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https://www.readbyqxmd.com/read/30298658/lupus-nephritis-an-update-on-treatments-and-pathogenesis
#1
REVIEW
Desmond Yh Yap, Susan Yung, Tak Mao Chan
Immunosuppressive therapies for lupus nephritis (LN) have improved significantly over the past few decades, resulting in growing number of choices for treatment individualization and improved renal and patient outcomes. Corticosteroids combined with mycophenolate or cyclophosphamide induces a satisfactory response in a high proportion of Asian and Caucasian patients, but the rate of improvement varies considerably between patients. Relatively low disease flare rate was observed in Chinese patients receiving low-dose prednisolone and mycophenolate maintenance...
October 2018: Nephrology
https://www.readbyqxmd.com/read/30294950/blood-concentrations-of-complement-split-product-ic3b-and-serum-c3-associate-with-sle-disease-activity
#2
Alfred H J Kim, Vibeke Strand, Deepali P Sen, Qiang Fu, Nancy L Mathis, Martin J Schmidt, Robin R Bruchas, Nick R Staten, Paul K Olson, Chad M Stiening, John P Atkinson
OBJECTIVE: To examine correlations between blood levels of complement split product iC3b and serum component C3 with clinically meaningful changes in disease activity in patients with systemic lupus erythematosus (SLE). METHODS: 159 consecutive subjects with American College of Rheumatology or Systemic Lupus International Collaborating Clinics classified SLE were enrolled into CASTLE (Complement Activation Signatures in Systemic Lupus Erythematosus), a prospective observational study...
October 8, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/30291433/-pathogenesis-of-systemic-lupus-erythematosus
#3
REVIEW
S Finzel, S Schaffer, M Rizzi, R E Voll
The causes of diseases and disorders of the immune system, which lead to the development of systemic lupus erythematosus (SLE), are not yet completely understood; however, it is known that there are various mechanisms, which can lead to SLE. The development of the disease is based on an underlying genetic disposition but is first triggered by exposure to environmental factors, such as sunburn, viral infections or vitamin D deficiency. Disease flares can also be triggered by environmental factors. Many disease manifestations are caused by pathogenic autoantibodies; hence, B‑cells and plasma cells play a critical role in the pathogenesis of SLE...
October 5, 2018: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/30290717/therapeutic-potential-of-allogeneic-mesenchymal-stromal-cells-transplantation-for-lupus-nephritis
#4
J Barbado, S Tabera, A Sánchez, J García-Sancho
Animal and human studies have suggested the potential of mesenchymal stromal cells (MSCs) to treat systemic lupus erythematosus (SLE). Here, we present the results of compassionate MSC treatments for three SLE patients to provide the proof of concept for a randomized and controlled clinical trial. Three patients of different ethnicities who suffer from chronic SLE, and who presented with class IV active proliferative nephritis confirmed by biopsy, were treated with allogeneic MSCs from healthy donors. Ninety million cells were infused intravenously into each patient during high and very high activity disease flare-ups and follow-up was continued for 9 months...
October 5, 2018: Lupus
https://www.readbyqxmd.com/read/30275264/longterm-data-on-sirolimus-treatment-in-patients-with-lupus-nephritis
#5
Desmond Y H Yap, Colin Tang, Gary C W Chan, Lorraine P Y Kwan, Maggie K M Ma, Maggie M Y Mok, Tak Mao Chan
OBJECTIVE: To expand the limited longterm data on sirolimus treatment in patients with lupus nephritis (LN). Our pilot short-term data suggested efficacy of sirolimus treatment in these patients. METHODS: We retrospectively reviewed 16 class III/IV/V patients with LN who have received prednisolone (PSL) and sirolimus either as initial or maintenance treatment. RESULTS: Sixteen patients received sirolimus treatment (9 because of intolerance to standard immunosuppressants and 7 because of a history of malignancy) for 45...
