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https://www.readbyqxmd.com/read/29343274/coexistent-sickle-cell-anemia-and-autoimmune-disease-in-eight-children-pitfalls-and-challenges
#1
Valerie Li-Thiao-Te, Florence Uettwiller, Pierre Quartier, Florence Lacaille, Brigitte Bader-Meunier, Valentine Brousse, Mariane de Montalembert
BACKGROUND: Patients with sickle cell disease (SCD) present a defective activation of the alternate complement pathway that increases the risk of infection and is thought to predispose to autoimmune disease (AID). However, coexisting AID and SCD is rarely reported, suggesting possible underdiagnosis due to an overlapping of the symptoms. STUDY DESIGN: Among 603 patients with SCD followed between 1999 and June 2016, we retrospectively searched for patients with coexisting SCD and AID...
January 17, 2018: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29338588/epstein-barr-virus-infection-and-type-i-interferon-signature-in-patients-with-systemic-lupus-erythematosus
#2
L Han, Y Zhang, Q Wang, M Xin, K Yang, K Lei, M Sun
Epstein-Barr (EB) virus infection has long been speculated to evoke systemic lupus erythematosus (SLE). Since a virus infection can induce interferon (IFN) system activation, we aimed to discover the relationship between the two in the progression of SLE in a Chinese inpatient cohort. Methods Peripheral blood mononuclear cells and sera were isolated from 116 SLE patients and 76 healthy controls. Antibodies against EBV-VCA (IgM and IgG) and EBNA (IgG) along with IFNα in patient sera were detected with enzyme-linked immunosorbent assays...
January 1, 2018: Lupus
https://www.readbyqxmd.com/read/29336179/belimumab-and-antipneumococcal-vaccination-in-patients-with-systemic-lupus-erythematosus
#3
Tudor Azoicai, Sabina Antoniu, Irina Draga Caruntu, Doina Azoicai, Ileana Antohe, Cristina Gavrilovici
In systemic lupus erythematosus (SLE), flares can be caused by infections. In particular, Streptococcus pneumoniae infection can be severe or even potentially lethal in absence of previous immunization or in case of "aggressive" systemic antibiotic therapy. Immunization efficacy, however, can be reduced in such patients with the use of the various immunosuppressive therapeutic regimens. In particular, the use of novel monoclonal antibodies against B lymphocytes raises concerns over the potential interference with antipneumococcal vaccination...
January 16, 2018: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/29332707/cutaneous-hemophagocytosis-clinicopathologic-features-of-21-cases
#4
Francesca Boggio, Viviana Lora, Carlo Cota, Amanda Pereira, Robert Müllegger, Lucia Prieto-Torres, Lorenzo Cerroni
BACKGROUND: Hemophagocytosis is well known in cytotoxic cutaneous T-cell lymphomas (CTCLs), in which it may represent a sign of hemophagocytic lymphohistiocytosis syndrome (HLHS), and is also typical of cutaneous Rosai-Dorfman disease (cRDD) (without prognostic relevance). Only rarely, has cutaneous hemophagocytosis (CH) been described in other skin conditions. OBJECTIVE: To characterize the clinicopathologic features of CH in skin biopsy specimens from patients with conditions other than CTCL or cRDD...
February 2018: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/29329127/cutis-laxa-acquisita-after-urticarial-vasculitis-in-sle-patients
#5
Kimberly B Golisch, Silvija P Gottesman, Patricia Ferrer, Keliegh S Culpepper
Cutis laxa is a rare connective tissue disease involving damage to dermal elastic fibers creating a clinical appearance of loose, sagging skin. The condition can be either acquired or genetic. Autoimmune diseases, neoplasms, infections, and medications have been proposed as the cause of, or in association with, the acquired form. In nearly 50% of cases, erythematous plaques present before the onset of cutis laxa. Separately, urticarial vasculitis and systemic lupus erythematosus have been linked to cutis laxa acquisita...
