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https://www.readbyqxmd.com/read/28818881/selexipag-for-the-treatment-of-connective-tissue-disease-associated-pulmonary-arterial-hypertension
#1
Sean Gaine, Kelly Chin, Gerry Coghlan, Richard Channick, Lilla Di Scala, Nazzareno Galiè, Hossein-Ardeschir Ghofrani, Irene M Lang, Vallerie McLaughlin, Ralph Preiss, Lewis J Rubin, Gérald Simonneau, Olivier Sitbon, Victor F Tapson, Marius M Hoeper
Patients with connective tissue disease-associated pulmonary arterial hypertension (PAH-CTD) have a poor prognosis compared with other aetiologies. The underlying CTD can influence treatment response and outcomes. We characterised the GRIPHON study PAH-CTD subgroup and evaluated response to selexipag.Of 334 patients with PAH-CTD, PAH was associated with systemic sclerosis (PAH-SSc) in 170, systemic lupus erythematosus (PAH-SLE) in 82 and mixed CTD/CTD-other in 82. For the primary composite endpoint of morbidity/mortality, hazard ratios (HR) and 95% CI were calculated using Cox proportional hazard models...
August 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28811201/the-unmet-need-in-rheumatology-reports-from-the-targeted-therapies-meeting-2017
#2
Kevin L Winthrop, Vibeke Strand, Désirée van der Heijde, Philip Mease, Mary Crow, Michael Weinblatt, Joan Bathon, Gerd R Burmester, Maxime Dougados, Johnathan Kay, Xavier Mariette, Ronald Van Vollenhoven, Joachim Sieper, Fritz Melchers, Ferdinand C Breedfeld, Joachim Kalden, Josef S Smolen, Daniel E Furst
The 19th annual international Targeted Therapies meeting brought together over 100 leading basic scientists and clinical researchers from around the world in the field of immunology, molecular biology and rheumatology and other specialties. During the meeting, breakout sessions were held consisting of 5 disease-specific groups with 20-40 experts assigned to each group based on clinical or scientific expertise. Specific groups included: rheumatoid arthritis, psoriatic arthritis, axial spondyloarthritis, systemic lupus erythematous, connective tissue diseases (e...
August 12, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28804819/performance-of-activated-partial-thromboplastin-time-aptt-determining-reagent-sensitivity-to-factor-deficiencies-heparin-and-lupus-anticoagulants
#3
Geoffrey Kershaw
The activated partial thromboplastin time (APTT) is a useful global assay for the assessment of the contact factor pathway of hemostasis and its inhibitors. The test is usually performed on fully automated analyzers using commercially prepared reagents. The three main clinical areas of interest are detection of factor deficiencies, detection of lupus anticoagulants and in the monitoring of therapy with unfractionated heparin. Methods are described here for assessing APTT reagents for their sensitivity to clotting time prolongation in each of these areas of interest...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28803702/patient-and-physician-reported-satisfaction-with-systemic-lupus-erythematosus-treatment-in-us-clinical-practice
#4
Katie Pascoe, Steve Lobosco, David Bell, Ben Hoskin, David J Chang, Bonnie Pobiner, Sulabha Ramachandran
PURPOSE: This two-part study comprised two descriptive, cross-sectional surveys to evaluate treatment satisfaction among patients with systemic lupus erythematosus (SLE) and their physicians from US clinical practices. The Lupus Plus Project (LPP; part one) involved belimumab-containing regimens; the Disease Specific Program (DSP; part two) included all treatments and was designed to build on the body of evidence from part one. METHODS: The LPP recruited patients receiving belimumab, and comprised 2 paper questionnaires: a patient self-completion questionnaire (PSC) and a patient record form (PRF) completed by the physician...
August 10, 2017: Clinical Therapeutics
https://www.readbyqxmd.com/read/28800401/lupus-nephritis-and-b-cell-targeting-therapy
#5
Matthias Cassia, Federico Alberici, Maurizio Gallieni, David Jayne
Lupus Nephritis (LN) is a severe manifestation of Systemic Lupus Erythematosus (SLE) with a significant prognostic impact. Over a prolonged course, an exhaustion of treatment alternatives may occur and further therapeutic options are needed. B cells play a pivotal role in disease pathogenesis and represent an attractive therapeutic target. Areas covered: This review provides an update regarding targeting B cells in LN. The rational for this approach, as well as currently available and future targets are discussed...