October 1, 2018: Journal of Rheumatology
https://www.readbyqxmd.com/read/30275257/hydroxychloroquine-levels-throughout-pregnancies-complicated-by-rheumatic-disease-implications-for-maternal-and-neonatal-outcomes
#6
Stephen J Balevic, Michael Cohen-Wolkowiez, Amanda M Eudy, Thomas P Green, Laura E Schanberg, Megan E B Clowse
OBJECTIVE: Pregnancies in women with active rheumatic disease often result in poor neonatal outcomes. Hydroxychloroquine (HCQ) reduces disease activity and flares; however, pregnancy causes significant physiologic changes that may alter HCQ levels and lead to therapeutic failure. Therefore, our objective was to evaluate HCQ concentrations during pregnancy and relate levels to outcomes. METHODS: We performed an observational study of pregnant patients with rheumatic disease who were taking HCQ from a single center during 2013-2016...
October 1, 2018: Journal of Rheumatology
https://www.readbyqxmd.com/read/30270156/catatonia-associated-with-systemic-lupus-erythematosus-sle-a-report-of-2-cases-and-a-review-of-the-literature
#7
REVIEW
Annabel Boeke, Bianca Pullen, Lucas Coppes, Michel Medina, Joseph J Cooper
BACKGROUND: Systemic lupus erythematosus (SLE) is known to cause neuropsychiatric symptoms (NPSLE). While not formally recognized as a syndrome associated with NPSLE, catatonia has frequently been reported. OBJECTIVE: It is important for clinicians to recognize and treat catatonia as a potential manifestation of NPSLE. We present 2 cases of SLE with catatonia and review the cases reported in the literature. METHODS: We performed a PubMed search for reported cases of catatonia in SLE...
June 27, 2018: Psychosomatics
https://www.readbyqxmd.com/read/30261321/dna-as-a-self-antigen-nature-and-regulation
#8
REVIEW
Chetna Soni, Boris Reizis
High-affinity antibodies to double-stranded DNA are a hallmark of systemic lupus erythematosus (SLE) and are thought to contribute to disease flares and tissue inflammation such as nephritis. Notwithstanding their clinical importance, major questions remain about the development and regulation of these pathogenic anti-DNA responses. These include the mechanisms that prevent anti-DNA responses in healthy subjects, despite the constant generation of self-DNA and the abundance of DNA-reactive B cells; the nature and physical form of antigenic DNA in SLE; the regulation of DNA availability as an antigen; and potential therapeutic strategies targeting the pathogenic DNA in SLE...
September 24, 2018: Current Opinion in Immunology
https://www.readbyqxmd.com/read/30257684/successful-use-of-ofatumumab-in-two-cases-of-early-onset-juvenile-sle-with-thrombocytopenia-caused-by-a-mutation-in-protein-kinase-c-%C3%AE
#9
Linda Lei, Sabina Muhammad, Muthana Al-Obaidi, Neil Sebire, Iek Leng Cheng, Despina Eleftheriou, Paul Brogan
BACKGROUND: We previously described an endogamous Pakistani kindred in whom we identified a novel homozygous missense mutation in the PRKCD gene encoding for protein kinase C δ (PKCδ) as a cause of monogenic systemic lupus erythematosus (SLE). PKCδ has a role in the negative regulation of B cells. Given the nature of the disease, a logical targeted therapeutic approach in these patients is B cell depletion. Indeed, the 3 siblings all had a marked clinical response and resolution of symptoms with rituximab, although 2 of the siblings had severe reactions to rituximab thus precluding further treatment with this...
September 26, 2018: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/30255104/fetal-and-maternal-outcomes-of-planned-pregnancy-in-patients-with-systemic-lupus-erythematosus-a-retrospective-multicenter-study
#10
Dongying Chen, Minxi Lao, Jianyu Zhang, Yanfeng Zhan, Weinian Li, Xiaoyan Cai, Zhongping Zhan
Objective: To investigate the fetal and maternal outcomes as well as predictors of APOs in women with SLE who conceived when the disease was stable, the so-called "planned pregnancy." Methods . A retrospective multicenter study of 243 patients with SLE who underwent a planned pregnancy was performed. APOs in fetus and mothers were recorded. Results: The average age at conception was 28.9 ± 3.9 years. Duration of SLE prior to pregnancy was 4.4 ± 4...
