Background:Cytopenias are common in systemic lupus erythematosus (SLE), and it is fundamental to determine their etiology in order to establish an adequate therapeutic strategy.Objectives:To describe the findings in the bone marrow aspirations (BMA) and biopsies of patients with SLE and cytopenias, as well as clinical and laboratory features associated with the etiology of the hematological abnormalities.Methods:We performed a retrospective study in a third-level hospital in Mexico City. We included patients who fulfilled ACR criteria for SLE, presented with cytopenias and had a BMA and biopsy performed between 2000 and 2016. We described the main aspirate and biopsy findings, and also analyzed the final diagnosis and its association with clinical, laboratory and serological features.Results:We included 101 patients; median age was 32 years and 81.2% were women. Leukopenia (<3000 cells/µl) was found in 47.5% of patients, with 29.7% having moderate or severe neutropenia (<1000 cells/µl). Lymphopenia (<1000 cells/μl) was a common finding (71.3% of patients). Moderate-to-severe thrombocytopenia (<50 K/µl) was present in 28.7% of patients. Finally, 25.8% of patients presented with pancytopenia.Regarding bone marrow findings, there was erythroid dysplasia in 50.5% of patients, granulocytic dysplasia in 28.7% and megakaryocytic dysplasia in 16%. Myelofibrosis was found in 2.6%. An increase in plasma cells (≥5%) was found in 21.8%.In 72.3% of patients, bone marrow interpretation was conclusive. The most common diagnoses were disease activity (24.8%) and drug-associated myelotoxicity (28.7%). When compared to other etiologies, in patients with cytopenias secondary to disease activity, it was more frequent for the bone marrow be hypercellular (56 vs 23%, p=0.006) and to have increased megakaryocytes (40 vs 17.4%, p=0.048). Conversely, granulocytic dysplasia was less common in this group of patients (17.4% vs 54.3%, p=0.036)We analyzed factors associated with both activity and toxicity as final diagnoses (Table 1). After multivariate analysis, a neutrophil count <1000 cells/µl was a protective factor for disease activity (OR 0.021; 95% CI 0.001-0.428, p 0.012). On the other hand, a history of renal activity (OR 4.3; 95% CI 1.3-14.2, p=0.024) and neutrophils less than 1000 cells/µl (OR 4.05; 95% CI 1.15-14.19, p=0.029) were found to be independent risk factors for myelotoxicity.Table 1CharacteristicDisease activity(N=25)Drug-associated toxicity(N=28)pHistory of disease activity- Mucocutaneous- Hematologic- Renal80%100%44%86%96.4%78.6%0.71910.012Neutrophils (cells/μl)- Mild neutropenia (1000-1499)- Moderate neutropenia (500-999)- Severe neutropenia (<500)16% 4% 4%17.9% 7.1% 39.3%0.004Lymphopenia (<1000 cells/µl)68%70%1Platelets (K/µl)- MIld thrombocytopenia (50-99)- Severe thrombocytopenia (<50)16%28%10.7%32.1%0.859SLEDAI score6 (0-23)6 (1-14)0.370SLICC damage index0 (0-5)0 (0-6)0.791Treatment- Azathioprine- Mofetil mycophenolate- Hydroxychloroquine- Prednisoneo ≤7.5 mgo 7.6-30 mgo >30 mg24%0%4%56%16%28%53.6%14.3%35.7%46.2%28...