August 18, 2017: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/28795307/sex-symptom-severity-and-quality-of-life-in-rheumatology
#6
REVIEW
Marco Krasselt, Christoph Baerwald
Inflammatory rheumatic diseases such as rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) show a striking female predominance ranging from 3:1 in RA up to 9:1 in SLE. The background for those gender bias is not fully understood yet, but seems to be the result of a complex interaction between sex hormones, (epi-)genetics, and possibly even the composition of gut microbiota. Moreover, time of disease onset, the clinical phenotype including co-morbidities as well as the course of the diseases during life differ between genders...
August 9, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/28794515/the-complement-system-as-a-potential-therapeutic-target-in-rheumatic-disease
#7
REVIEW
Leendert A Trouw, Matthew C Pickering, Anna M Blom
Complement activation is associated with common rheumatic diseases such as systemic lupus erythematosus (SLE), rheumatoid arthritis (RA) and systemic vasculitis. Evidence linking complement activation to these diseases includes the presence of complement deposition in affected tissues, decreased levels of complement proteins and high levels of complement activation fragments in the blood and/or synovial fluid of patients with these diseases, as well as data from experimental models. Eculizumab, a monoclonal antibody that inhibits the complement component C5, is now approved for the treatment of rare conditions involving complement hyperactivation, and the success of this therapy has renewed interest in understanding the utility of complement inhibition in rheumatological practice, particularly for SLE...
August 10, 2017: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/28794288/kidney-disease-in-oman-a-view-of-the-current-and-future-landscapes
#8
Intisar Hamed Al Alawi, Issa Al Salmi, Adhra Al Mawali, John A Sayer
Oman is located in the southeast of Arabian Peninsula with a relatively young population of about 3 831 553 people. The Ministry of Health, which is the healthcare provider, is facing a challenge with the increased levels of noncommunicable diseases including chronic kidney disease. A growing number of patients progress to end-stage kidney disease (ESKD), demanding renal replacement therapy. In 2014, there were 1339 of ESKD patients receiving dialysis and almost 1400 patients received kidney transplants. The estimated annual incidence of ESKD is 120 patients per million population...
July 2017: Iranian Journal of Kidney Diseases
https://www.readbyqxmd.com/read/28789968/b-cell-phenotypes-signaling-and-their-roles-in-secretion-of-antibodies-in-systemic-lupus-erythematosus
#9
Yoshiya Tanaka, Satoshi Kubo, Shigeru Iwata, Maiko Yoshikawa, Shingo Nakayamada
B cells play a pivotal role in the initiation and perpetuation of SLE. Because SLE is molecularly and clinically heterogeneous, efficacious targeted therapy to clinical remission has not yet been established in SLE. We have found i) statistical clustering between Tfh cells and class-switched memory B cells and the upregulated transition from CXCR5(+) IgM memory B cells to CXCR3(+) class-switched memory B cells in SLE by 8-color flow cytometry, ii) the involvement of Syk, Btk and JAK in the activation and differentiation of B cells in SLE, iii) SLE patients was divided to 3 groups based on immunophenotypic analysis and statistical analysis and patients in the Tfh/class-switched B cell-dominant group were most refractory to conventional therapies although 3 groups had similar clinical features...
August 5, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28782595/dhea-supplementation-to-dexamethasone-treated-rabbits-alleviates-oxidative-stress-in-kidney-cortex-and-attenuates-albuminuria
#10
Anna Kiersztan, Nina Trojan, Aleksandra Tempes, Paweł Nalepa, Joanna Sitek, Katarzyna Winiarska, Michał Usarek
Our recent study has shown that dehydroepiandrosterone (DHEA) administered to rabbits partially ameliorated several dexamethasone (dexP) effects on hepatic and renal gluconeogenesis, insulin resistance and plasma lipid disorders. In the current investigation, we present the data on DHEA protective action against dexP-induced oxidative stress and albuminuria in rabbits. Four groups of adult male rabbits were used in the in vivo experiment: (1) control, (2) dexP-treated, (3) DHEA-treated and (4) both dexP- and DHEA-treated...