2018: Journal of Immunology Research
https://www.readbyqxmd.com/read/30247224/visceral-leishmaniasis-versus-systemic-lupus-erythematosus-flare-old-axioms-sometimes-stay-true-other-times-not
#11
Maria Inês Seixas, Tiago Marques, Cristina Ferreira, José Carlos Romeu, Carla Macieira
No abstract text is available yet for this article.
September 21, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/30237973/miliary-tuberculosis-mimicking-systemic-lupus-erythematosus-flare
#12
Fatehi Elzein, Ahmed Elzein, Nazik Mohammed, Ramiz Alswailem
A 26-year-old woman was diagnosed with and treated for systemic lupus erythematosus (SLE) in 2002. She was admitted 11 years later with nephrotic-range proteinuria and lupus nephritis and received two doses of rituximab after failing on steroids and mycophenolate mofetil. Four months later, she presented with fever and joint pain/swelling. Gram stains, joint aspirates, and blood culture all yielded negative results for bacteria. She was discharged after treatment for a possible flare of lupus, but two weeks later, she presented again with a cough and shortness of breath in addition to the flare symptoms...
2018: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/30215901/rheumatologic-tests-a-primer-for-family-physicians
#13
Yousaf Ali
Patients with a suspected connective tissue disorder should undergo serologic testing to confirm the diagnosis and, in some cases, to monitor disease activity and predict flares. Patients with suspected systemic lupus erythematosus should be tested for antinuclear antibodies. However, antinuclear antibodies are not specific and may be present in many other connective tissue disorders and nonrheumatologic diseases. Thus, patients with suspected systemic lupus erythematosus should undergo further testing to confirm the diagnosis...
August 1, 2018: American Family Physician
https://www.readbyqxmd.com/read/30215770/pharmacokinetics-and-pharmacogenomics-of-mycophenolic-acid-and-its-clinical-correlations-in-maintenance-immunosuppression-for-lupus-nephritis
#14
Desmond Y H Yap, Chun Hay Tam, Susan Yung, Sunny Wong, Colin S O Tang, Temy M Y Mok, Catherine K Y Yuen, Maggie K M Ma, Chak Sing Lau, Tak Mao Chan
Background: There is little data on mycophenolic acid (MPA) pharmacokinetics and pharmacogenomics and optimal MPA exposure in lupus nephritis (LN) patients during long-term maintenance. Methods: We measured blood MPA levels at 1, 2, 4, 8, 10 and 12-h post-dose (i.e. C1, C2, C4, C8, C10 and C12) in 88 stable LN patients receiving maintenance prednisolone and mycophenolate mofetil, repeated every 6 months. The relationship between MPA exposure and single nucleotide polymorphisms (SNPs) of adenosine triphosphate-binding cassette subfamily C member 2 (ABCC2; rs2273697, rs3740066, rs717620 and rs17222723), organic anion-transporting polypeptides (OATPs; rs7311358 and rs4149117) and uridine diphosphate glucuronosyltransferase (UGT; rs17863762, rs6714486, rs17868320 and rs72551330) was also investigated...
September 11, 2018: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/30214327/macrophage-activation-syndrome-early-diagnosis-is-key
#15
REVIEW
Butsabong Lerkvaleekul, Soamarat Vilaiyuk
Macrophage activation syndrome (MAS) is a life-threatening condition, and it is a subset of hemophagocytic lymphohistiocytosis (HLH). The clinical features include a persistent high-grade fever, hepatosplenomegaly, lymphadenopathy, hemorrhagic manifestations, and a sepsis-like condition. From the clinical features, it is usually difficult to differentiate between a true sepsis, disease flare-ups, or MAS. Although the laboratory abnormalities are similar to those of a disseminated intravascular coagulation, which shows pancytopenia, coagulopathy, hypofibrinogenemia, and an elevated d-dimer test, it can also be a late stage of MAS...