Background Splenectomy is a therapy for patients with treatment‐refractory autoimmune cytopenias. Antiphospholipid antibodies (aPL) can be identified in 25%–85% of these patients. In this study, we sought to identify whether the presence of aPL was associated with worse outcomes in autoimmune cytopenia's patients who had undergone splenectomy. Methods We conducted a retrospective cohort study of patients who underwent splenectomy from 2000 to 2018. We describe clinical characteristics and outcomes in patients with autoimmune cytopenia's diagnosis with positive determinations of aPL. Additionally, we performed a case–control sub‐analysis 1:1 of the cases with autoimmune cytopenia's matched control patients with negative aPL determination. Results A splenectomy was performed in 707 patients, of which we included 34 for the analysis. The median age at the time of splenectomy was 37 years (range 19–61), 53% corresponded to immune thrombocytopenia (ITP) and 47% to autoimmune hemolytic anemia (AIHA). Compared with controls (n = 34), patients had more treatment lines in addition to steroids (p = .02). There were no differences in complete response rate, 65% in cases and 80% in controls (p = .17). However, there was numerically a higher incidence of early infections (21% of cases vs. 3% controls, p = .05). During the entire follow‐up, 15% of aPL patients compared with 9% of control patients had a thrombotic event (p = .70). Discussion Splenectomy for treatment‐refractory autoimmune cytopenia's patients with persistent aPL is an effective treatment despite some safety concerns related to early infections. These results suggest that the presence of aPL should not impact the decision to undergo splenectomy.
Background:Whereas SLE is uncommon in men, the disease is usually more severe and requires more aggressive immunosuppression in male patients. There are multiple studies regarding sexual aspects in women with SLE, but information about sexual function in male patients is quite scant.Objectives:To determine the relationship between SLE and sexual function alterations in men, through the application of validated questionnaires.Methods:We performed a longitudinal study in a third-level referral center in Mexico City. We included men aged ≥16 years who fulfilled ACR criteria for SLE and who were sexually active. All subjects answered the International Index of Erectile Function-15 (IIEF-15), the SF-36 and the HAQ in two visits. Other clinical, serological and demographic variables were measured. Oxidized LDL was quantified by ELISA.Results:We included 108 male SLE patients. Mean age was 37.2±1.1 years and most patients (87%) were taking immunosuppressive therapy. Comorbidities were present in 58% of subjects, with dyslipidemia and hypertension being the most prevalent (34% and 28%, respectively).The prevalence of sexual dysfunction (SD) was 53%. In the basal visit, the only significant differences between the patients with SD and those without SD were a lower education degree (p=0.007) and persistent lymphopenia (p=0.01). There was a positive correlation between global IIEF-15 score and SF-36 score (r=0.46, p=0.001). The physical function domain had the highest correlation (r=0.50, p=0.001). Likewise, there was a weak negative correlation between IIEF-15 and HAQ score (r=-0.25, p=0.012). Also, the IIEF-15 had a weak correlation with the absolute lymphocyte count (r=0.27, p=0.005) and oxidized LDL (r=0.31, p=0.04).In the follow-up visit the only significant differences between the patients with SD when compared with subjects without SD was a low absolute lymphocyte count (1031±89 vs 1458±119, p=0.005); the correlations mentioned in the baseline visit remained significant. Regarding erectile function, 44% of the subjects had some degree of dysfunction. The rest of the variables are shown in Table 1.Abstract AB1250 Table 1Demographic, clinical and laboratory featuresVariableMean ± SEMDemographicAge (years)37.2 ± 1.1Body mass index (kg/m2)26.5 ± 0.4Less than 10 years of schooling (n,%)21/108 (19.4)Time since SLE diagnosis (years)9.1 ± 0.6Clinical FeaturesTotal score IIEF-1558.7 ± 1.3Erectile function23.9 ± 0.6Intercourse satisfaction10.9 ± 0.3Orgasmic function8.1 ± 0.2Sexual desire7.5 ± 0.1Overall satisfaction8.1 ± 0.1Total score SF-3669.2 ± 1.3Secondary antiphospholipid syndrome (n,%)16/108 (14.8)SLEDAI score (points)4.2 ± 0.4Others comorbidities (n,%)63/108 (58.3)Laboratory featuresHemoglobin (mg/dl)15.3 ± 0.2Leukocytes (mm3) (x103)5.9 ± 0.2Absolute lymphocyte count (mm3)1362.6 ± 76.0Serum creatinine (mg/dl)1.4 ± 0.1C3 levels104.1 ± 3.2C4 levels19.7 ± 1.2Anti-dsDNA antibodies208.2 ± 70.4Use of immunosuppressive treatment (n,%)95/108 (87.9)Prednisone (n,%)58/108 (53.7)Azathioprine (n,%)31/108 (28.7)Antimalarial (n,%)73/1...
