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Pure red cell aplasia in systemic lupus erythematosus, a nationwide retrospective cohort and review of the literature.
Lobbes, H, Mahévas, M, Alviset, S, Galicier, L, Costedoat-Chalumeau, N, Amoura, Z, Alric, L, Hot, A, Durupt, S, Michel, M, et al
Rheumatology (Oxford, England). 2021;(1):355-366
Abstract
OBJECTIVES To characterize the clinical and biological course, management and response to treatment in SLE-associated pure red cell aplasia (PRCA). METHODS This was a nationwide, multicentre, retrospective cohort study. From 2006 to 2018, we included adults with a diagnosis of PRCA supported by bone marrow examination and SLE or biologic manifestations of SLE after ruling out parvovirus B19 infection. RESULTS We enrolled 24 patients (20 women). SLE was diagnosed before PRCA for 14 patients (median delay 81 months). At PRCA diagnosis, mean age, haemoglobin level, and reticulocyte and differential erythroblast count were 39.2 (13.2) years, 62 ( 20) g/l, 9.1 (7.6) × 109/l and 2.8 ( 2.5)%, respectively. Eleven (45%) patients experienced multiple PRCA flares (median 6, range 2-11). CS therapy resulted in only three complete sustained responses, and 19 (79%) patients required immunosuppressive agents with highly variable regimens. After a median follow-up of 76 months (range 13-173), 17 (71%) patients showed complete response for PRCA, 5 (21%) partial response and 2 (8%) treatment failure. In total, 21 (87%) patients required red blood cell transfusion; 5 had a diagnosis of transfusion-related iron overload. Eighteen (75%) patients experienced severe infectious events requiring hospitalization. CONCLUSION SLE-associated PRCA is a severe condition. Repeated red blood cell transfusions and several lines of immunosuppressant therapy are mostly required, with high risk of severe infectious events and iron overload. Despite sustained response for PRCA and SLE obtained in most patients, the best therapeutic strategy remains to be determined.
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2.
Telitacicept: First Approval.
Dhillon, S
Drugs. 2021;(14):1671-1675
Abstract
Telitacicept (Tai'ai®) is fusion protein comprising a recombinant transmembrane activator and calcium modulator and cyclophilin ligand interactor (TACI) receptor fused to the fragment crystallizable (Fc) domain of human immunoglobulin G (IgG). Telitacicept is being developed by Yantai Rongchang Pharmaceutical through its subsidiary RemeGen for the treatment of B cell-mediated autoimmune diseases, such as systemic lupus erythematosus (SLE), rheumatoid arthritis (RA) and multiple sclerosis (MS). Telitacicept binds to and neutralizes the activity of two cell-signalling molecules, B-lymphocyte stimulator (BLyS) and a proliferation-inducing ligand (APRIL), thereby suppressing the development and survival of plasma cells and mature B cells. In March 2021, telitacicept received its first approval in China for the treatment of patients with active SLE. Clinical studies of telitacicept in several other indications, including IgA nephropathy, MS, myasthenia gravis, neuromyelitis optica spectrum disorders, RA and Sjögren's syndrome are underway in China. This article summarizes the milestones in the development of telitacicept leading to this first approval for SLE.
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Scleroderma-associated thrombotic microangiopathy in overlap syndrome of systemic sclerosis and systemic lupus erythematosus: A case report and literature review.
Xie, X, Wang, G, Cheng, H, Sun, L, Dong, H
Medicine. 2020;(41):e22582
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Abstract
RATIONALE Systemic sclerosis (SSc) is a serious multisystem connective tissue disease. When SSc is accompanied by systemic lupus erythematosus (SLE), called SSc-SLE overlap syndrome. SSc associated thrombotic microangiopathy (SSc-TMA) can lead to scleroderma renal crisis, it mainly manifests hypertension or even malignant hypertension, acute kidney injury, and higher mortality. The case of SSc-SLE overlap syndrome combined with SSc-TMA has rarely been reported. PATIENT CONCERNS We report the case of an elderly male with SSc-SLE overlap syndrome combined with scleroderma renal crisis and SSc-TMA. DIAGNOSES The patient has typical of SSc on the face and hands, combined with pulmonary artery hypertension, interstitial lung disease, heart failure and malignant hypertension, as well as SLE, lupus nephritis class V, and TMA, which were definitively diagnosed by clinical laboratory examination and renal histopathology. INTERVENTIONS The patient was treated with prednisone, cyclophosphamid, renin-angiotensin system inhibitors, diuretics, and acetylcysteine. OUTCOMES The patient died suddenly of heart failure on the 35th day after discharge. LESSONS The occurrence of TMA leads to the deterioration of the prognosis of SSC-SLE overlap syndrome. The diagnosis of SSC-TMA in SSc-SLE overlap syndrome depends on clinical laboratory examination and renal histopathology.
