheumatoid arthritis in the post operative course.

Anti B-cell therapy against refractory thrombocytopenia in SLE and MCTD patients: long-term follow-up and review of the literature

The objective of this study was to retrospectively evaluate the clinical and immunological effects of anti-B cell treatment in patients with systemic lupus erythematosus (SLE) and mixed connective-tissue disease (MCTD) with autoimmune thrombocytopenia (AITP) refractory to conventional immunosuppressive treatment. Rituximab (RTX) was added to the ongoing treatment of 16 patients (median age 36 years, range 17-84, all female) with treatment-resistant AITP. Thirteen patients had SLE and three had MCTD. RTX was given intravenously on four occasions during four consecutive weeks at a dose of 375 mg/m(2). Clinical and laboratory disease activity variables recorded at every follow-up visit were analyzed. The median disease duration before RTX treatment was nine years (range 0.2-27) and the median post-treatment follow-up time was 28 months (range 3 to 92). Ten patients (63%) were treated repeatedly with RTX during the follow-up period. Complete depletion of B cells was achieved in 94% of cases one month after RTX treatment. A significant increase (p = 0.0001) of platelet counts was seen already after one month (median 58 × 10(9)/ml vs 110 × 10(9)/ml) whereas within three months platelet counts normalized in 10 patients (median 223 × 10(9)/ml). Three patients did not respond to RTX treatment (median platelet count 69 × 10(9)/ml). High titers of anti-platelet antibodies were detected in seven patients before RTX treatment, and the autoantibody titers decreased significantly (p < 0.03) after RTX treatment in six of these patients who also achieved complete remission. A review of the literature revealed 24 articles including 18 case reports, one retrospective cohort study and five prospective studies documenting the outcomes of 65 RTX-treated patients with SLE- or MCTD-related thrombocytopenia with an overall treatment response rate of 80%. In conclusion, these findings indicate that RTX is an additional potent therapeutic treatment option for SLE patients with AITP refractory to conventional immunosuppressive treatment whereas best response may be expected in patients with high titers of anti-platelet antibodies at baseline.

Two Cases of Refractory Thrombocytopenia in Systemic Lupus Erythematosus that Responded to Intravenous Low-Dose Cyclophosphamide

Treatment of thrombocytopenia in systemic lupus erythematosus (SLE) is considered in cases of current bleeding, severe bruising, or a platelet count below 50,000/µL. Corticosteroid is the first choice of medication for inducing remission, and immunosuppressive agents can be added when thrombocytopenia is refractory to corticosteroid or recurs despite it. We presented two SLE patients with thrombocytopenia who successfully induced remission after intravenous administration of low-dose cyclophosphamide (CYC) (500 mg fixed dose, biweekly for 3 months), followed by azathioprine (AZA) or mycophenolate mofetil (MMF). Both patients developed severe thrombocytopenia in SLE that did not respond to pulsed methylprednisolone therapy, and started the intravenous low-dose CYC therapy. In case 1, the platelet count increased to 50,000/µL after the first CYC infusion, and remission was maintained with low dose prednisolone and AZA. The case 2 achieved remission after three cycles of CYC, and the remission continued with low dose prednisolone and MMF.

Serum L-ficolin levels in patients with systemic lupus erythematosus

Objective L-ficolin plays an important role in innate immunity and is involved in apoptosis. The objective of this study was to investigate the relationship between serum L-ficolin levels and clinical manifestations in patients with systemic lupus erythematosus (SLE).Methods Serum L-ficolin levels were determined by enzyme-linked immunosorbent assay in 66 SLE patients and 50 healthy controls.Results Median serum L-ficolin levels were 5.0 and 8.7 μg/ml in SLE patients and controls, respectively (p = 0.0001). There were no significant differences in serum L-ficolin levels between the active disease group [SLE Disease Activity Index (SLEDAI) > 6] and the inactive disease group (SLEDAI < 5). Decreased serum L-ficolin levels were associated with thrombocytopenia (median of with vs. without thrombocytopenia 3.4 vs. 5.3 μg/ml, p = 0.008). There were no correlations between serum L-ficolin levels and SLEDAI, serum C3, or serum C4 levels.Conclusion The association between L-ficolin and thrombocytopenia suggests a pathogenic role for L-ficolin in thrombocytopenia in SLE.

Serum L-ficolin levels in patients with systemic lupus erythematosus

L-ficolin plays an important role in innate immunity and is involved in apoptosis. The objective of this study was to investigate the relationship between serum L-ficolin levels and clinical manifestations in patients with systemic lupus erythematosus (SLE). Serum L-ficolin levels were determined by enzyme-linked immunosorbent assay in 66 SLE patients and 50 healthy controls. Median serum L-ficolin levels were 5.0 and 8.7 μg/ml in SLE patients and controls, respectively (p = 0.0001). There were no significant differences in serum L-ficolin levels between the active disease group [SLE Disease Activity Index (SLEDAI) >6] and the inactive disease group (SLEDAI <5). Decreased serum L-ficolin levels were associated with thrombocytopenia (median of with vs. without thrombocytopenia 3.4 vs. 5.3 μg/ml, p = 0.008). There were no correlations between serum L-ficolin levels and SLEDAI, serum C3, or serum C4 levels. The association between L-ficolin and thrombocytopenia suggests a pathogenic role for L-ficolin in thrombocytopenia in SLE.