Background:Thrombocytopenia and autoimmune hemolytic anemia (AIHA) are common hematologic manifestations in primary antiphospholipid syndrome (APS). Although splenectomy is considered a second-line treatment in both primary immune thrombocytopenia (ITP) and idiopathic AIHA, its role in APS patients with either one of these manifestations has not been adequately defined, mainly because of the theoretically increased risk of thrombosis for patients with APS who undergo surgery.Objectives:To determine the safety and efficacy of splenectomy for steroid-refractory thrombocytopenia or autoimmune hemolytic anemia in patients with primary APS, when compared to patients with ITP or idiopathic AIHA.Methods:We performed a retrospective, single-center, case-control study. We included patients with primary APS and either thrombocytopenia, or autoimmune hemolytic anemia/Evans syndrome who underwent splenectomy between 2000 and 2018. The control group was made up by patients with primary immune cytopenias (ITP or AIHA) who also underwent splenectomy during that period. Cases and controls were adjusted by age, the hematologic manifestation and date of splenectomy. We recorded demographic, clinical and serologic characteristics at the time of surgery and during follow-up.Results:We included 34 patients in each group. Thrombocytopenia was the indication for splenectomy in 53% of patients, with AIHA or Evans syndrome comprising the remaining 47%. Most patients were female (78%) and median age was 37 years. Among APS patients, 41% had triple antibody positivity.There were no differences regarding comorbidities between groups. Patients with APS received more immunosuppressive treatment lines before splenectomy compared to controls (p=0.02), and there was a trend for more high-dose steroid cycles in the APS group (p=0.07). Median time to splenectomy was 54 months in APS patients and 18 months in controls, but without statistical significance.Regarding splenectomy, most were laparoscopic (88%) and surgical complications were similar between groups (18%). However, patients with APS had a higher incidence of global non-surgical complications in the first month (50 vs 23%, p=0.04), most of them being infections (21 vs 3%, p=0.05). There was no difference in the incidence of postsurgical thrombosis, venous or arterial, between groups.Most patients achieved a global response after one month (85% in APS group, 91% in controls, p=0.7). Complete response was observed in 65% and 79% of cases and controls, respectively (p=0.27). Median follow-up time was 52 months for APS patients and 41 months for controls. There were no differences regarding relapse which required any treatment adjustment between cases and controls (44% and 38%, respectively, p=0.8, Fig 1). However, 47% of APS patients received a prolonged maintenance immunosuppressive treatment, compared with 6% of controls (p<0.01). The incidence of infections and thrombosis during follow-up was similar between groups (p=0.15 and p=0.7, respectively; Fig 2).Conclusion:Splenectomy is associate...
Objectives Our study aims to describe the association between SLE and sexual function, analysing demographic variables, comorbidities and other disease-related factors. As an exploratory objective, the impact of asking about sexual function during outpatient consultation was evaluated. Methods From 2018 to 2019, we invited sexually active men diagnosed with SLE to complete questionnaires that evaluated their sexual function and quality of life. Additionally, patients were asked if they believed they had sexual dysfunction, whether they would be interested in receiving specialized sexual care, and if they considered SLE to be detrimental to their sexual function. Epidemiological and disease-related data were retrieved from the patients’ clinical records. Results We included 124 men with SLE. Twenty-two (18%) patients answered positively when asked if they believed they had sexual dysfunction. These patients had lower overall erectile function scores and lower physical function scores than those who did not consider they had sexual dysfunction. In the multivariable analysis, factors that were associated with better sexual function were high physical function (B = 0.126, p = .031), lower BMI (B = 0.53, p = .010) and the patient’s perception of normal sexual function (B = 13.0, p < .001). Comorbidities associated with worse sexual function were type 2 diabetes (B = −8.1, p = .017) and a history of thrombosis (B = −5.12, p = .019). Conclusion Sexual function of male patients with SLE is impaired, independently of disease activity, chronic disease damage or pharmacological treatment. A simple question about perception of sexual function in the outpatient clinic can be used to help determine which patients could benefit from a multidisciplinary intervention to improve sexual health.
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