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Primary breast diffuse large B-cell lymphoma in a patient with systemic lupus erythematosus: A case report and review of the literature.
Shen, F, Li, G, Jiang, H, Zhao, S, Qi, F
Medicine. 2020;(33):e21736
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RATIONALE Pilot studies have reported that patients with systemic lupus erythematosus (SLE) appear more likely to develop into neoplasia, especially lymphatic hyperplasia diseases. To our knowledge, this is the first case report of the concomitant onset of SLE and primary breast diffuse large B-cell lymphoma (PB-DLBCL). PATIENT CONCERNS We reported an unusual case of the occurrence of primary breast diffuse large B-cell lymphoma in a 25-year-old female patient who had been diagnosed with SLE and treated with immunosuppressive drugs for about 4 years. She presented a 7-week history of a painless mass above the left breast and no history suggestive of any nipple discharge, fever, and weight loss. DIAGNOSIS Ultrasonography of the breast showed that there was 1 mass in the left breast. After breast mass surgical resection, histopathological examinations were performed and revealed that it was primary breast diffuse large B-cell lymphoma. INTERVENTIONS Treatment strategy with vincristine and dexamethasone was used to improve symptoms. However, the patient's renal function deteriorated and the blood potassium rose continuously and she and their family members refused the follow-up treatments. OUTCOMES The patient died 8 months after she was discharged from the hospital. LESSONS PB-DLBCL is a rare occurrence in SLE patients. Therefore, a careful examination is very important in SLE cohort, as activity of the disease and malignancy may mimic each other. Meanwhile, when symptoms cannot be explained or insensitive to treatment, the occurrence of malignant tumors must be highly considered.
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2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus.
Aringer, M, Costenbader, K, Daikh, D, Brinks, R, Mosca, M, Ramsey-Goldman, R, Smolen, JS, Wofsy, D, Boumpas, DT, Kamen, DL, et al
Annals of the rheumatic diseases. 2019;(9):1151-1159
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OBJECTIVE To develop new classification criteria for systemic lupus erythematosus (SLE) jointly supported by the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR). METHODS This international initiative had four phases. (1) Evaluation of antinuclear antibody (ANA) as an entry criterion through systematic review and meta-regression of the literature and criteria generation through an international Delphi exercise, an early patient cohort and a patient survey. (2) Criteria reduction by Delphi and nominal group technique exercises. (3) Criteria definition and weighting based on criterion performance and on results of a multi-criteria decision analysis. (4) Refinement of weights and threshold scores in a new derivation cohort of 1001 subjects and validation compared with previous criteria in a new validation cohort of 1270 subjects. RESULTS The 2019 EULAR/ACR classification criteria for SLE include positive ANA at least once as obligatory entry criterion; followed by additive weighted criteria grouped in seven clinical (constitutional, haematological, neuropsychiatric, mucocutaneous, serosal, musculoskeletal, renal) and three immunological (antiphospholipid antibodies, complement proteins, SLE-specific antibodies) domains, and weighted from 2 to 10. Patients accumulating ≥10 points are classified. In the validation cohort, the new criteria had a sensitivity of 96.1% and specificity of 93.4%, compared with 82.8% sensitivity and 93.4% specificity of the ACR 1997 and 96.7% sensitivity and 83.7% specificity of the Systemic Lupus International Collaborating Clinics 2012 criteria. CONCLUSION These new classification criteria were developed using rigorous methodology with multidisciplinary and international input, and have excellent sensitivity and specificity. Use of ANA entry criterion, hierarchically clustered and weighted criteria reflect current thinking about SLE and provide an improved foundation for SLE research.
