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Lupus therapy

Vikas Pathak, Robert Aris, Brian C Jensen, Wei Huang, Hubert James Ford
BACKGROUND: Plasma pro-BNP (brain natriuretic peptide) levels are often elevated in response to right ventricular (RV) volume and pressure overload, parameters potentially affected by exercise. Plasma pro-BNP levels change in association with long-term changes in pulmonary hemodynamics, thereby serving as a potential biomarker in pulmonary arterial hypertension (PAH). The 6-min Walk Test (6MWT) and pro-BNP level are often checked in a single office visit. There is no universal standard for measuring Pro-BNP levels relative to the timing of the 6MWT...
March 21, 2018: Lung
Mei Liu, Qiang Guo, Chunmei Wu, Delphine Sterlin, Shyamal Goswami, Ying Zhang, Teng Li, Chunde Bao, Nan Shen, Qiong Fu, Xiaoming Zhang
A hallmark of systemic lupus erythematosus (SLE) is the breaking of B-cell tolerance with the generation of high-affinity autoantibodies; however, the antibody-independent features of the B-cell compartment in SLE are less understood. In this study, we performed an extensive examination of B-cell subsets and their proinflammatory properties in a Chinese cohort of new-onset SLE patients. We observed that SLE patients exhibited an increased frequency of transitional B cells compared with healthy donors and rheumatoid arthritis patients...
March 21, 2018: Cellular & Molecular Immunology
Sherry Masoud, Stephen P McAdoo, Rachna Bedi, Thomas D Cairns, Liz Lightstone
Objective: B cell depletion, most commonly with rituximab, is an evolving therapeutic approach in SLE. Infusion reactions after rituximab are common, and may prevent re-treatment in patients who previously demonstrated beneficial response. We have used ofatumumab, a fully humanized anti-CD20 mAb, as an alternative B cell-depleting agent in patients with SLE who are rituximab-intolerant due to severe infusion reactions. Methods: A single-centre retrospective case series of 16 patients were treated with ofatumumab for SLE between 2012 and 2015...
March 19, 2018: Rheumatology
Chen Ben David, Nicola L Bragazzi, Abdulla Watad, Kassem Sharif, Aaron Whitby, Howard Amital, Mohammad Adawi
RATIONALE: Hidradenitis suppurativa (HS) is a chronic inflammatory condition characterized by recurrent swollen, deep, and painful abscesses. Several autoimmune conditions have been shown to be associated with HS including inflammatory bowel disease and spondyloarthropathies. PATIENT CONCERNS: 40-year-old female with systemic lupus erythematous (SLE) presented with recurrent abscesses and nodules on her extremities. DIAGNOSIS: Early considerations related the described dermatologic findings to the dermatologic manifestations of SLE, however findings from lesion biopsy were suggestive of HS...
March 2018: Medicine (Baltimore)
Cynthia Louis, Chris Burns, Ian Wicks
The pathogenesis of autoimmune diseases, such as rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) is driven by genetic predisposition and environmental triggers that lead to dysregulated immune responses. These include the generation of pathogenic autoantibodies and aberrant production of inflammatory cytokines. Current therapies for RA and other autoimmune diseases reduce inflammation by targeting inflammatory mediators, most of which are innate response cytokines, resulting in generalized immunosuppression...
2018: Frontiers in Immunology
Theresa L Wampler Muskardin, Timothy B Niewold
The type I interferon pathway has been implicated in the pathogenesis of a number of rheumatic diseases, including systemic lupus erythematosus, Sjögren syndrome, myositis, systemic sclerosis, and rheumatoid arthritis. In normal immune responses, type I interferons have a critical role in the defence against viruses, yet in many rheumatic diseases, large subgroups of patients demonstrate persistent activation of the type I interferon pathway. Genetic variations in type I interferon-related genes are risk factors for some rheumatic diseases, and can explain some of the heterogeneity in type I interferon responses seen between patients within a given disease...
March 21, 2018: Nature Reviews. Rheumatology
Jing Du, Ying Wang, Yan-Chun Li, Tong-Tong Wang, Yong-Lie Zhou, Zhen-Hua Ying
Systemic lupus erythematosus (SLE) is an autoimmune disease that affects many organs, but multisystem dysfunction is rare. Here, we report a case of a 29-year-old woman who was initially diagnosed with SLE complications including lupus nephritis, lupus encephalopathy, renal hypertension, thrombocytopenia, anaemia and hyperkalaemia. She recovered following treatment with high dose methylprednisolone, intravenous immunoglobulin (IVIG) and continuous renal replacement therapy (CRRT). However, a few days after hospital discharge, she developed gastrointestinal bleeding...