January 11, 2018: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/29325308/-opportunistic-infection-in-systemic-lupus-erythematosus-patients-the-disease-spectrum-and-the-characteristics-of-peripheral-lymphocyte-subsets
#6
L Qin, Z F Qiu, J Xie, T R Geng, J L Zhao, L Wan, T S Li
Objective: To investigate the common opportunistic infections and the characteristics of peripheral lymphocyte subsets in patients with systemic lupus erythematosus (SLE). Methods: From December 2013 to December 2016, peripheral lymphocyte subsets were consecutively detected by flow cytometry in treated SLE patients with or without opportunistic infections (OIs) . The lymphocyte subsets in healthy donors were used as normal control group. Results: A total of 145 treated SLE patients were enrolled including 108 with OIs and 37 without OIs...
January 1, 2018: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/29321714/cavum-vergae-liability-and-steroid-treatment-manic-episode-brain-imaging-findings-and-clinical-follow-up-of-a-systemic-lupus-erythematosus-case
#7
Emre Misir, İbrahim Tolga Binbay, Bilge Targitay, Hidayet Ece Arat, Gerçek Can, Köksal Alptekin
Systemic lupus erythematosus (SLE) or Lupus is a chronic and idiopathic autoimmune connective tissue disease that involves several organs and organ systems. SLE may lead to a group of psychiatric manifestations, including delirium, anxiety disorders, cognitive dysfunction, mood disorders, and psychosis, which are caused by organic or non-organic factors. In addition, it is thought that the most common cause of neuropsychiatric lupus is corticosteroid use; central nervous system involvement and inflammatory processes also have an important role in the development of psychiatric manifestations...
December 2017: Noro Psikiyatri Arsivi
https://www.readbyqxmd.com/read/29320974/head-to-head-comparison-of-adverse-effects-and-efficacy-between-high-dose-deflazacort-and-high-dose-prednisolone-in-systemic-lupus-erythematosus-a-prospective-cohort-study
#8
A Ganapati, R Ravindran, T David, B Yadav, V Jeyaseelan, L Jeyaseelan, D Danda
Background Deflazacort (DFZ), an oxazoline derivative of prednisolone (PDN), has a dose equivalence of 1.2:1 (mg) to PDN. No study to date has compared adverse effects and efficacy of high doses of DFZ as against high-dose PDN in systemic lupus erythematosus (SLE). Objectives To compare adverse effects of high dose DFZ and PDN in SLE patients, especially in terms of cushingoid features and gain in body weight, 3 and 6 months after initiation of these agents. Methods In both the steroid arms, the following outcome parameters were assessed at 3 and 6 months: (a) cushingoid features by Cushing's Severity Index (CSI) (b) hirsutism by modified Ferriman Gallwey score (c) weight gain by difference (Δ, delta) of weight (in kilograms)...
January 1, 2018: Lupus
https://www.readbyqxmd.com/read/29320972/rituximab-use-in-pediatric-lupus-anticoagulant-hypoprothrombinemia-syndrome-report-of-three-cases-and-review-of-the-literature
#9
K Cetin Gedik, S Siddique, C L Aguiar
Lupus anticoagulant hypoprothrombinemia syndrome (LA-HPS) is a rare condition that may predispose both to thrombosis and bleeding due to positive lupus anticoagulant (LA) and factor II (FII) deficiency. It can be seen in association with infections or systemic lupus erythematosus (SLE) and may require glucocorticoids (GCs) and/or immunosuppressive medications. Pediatric LA-HPS cases in the literature and three cases that received only rituximab (RTX) for LA-HPS (in addition to GCs) at two institutions between January 2010 and June 2017 were analyzed descriptively...
January 1, 2018: Lupus
https://www.readbyqxmd.com/read/29317407/a20-haploinsufficiency-ha20-clinical-phenotypes-and-disease-course-of-patients-with-a-newly-recognised-nf-kb-mediated-autoinflammatory-disease
#10
Florence A Aeschlimann, Ezgi D Batu, Scott W Canna, Ellen Go, Ahmet Gül, Patrycja Hoffmann, Helen L Leavis, Seza Ozen, Daniella M Schwartz, Deborah L Stone, Annet van Royen-Kerkof, Daniel L Kastner, Ivona Aksentijevich, Ronald M Laxer
OBJECTIVES: The association between mutations in TNFAIP3, encoding the NF-kB regulatory protein A20, and a new autoinflammatory disease has recently been recognised. This study aims at describing the clinical phenotypes and disease course of patients with A20 haploinsufficiency (HA20). METHODS: Data for all cases from the initial publication, and additional cases identified through collaborations since, were collected using standardised data collection forms. RESULTS: A total of 16 patients (13 female) from seven families with a genetic diagnosis of HA20 were included...