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Take a look at the eyes in Systemic Lupus Erythematosus: A novel point of view.
Conigliaro, P, Cesareo, M, Chimenti, MS, Triggianese, P, Canofari, C, Barbato, C, Giannini, C, Salandri, AG, Nucci, C, Perricone, R
Autoimmunity reviews. 2019;(3):247-254
Abstract
Systemic Lupus Erythematosus (SLE) is a connective tissue disease that involves multiple organs. Ocular structures and visual pathways can be affected in SLE because of disease-related eye involvement or drug toxicity. All the part of the eye may be interested with an external, anterior involvement, responsible of the dry eye disease, or posterior (retina) and neuro-ophtalmic manifestations. Retinopathy in SLE is suggestive of high disease activity being a marker of poor visual outcome and prognosis for survival. The early diagnosis is thus the key to a better management and successful treatment. Antimalarial drugs are the cornerstone of SLE treatment and recently the American Academy of Ophthalmology updated the recommendations for hydroxychloroquine retinal toxicity screening which includes the standard automated visual fields and the spectral domain optical coherent tomography. More recently new imaging techniques have been investigated to assess retinal function and reveal subclinical eye involvement. In this review we focalize on the evidence of eye manifestations in SLE, the eye drug toxicity related to antimalarial agents and steroids, and the methods employed for the eye screening. Moreover, the future perspectives on new techniques, such as the optical coherence tomography angiography, are dissected giving new insights on evaluation of microvasculature of the retina and choroid in SLE.
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Managing cancer risk in patients with systemic lupus erythematous.
Ladouceur, A, Bernatsky, S, Ramsey-Goldman, R, Clarke, AE
Expert review of clinical immunology. 2018;(10):793-802
Abstract
INTRODUCTION Numerous studies have clearly demonstrated that there is an altered cancer risk profile in patients with systemic lupus erythematous (SLE) versus the general population. This includes a higher risk of certain cancers (e.g. hematologic, lung) and a decreased risk of others (e.g. breast cancer). Several determinants could be behind this altered risk; these include immunosuppressant drugs, viral exposures, genetic factors, and other variables. Area covered: We review what is known regarding specific risk profiles and risk factors for some key cancers in SLE, including hematologic malignancies and lung cancers. In light of this, we examine current guidelines and practices regarding cancer screening, and propose ways that patients and physicians might help manage cancer risk in SLE. Expert commentary: Despite significant progress over the past decade, not many risk factors have been precisely identified. A better understanding of the elements that drive malignancy risk in systemic autoimmune rheumatic diseases may permit the further development of guidelines (regarding. cancer screening) for SLE patients. ABBREVIATIONS AbTG: Anti-thyroglobulin antibodies; AbTPO: Anti-peroxydase antibodies; AML: Acute myeloid leukemia; APRIL A proliferating-inducing ligand; BAFF B-cell activating factor; CAPA Canadian Arthritis Patient Alliance; CI: Confidence interval; CIN: Cervical intraepithelial neoplasia; CT: Computed tomography; DLBCL Diffuse large B cell lymphoma; dsDNA: Double-stranded DNA; EBV: Epstein-Barr virus; EULAR European League Against Rheumatism; IBD: Inflammatory bowel disease; HBV: Hepatitis B virus; HCV: Hepatitis C virus; HIV: Human immunodeficiency virus; HR: Hazard ratio; HSIL High-grade cervical squamous intraepithelial lesions; HSV: Herpes simplex virus; HL: Hodgkin lymphoma; HPV: Human papillomavirus; MALT Mucosa-associated lymphoid tissue; MDS: Myelodysplastic syndrome; MESNA 2-mercaptoethane sodium sulfonate; NHL: Non-Hodgkin lymphoma; OR: Odds ratio; Pap: Papanicolaou; RA: Rheumatoid arthritis; SLE: Systemic Lupus erythematous; SLICC Systemic International Collaborating Clinics; SNPs: Single-nucleotide polymorphisms; SOGC Society of Obstetricians and Gynaecologists of Canada.
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Hydroxychloroquine retinopathy - implications of research advances for rheumatology care.