January 1, 2018: Journal of International Medical Research
Susan Malkiel, Ashley N Barlev, Yemil Atisha-Fregoso, Jolien Suurmond, Betty Diamond
Plasma cells (PCs) are responsible for the production of protective antibodies against infectious agents but they also produce pathogenic antibodies in autoimmune diseases, such as systemic lupus erythematosus (SLE). Traditionally, high affinity IgG autoantibodies are thought to arise through germinal center (GC) responses. However, class switching and somatic hypermutation can occur in extrafollicular (EF) locations, and this pathway has also been implicated in SLE. The pathway from which PCs originate may determine several characteristics, such as PC lifespan and sensitivity to therapeutics...
2018: Frontiers in Immunology
Antonis Fanouriakis, Christina Adamichou, Sofia Koutsoviti, Stylianos Panopoulos, Chrysanthi Staveri, Anastasia Klagou, Christina Tsalapaki, Lamprini Pantazi, Styliani Konsta, Clio P Mavragani, Despoina Dimopoulou, Styliani Ntali, Georgios Katsikas, Kyriaki A Boki, Dimitrios Vassilopoulos, Pinelopi Konstantopoulou, Stamatis-Nick Liossis, Antonia Elezoglou, Maria Tektonidou, Prodromos Sidiropoulos, Abdulsamet Erden, Petros P Sfikakis, George Bertsias, Dimitrios T Boumpas
BACKGROUND: Low disease activity is a validated target of current systemic lupus erythematosus (SLE) therapy. The aim of this study was to assess the ability of belimumab to achieve low disease activity states in real-life settings. METHODS: Multicentre prospective observational study of consecutive SLE patients receiving belimumab for at least 3 months, due to active disease refractory to at least one conventional immunosuppressant. Disease activity, including the recently defined lupus low disease activity state (LLDAS) and remission (clinical SLEDAI-2K = 0), accrual of organ damage, flares and side effects were documented...
February 23, 2018: Seminars in Arthritis and Rheumatism
Franchesca Cruz-Pérez, Salvador Vilá, Grissel Ríos, Luis M Vilá
Macrophage-activating syndrome (MAS) is a rare condition characterized by dysfunctional macrophage activation leading to overproduction of cytokines and phagocytosis of erythrocytes, leukocytes, and platelets. MAS is associated with infectious diseases, malignancies, and autoimmune rheumatic disorders. Herein, we present a 22-year-old Hispanic woman with SLE who was hospitalized because of a three-week history of fever, fatigue, polyarthralgia, nausea, and abdominal pain. Initial laboratories showed severe pancytopenia with marked elevation of liver enzymes and ferritin levels...
2018: Case Reports in Rheumatology
Negiin Pourafshar, Ashkan Karimi, Xuerong Wen, Eric Sobel, Shirin Pourafshar, Nikhil Agrawal, Emma Segal, Rajesh Mohandas, Mark S Segal
Background: Monitoring of mycophenolic acid (MPA) levels may be useful for effective mycophenolate mofetil (MMF) dosing. However, whether commonly obtained trough levels are an acceptable method of surveillance remains debatable. We hypothesized that trough levels of MPA would be a poor predictor of area under the curve (AUC) for MPA. Methods: A total of 51 patients with lupus nephritis who were on MMF 1500 mg twice a day and had a 4-h AUC done were included in this study...
March 13, 2018: Nephrology, Dialysis, Transplantation
Reiko Yamada, Takashi Sakuno, Hiroyuki Inoue, Hiroshi Miura, Toshifumi Takeuchi, Yasunori Shiono, Hiroaki Okuse, Misaki Nakamura, Masaki Katsurahara, Yasuhiko Hamada, Kyosuke Tanaka, Noriyuki Horiki, Yoshiyuki Takei
A 49-year-old man was admitted to our hospital with pancreatitis. He was diagnosed with systemic lupus erythematosus at 34 years of age and was being treated with oral tacrolimus (3 mg/day) and predonine (10 mg/day) for the past 15 months. The computed tomography (CT) scan showed the mass lesion had invaded the pancreatic head via thickening of the duodenal wall. Upper gastrointestinal endoscopy showed the all-round ulcerative lesion from the superior duodenal angle to the descending portion. Histological examination confirmed the diagnosis of diffuse large B cell lymphoma (DLBCL)...
March 15, 2018: Clinical Journal of Gastroenterology
Ole Petter Rekvig
Systemic lupus erythematosus (SLE) is an inadequately defined syndrome. Etiology and pathogenesis remain largely unknown. SLE is on the other hand a seminal syndrome that has challenged immunologists, biologists, genetics, and clinicians to solve its nature. The syndrome is characterized by multiple, etiologically unlinked manifestations. Unexpectedly, they seem to occur in different stochastically linked clusters, although single gene defects may promote a smaller spectrum of symptoms/criteria typical for SLE...