2018: Open Access Rheumatology: Research and Reviews
https://www.readbyqxmd.com/read/30184477/cytokine-targets-in-lupus-nephritis-current-and-future-prospects
#16
Christina Adamichou, Spyros Georgakis, George Bertsias
Despite advancements in the care of lupus nephritis, a considerable proportion of patients may respond poorly or flare while on conventional immunosuppressive agents. Studies in murine and human lupus have illustrated a pathogenic role for several cytokines by enhancing T- and B-cell activation, autoantibodies production and affecting the function of kidney resident cells, therefore supporting their potential therapeutic targeting. To this end, there is limited post-hoc randomized evidence to suggest beneficial effect of belimumab, administered on top of standard-of-care, during maintenance therapy in lupus nephritis...
September 2, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/30180771/belimumab-restores-treg-th17-balance-in-patients-with-refractory-systemic-lupus-erythematosus
#17
M Prete, P Leone, M A Frassanito, V Desantis, C Marasco, S Cicco, F Dammacco, A Vacca, V Racanelli
Belimumab, a specific inhibitor of the soluble B lymphocyte stimulator (BlyS), is the first biological drug approved by the United States Food and Drug Administration for the treatment of patients with active systemic lupus erythematosus (SLE) refractory to standard therapy. Given that an imbalance between regulatory T cells (Treg) and interleukin (IL)-17A-secreting T cells (Th17) has been reported in various autoimmune disorders, we assessed the frequency of both Treg and Th17 peripheral blood populations before and after belimumab administration in 20 patients with active SLE refractory to standard therapy...
September 4, 2018: Lupus
https://www.readbyqxmd.com/read/30179587/precision-medicine-in-lupus-nephritis-can-biomarkers-get-us-there
#18
REVIEW
Dawn J Caster, Michael L Merchant, Jon B Klein, David W Powell
Patients with systemic lupus erythematosus frequently develop lupus nephritis (LN), a condition that can lead to end-stage kidney disease. Multiple serum and urine biomarkers for LN have been proposed in recent years, yet none have become incorporated into clinical use. The majority of studies have been single center with significant variability in cohorts, assays, and sample storage, leading to inconclusive results. It has become clear that no single biomarker is likely to be sufficient to diagnose LN, identify flares, and define the response to therapy and prognosis...
August 10, 2018: Translational Research: the Journal of Laboratory and Clinical Medicine
https://www.readbyqxmd.com/read/30151719/all-cause-hospitalizations-in-systemic-lupus-erythematosus-from-a-single-medical-center-in-israel
#19
Ofer Levy, Andrey Markov, Yulia Drob, Ilia Maslakov, Moshe Tishler, Mirit Amit-Vazina
OBJECTIVE: Clinical features of systemic lupus erythematosus (SLE) have been described from different geographical regions in the world. The aim of this study was to obtain local and contemporary data on all-cause hospitalizations of SLE patients in an Israeli Medical Center. METHODS: This is a retrospective observational single-center study. Revision of medical records of hospitalized lupus patients during 5-year period (January 2012-December 2016) was performed...
October 2018: Rheumatology International
https://www.readbyqxmd.com/read/30146745/disease-severity-and-economic-burden-in-japanese-patients-with-systemic-lupus-erythematosus-a-retrospective-observational-study
#20
Yoshiya Tanaka, Akiko Mizukami, Akihiro Kobayashi, Chie Ito, Taizo Matsuki
AIM: To describe the healthcare resource utilization (HRU), direct medical costs and clinical characteristics for Japanese patients with mild, moderate or severe systemic lupus erythematosus (SLE). The primary objectives were to describe HRU and examine the direct medical costs for Japanese patients with mild, moderate, or severe SLE over the 3-year study period. Secondary objectives included recording patient demographics, clinical characteristics and frequency and cost of mild, moderate or severe flares...
August 2018: International Journal of Rheumatic Diseases
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