January 9, 2018: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/29312536/the-shared-and-specific-mechanism-of-four-autoimmune-diseases
#11
Meiwei Luan, Zhenwei Shang, Yanbo Teng, Xinren Chen, Mingming Zhang, Hongchao Lv, Ruijie Zhang
Interaction between genetic and epigenetic mechanisms may lead to autoimmune diseases. The features of these diseases show familial aggregation. The generality and specificity are keys to studying pathogenesis and etiology of them. This research integrated data of genetics and epigenetics, to find disease-related genes based on the levels of expression and regulation, and explored then to the shared and specific mechanism of them by analyzing shared and specific pathways of common four autoimmune diseases, including Type 1 Diabetes Mellitus (T1D), Multiple Sclerosis (MS), Rheumatoid Arthritis (RA) and Systemic Lupus Erythematosus (SLE)...
December 12, 2017: Oncotarget
https://www.readbyqxmd.com/read/29310419/proliferative-lupus-nephritis-in-the-absence-of-overt-systemic-lupus-erythematosus-a-historical-study-of-12-adult-patients
#12
Maxime Touzot, Cécile Saint-Pastou Terrier, Stanislas Faguer, Ingrid Masson, Hélène François, Lionel Couzi, Aurélie Hummel, Nathalie Quellard, Guy Touchard, Noémie Jourde-Chiche, Jean-Michel Goujon, Eric Daugas
Severe lupus nephritis in the absence of systemic lupus erythematosus (SLE) is a rare condition with an unclear clinical presentation and outcome.We conducted a historical observational study of 12 adult (age >18 years) patients with biopsy-proven severe lupus nephritis or lupus-like nephritis without SLE immunological markers at diagnosis or during follow-up. Excluded were patients with chronic infections with HIV or hepatitis B or C; patients with a bacterial infectious disease; and patients with pure membranous nephropathy...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29302827/colonic-perforation-due-to-severe-cytomegalovirus-disease-in-granulomatosis-with-polyangiitis-after-immunosuppression
#13
REVIEW
Alessandra Soriano, Nazareno Smerieri, Stefano Bonilauri, Loredana De Marco, Alberto Cavazza, Carlo Salvarani
Granulomatosis with polyangiitis (GPA) is a small-vessel necrotizing granulomatous vasculitis typically involving upper airways, lungs, and kidneys, which may lead to end-organ damage and life-threatening complications. Major infections during GPA course represent a considerable concern in the management of the disease. Cytomegalovirus (CMV) infection and disease are rare but significant complications in the course of GPA being associated with high morbidity and mortality rates. Colonic perforation due to CMV colitis is exceedingly rare and has so far almost exclusively been documented in HIV, renal transplant, and systemic lupus erythematosus patients...
January 4, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29285180/herpes-zoster-in-patients-with-systemic-lupus-erythematosus-clinical-features-complications-and-risk-factors
#14
Dongying Chen, Hao Li, Jingyi Xie, Zhongping Zhan, Liuqin Liang, Xiuyan Yang
Herpes Zoster (HZ) is reported as the most prevalent viral infection in patients with systemic lupus erythematosus (SLE). The aim of the present study was to investigate the clinical features, complications, and potential risk factors of HZ in patients with SLE from Southern China. A retrospective study was performed among patients with SLE admitted to the First Affiliated Hospital of Sun Yet-Sen University (Guangzhou, China) between 2009 and 2013. Demographic information, clinical and laboratory data, and medications used were collected and analyzed...