2018: Frontiers in Immunology
Daniel Geh, Caroline Gordon
Systemic lupus erythematosus (SLE) is a multi-system autoimmune disease. There are three drugs licensed for the treatment of lupus: corticosteroids, hydroxychloroquine and belimumab. Immunosuppressants such as azathioprine, methotrexate and mycophenolate are also used. Despite these treatments there is still considerable morbidity. New treatments are needed for the management of active lupus. Epratuzumab a humanized IgG1 monoclonal antibody that targets CD22 resulting in selective B cell modulation that has been considered a potential treatment for SLE...
March 15, 2018: Expert Review of Clinical Immunology
Logan W Thomas, Ashley Elsensohn, Terese Bergheim, Jessica Shiu, Anand Ganesan
IMPORTANCE: Intramuscular (IM) steroids can be used to treat a wide variety of dermatologic diseases. Although seemingly effective and safe, this form of corticosteroid therapy may be underused amongst dermatologists. OBJECTIVE: The objective of this review is to determine the evidence regarding the efficacy and side effect profile of intramuscular triamcinolone in the treatment of dermatologic disease. EVIDENCE REVIEW: A PubMed search engine was used for this study...
March 1, 2018: Journal of Drugs in Dermatology: JDD
Yingjie Lu, Hongmiao Wang, Hua Zheng, Xiao Li
Lupus vulgaris is a common form of cutaneous tuberculosis in China, mostly involving the head and neck region. Turkey ear is a clinically descriptive term, used for a massively enlarged earlobe with bluish-red or violaceous indurated plaques and nodules, which can be a sign of lupus vulgaris. A 47-year-old female presented with edema and reddish ulcerated lesions on both ears which was diagnosed as lupus vulgaris by conventional laboratory investigations and the patient showed good response to antituberculous therapy...
March 14, 2018: Indian Journal of Dermatology, Venereology and Leprology
Roberta Gualtierotti, Maria Orietta Borghi, Maria Gerosa, Tommaso Schioppo, Paola Larghi, Jens Geginat, Pier Luigi Meroni
OBJECTIVES: B cells play an important role in the initiation and progression of systemic lupus erythematosus (SLE). Accordingly, B cell-targeted therapy has been suggested as a new rational approach for treating lupus. Belimumab, a human monoclonal antibody directed against B lymphocyte stimulator (BLyS), was reported as the first biological treatment effective in reducing mild-to-moderate SLE disease activity by using different scoring systems and endpoints. Conversely clinical trials with rituximab, a chimeric monoclonal antibody directed against the CD20 expressed by B cells, have failed to achieve primary endpoints in spite of a number of reports showing its beneficial effects...
February 27, 2018: Clinical and Experimental Rheumatology
Ayako Ohyama, Hiroto Tsuboi, Hisashi Noma, Mayu Terasaki, Masaru Shimizu, Hirofumi Toko, Fumika Honda, Mizuki Yagishita, Hiroyuki Takahashi, Hiromitsu Asashima, Shinya Hagiwara, Yuya Kondo, Isao Matsumoto, Takayuki Sumida
OBJECTIVES: The purpose of this study is to clarify associations between maternal clinical features and adverse pregnancy outcomes (APOs) in mothers with connective tissue diseases (CTDs). METHODS: We retrospectively examined maternal clinical features including backgrounds, autoantibodies, CTD flare-ups, and therapies during pregnancies as well as fetal outcomes in 90 pregnancies (66 mothers) at our hospital from January 2006 to September 2016. RESULTS: Underlying CTDs were SLE (N = 41), MCTD (N = 10), RA (N = 15), SS (N = 10), and others (N = 14)...
March 13, 2018: Modern Rheumatology
Tomás Urrego, Blanca Ortiz-Reyes, Adriana L Vanegas-García, Carlos H Muñoz, Luis A González, Gloria Vásquez, José A Gómez-Puerta
BACKGROUND AND OBJECTIVE: Diagnosis of lupus nephritis (LN) is usually based on renal biopsy, which is an invasive technique that involves multiple risks. Therefore, different biomarkers have emerged as alternatives for the diagnosis of LN. Nonetheless, studies regarding urinary biomarkers in Latin American patients are limited. The objective of this study was to assess the diagnostic value of urinary transferrin and ceruloplasmin to differentiate patients who have renal involvement from those who do not...
March 9, 2018: Reumatología Clinica
Lu Li, Hai-Dong Fu
Neutrophil extracellular traps (NETs) represent a form of cell death distinct from apoptosis or necrosis. The imbalance between the formation and degradation of NETs has long been considered to be closely associated with the activity of autoimmune diseases such as systemic lupus erythematous (SLE). Reactive oxygen species derived from the nicotinamide adenine dinucleotide phosphate oxidase pathway or mitochondrial DNA pathway play a key role in the primary stage of NETs formation. The exposure or delayed degradation of abundant autoantigens, such as double-strand DNA, caused by abnormal activation of neutrophils can induce autoantibody to form immune complexes that deposit in local tissues and then induce the plasmacytoid dendritic cells to secrete the interferon alpha and other inflammatory factors...
March 2018: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
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