August 4, 2017: Journal of Steroid Biochemistry and Molecular Biology
https://www.readbyqxmd.com/read/28782090/purified-umbilical-cord-derived-mesenchymal-stem-cell-treatment-in-a-case-of-systemic-lupus-erythematosus
#11
Christopher D Phillips, Pornpatcharin Wongsaisri, Thein Htut, Terry Grossman
INTRODUCTION: Systemic lupus erythematosus (SLE) is a multiple organ system autoimmune disorder for which there is no known cure. METHODS: We report a case of a young adult lady with SLE and Sjogren's with diagnostic and clinical resolution following purified umbilical cord derived mesenchymal stem cell (MSC) and globulin component protein macrophage activating factor (GcMAF) therapy in a combined multidisciplinary integrative medicine protocol. RESULTS: Our patient had complete reversal of all clinical and laboratory markers...
December 2017: Clinical and Translational Medicine
https://www.readbyqxmd.com/read/28780657/combination-therapy-of-rituximab-and-mycophenolate-mofetil-in-childhood-lupus-nephritis
#12
Julien Hogan, Astrid Godron, Véronique Baudouin, Theresa Kwon, Jérôme Harambat, Georges Deschênes, Olivier Niel
BACKGROUND: In clinical trials, the addition of rituximab (RTX) to the combination therapeutic regimen of mycophenolate mofetil (MMF) and corticosteroids failed to improve outcome in lupus nephritis (LN). However, recent data suggest that RTX may have steroid-sparing beneficial effects with an efficacy similar to that of conventional regimens. We report our experience with RTX in the treatment of children with LN. METHODS: Patients treated with RTX for first occurrence of LN class III to V were enrolled in the study...
August 5, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28780512/repeated-administration-of-dapirolizumab-pegol-in-a-randomised-phase-i-study-is-well-tolerated-and-accompanied-by-improvements-in-several-composite-measures-of-systemic-lupus-erythematosus-disease-activity-and-changes-in-whole-blood-transcriptomic-profiles
#13
Chris Chamberlain, Peter J Colman, Ann M Ranger, Linda C Burkly, Geoffrey I Johnston, Christian Otoul, Christian Stach, Miren Zamacona, Thomas Dörner, Murray Urowitz, Falk Hiepe
OBJECTIVES: Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease associated with diffuse immune cell dysfunction. CD40-CD40 ligand (CD40L) interaction activates B cells, antigen-presenting cells and platelets. CD40L blockade might provide an innovative treatment for systemic autoimmune disorders. We investigated the safety and clinical activity of dapirolizumab pegol, a polyethylene glycol conjugated anti-CD40L Fab' fragment, in patients with SLE. METHODS: This 32-week randomised, double-blind, multicentre study (NCT01764594) evaluated repeated intravenous administration of dapirolizumab pegol in patients with SLE who were positive for/had history of antidouble stranded DNA/antinuclear antibodies and were on stable doses of immunomodulatory therapies (if applicable)...
August 5, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28774170/an-autopsy-case-of-epstein-barr-virus%C3%A2-associated-diffuse-large-b-cell-lymphoma-of-the-central-nervous-system-in-an-immunocompromised-host
#14
Sun-Young Park, Seong Ik Kim, Hannah Kim, Yoojin Lee, Sung-Hye Park
Lymphomas arising in the central nervous system (CNS) of immunocompromised hosts represent non-Hodgkin's lymphomas exclusively associated with Epstein-Barr virus (EBV). Here we report an autopsy case of EBV-associated CNS diffuse large B-cell lymphoma (DLBCL) in a host suffering from systemic lupus erythematosus who underwent immunosuppressive therapy. After autopsy, EBV-associated CNS DLBCL as well as pulmonary mixed aspergillosis and Pneumocystis jirovecii pneumonia were added to the clinical impressions of complicated pneumonia and cerebral hemorrhage in this immunocompromised patient...