Jorge, A, Ung, C, Young, LH, Melles, RB, Choi, HK
Nature reviews. Rheumatology. 2018;(12):693-703
Abstract
Despite advances in therapy for rheumatic diseases, hydroxychloroquine remains almost universally recommended for the treatment of systemic lupus erythematosus (SLE), and is often used in the management of other rheumatic diseases such as rheumatoid arthritis (RA). However, the major dose-limiting toxicity of hydroxychloroquine is retinopathy that can lead to loss of vision. New highly sensitive screening methods can identify early stages of retinopathy, and studies that include these modalities have indicated a substantially higher prevalence of hydroxychloroquine retinopathy than was previously recognized, resulting in revisions to ophthalmology guidelines and the recommendation of a low dose of hydroxychloroquine for many patients. However, the efficacy of low-dose hydroxychloroquine for treating SLE and other rheumatic diseases is unknown. Further studies are required to establish the effectiveness and retinal safety of the latest hydroxychloroquine treatment recommendations.
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Bone mineral density and vitamin D status in systemic lupus erythematosus (SLE): A systematic review.
Salman-Monte, TC, Torrente-Segarra, V, Vega-Vidal, AL, Corzo, P, Castro-Dominguez, F, Ojeda, F, Carbonell-Abelló, J
Autoimmunity reviews. 2017;(11):1155-1159
Abstract
Despite the improvement in the quality of life of patients with SLE due to scientific and technological advances, SLE remains a disease that over the years may produce irreversible damage to patients. Osteoporosis and secondary bone fractures are two of the major causes of irreparable injury in patients with SLE. Vitamin D insufficiency may play a vital role both in reduced bone mineral density (BMD) and in the appearance of fractures, although its mechanisms of action are still unclear. We performed a systematic review of the literature in order to determine the prevalence and predictors of reduced vitamin D plasma levels, bone loss and the presence of fractures in SLE patients. Our review encompassed all English-language publications using Medline and EMBase electronic databases from their inception (1966 and 1980, respectively) to December 2016. We included all intervention studies and observational studies in which vitamin D plasma levels, BMD and bone loss were measured and applied to patients with SLE. Previous studies suggested an increase in bone loss and fracture in patients with SLE compared with general population and although there is a high prevalence of vitamin D insufficiency in the general population, previous studies had demonstrated lower vitamin D levels in patients with SLE compared to age-matched controls. The etiology of reduced bone mass and reduced vitamin D plasma levels in SLE is multifactorial and includes a variety of intrinsic factors related to the disease itself and treatment side effects. SLE patients are at risk for developing these two comorbidities (reduced vitamin D plasma levels and low BMD) and it is therefore essential to study, monitor, prevent and treat bone metabolism disorders in SLE patients.
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Management of cardiovascular risk in systemic lupus erythematosus: a systematic review.
Andrades, C, Fuego, C, Manrique-Arija, S, Fernández-Nebro, A
Lupus. 2017;(13):1407-1419
Abstract
Systemic lupus erythematosus is associated with accelerated atherosclerosis and increased risk of cardiovascular complications. The aim of this study was to review the effectiveness of interventions for primary and secondary prevention of cardiovascular events and mortality and to review the effectiveness of interventions for cardiovascular risk factor reduction in systemic lupus erythematosus patients. A systematic review was conducted. Electronic databases Medline and Embase (1961-2015) were searched. Nineteen articles met the inclusion criteria and were selected. Low-calorie and/or low glycaemic index calories may be a useful option for secondary prevention in obese patients with systemic lupus erythematosus, and exercise would be useful in improving the endothelial function measured by flow-mediated dilation in this group of patients. The use of lipid-lowering drugs may improve the lipid profile in patients with systemic lupus erythematosus and hyperlipidaemia, but the effect of this treatment on overall cardiovascular mortality remains unknown. Antiplatelets, anticoagulants, antimalarials and lipid-lowering drugs may be effective in the primary and secondary prevention of major cardiovascular events, such as acute myocardial infarction or stroke. Similarly, lipid-lowering drugs and antimalarial drugs appear to reduce the serum levels of total cholesterol, low-density lipoprotein, glucose, diastolic blood pressure and calcium deposition at the coronary arteries. They may also improve insulin resistance and the level of high-density lipoproteins. It appears that treatment with antihypertensive drugs reduces blood pressure in patients with systemic lupus erythematosus, but the available studies are of low quality.