August 4, 2017: Journal of Pathology and Translational Medicine
https://www.readbyqxmd.com/read/28771665/tnf-antagonist-induced-lupus-a-critically-appraised-topic
#15
REVIEW
S E Momen, B Kirkham, J N Barker, C H Smith
TNF antagonist induced lupus is diagnosed upon a temporal relationship of TNF antagonist therapy and the development of four of the 11 American college of rheumatology (ACR) diagnostic criteria for lupus. We critically appraise the literature on TNF antagonist induced lupus, focusing on the diagnostic criteria, dermatological manifestations, management and outcome. Eighty cases of TNF antagonist induced lupus that met the ACR criteria were included and analysed. Infliximab was the most commonly reported agent (58...
August 3, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28770706/efficacy-of-belimumab-for-the-long-term-maintenance-therapy-of-thrombocytopenia-in-systemic-lupus-erythematosus
#16
Ginevra De Marchi, Luca Quartuccio, Milena Bond, Salvatore De Vita
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July 26, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28769820/inhibition-of-toll-like-receptor-signaling-as-a-promising-therapy-for-inflammatory-diseases-a-journey-from-molecular-to-nano-therapeutics
#17
REVIEW
Wei Gao, Ye Xiong, Qiang Li, Hong Yang
The recognition of invading pathogens and endogenous molecules from damaged tissues by toll-like receptors (TLRs) triggers protective self-defense mechanisms. However, excessive TLR activation disrupts the immune homeostasis by sustained pro-inflammatory cytokines and chemokines production and consequently contributes to the development of many inflammatory and autoimmune diseases, such as systemic lupus erythematosus (SLE), infection-associated sepsis, atherosclerosis, and asthma. Therefore, inhibitors/antagonists targeting TLR signals may be beneficial to treat these disorders...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28765121/novel-therapies-for-immune-mediated-inflammatory-diseases-what-can-we-learn-from-their-use-in-rheumatoid-arthritis-spondyloarthritis-systemic-lupus-erythematosus-psoriasis-crohn-s-disease-and-ulcerative-colitis
#18
REVIEW
Kenneth F Baker, John D Isaacs
The past three decades have witnessed remarkable advances in our ability to target specific elements of the immune and inflammatory response, fuelled by advances in both biotechnology and disease knowledge. As well as providing superior treatments for immune-mediated inflammatory diseases (IMIDs), such therapies also offer unrivalled opportunities to study the underlying immunopathological basis of these conditions.In this review, we explore recent approaches to the treatment of IMIDs and the insights to pathobiology that they provide...
August 1, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28764615/cerebral-tuberculosis-in-a-patient-with-systemic-lupus-erythematosus-following-cyclophosphamide-treatment-a-case-report
#19
S Cooray, H Zhang, R Breen, G Carr-White, R Howard, M Cuadrado, D D'Cruz, G Sanna
Central nervous system (CNS) tuberculosis (TB) is a rare but catastrophic event in patients with systemic lupus erythematosus (SLE). Here we report a case of cerebral TB in a patient with lupus myocarditis and nephritis, following cyclophosphamide immunosuppression. To our knowledge this is the first reported case of cerebral TB in SLE in a non-endemic country. A 31-year-old female with SLE and a history of regular travel to Kenya presented to our centre with clinical features of acute heart failure. She was diagnosed with severe lupus myocarditis, and a renal biopsy also confirmed lupus nephritis...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28761807/minocycline-induced-lupus-with-yellow-colored-chylous-exudative-pleural-effusion
#20
Daniel Starobin, Vladimir Guller, Alexander Gurevich, Sari Tal
Ninety years old male was admitted to hospital due to breathlessness. The prominent findings were extensive blue-grey skin pigmentation and large left chylothorax. Drug induced lupus was diagnosed due to either minocycline chronic treatment or no alternative illness to explain his sub-acute disease. Minocycline therapy was stopped with gradual improvement of pleural effusion and skin discoloration. This case is the first presentation of minocycline induced lupus with chylothorax.
2017: Respiratory Medicine Case Reports
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