December 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29284768/fitz-hugh-curtis-syndrome-caused-by-gonococcal-infection-in-a-patient-with-systemic-lupus-erythematous-a-case-report-and-literature-review
#15
Darío A Rueda, Luisina Aballay, Lisandro Orbea, Diego A Carrozza, Paola Finocchietto, Silvia B Hernandez, Mariano M Volpacchio, Horacio di Fonzo
BACKGROUND Fitz-Hugh-Curtis (FHC) syndrome is a perihepatitis linked to inflammatory pelvic disease. It can be caused by Neisseria gonorrhoeae or Chlamydia trachomatis infections. FHC syndrome usually presents with pain in the right hypochondrium and fever, associated with symptoms and signs of pelvic infection in women. CASE REPORT We present the case of a 22-year-old woman with systemic lupus erythematous (SLE) who presented with polyarthritis, cutaneous lesions, and abdominal pain. The diagnosis of FHC syndrome was based on the findings of abdominal computerized tomography (CT) and the isolation of Neisseria gonorrhoeae (NG) in blood cultures...
December 29, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/29282007/the-risk-of-nontuberculous-mycobacterial-infection-in-patients-with-sj%C3%A3-gren-s-syndrome-a-nationwide-population-based-cohort-study
#16
Wen-Cheng Chao, Ching-Heng Lin, Tsai-Ling Liao, Yi-Ming Chen, Chiann-Yi Hsu, Jun-Peng Chen, Der-Yuan Chen, Hsin-Hua Chen
BACKGROUND: Nontuberculous mycobacterial (NTM) infection in immunocompromized patients is currently a growing health concern, and we aimed to examine the relative risk of NTM infection in patients with Sjögren's syndrome (SS) compared with that in non-SS individuals. METHODS: We used the 2003-2012 Taiwanese National Health Insurance Research Database to identify 6554 incident SS cases during 2007-2012 and selected 98,310 non-SS controls matched (1:15) for age, gender, and the year of first SS diagnosis date after excluding those who had rheumatoid arthritis or systemic lupus erythematosus...
December 28, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/29276769/low-incidence-of-opportunistic-infections-in-lupus-patients-treated-with-cyclophosphamide-and-steroids-in-a-tertiary-care-setting
#17
Shubhasree Banerjee, Ann Biehl, Maryam Ghaderi-Yeganeh, Zerai Manna, Sarfaraz Hasni
Background: Infection is common cause of morbidity and mortality in systemic lupus erythematosus (SLE). Our objective was to determine incidence and types of infections, particularly opportunistic infections, in SLE patients receiving cyclophosphamide, and to identify contribution of variables like demographics, steroid, other immunosuppressives, white blood cell and absolute neutrophil count to infection risk. Patients and Methods: We did retrospective chart review of SLE patients in our institute over last 10 years, who received minimum six cyclophosphamide infusions...
March 2017: Medical Research Archives
https://www.readbyqxmd.com/read/29274214/helminth-infections-in-faecal-samples-of-apennine-wolf-canis-lupus-italicus-and-marsican-brown-bear-ursus-arctos-marsicanus-in-two-protected-national-parks-of-central-italy
#18
Barbara Paoletti, Raffaella Iorio, Donato Traversa, Cristina E Di Francesco, Leonardo Gentile, Simone Angelucci, Cristina Amicucci, Roberto Bartolini, Marianna Marangi, Angela Di Cesare
This article reports the results of a copromicroscopic and molecular investigation carried out on faecal samples of wolves (n=37) and brown bears (n=80) collected in two protected national parks of central Italy (Abruzzo Region). Twenty-three (62.2%) samples from wolves were positive for parasite eggs. Eight (34.78%) samples scored positive for single infections, i.e. E. aerophilus (21.74%), Ancylostoma/Uncinaria (4.34%), Trichuris vulpis (4.34%), T. canis (4.34%). Polyspecific infections were found in 15 samples (65...
2017: Annals of Parasitology
https://www.readbyqxmd.com/read/29259927/gelatinous-transformation-of-bone-marrow-rare-or-underdiagnosed
#19
REVIEW
Khushdeep Kaur Shergill, Gagandeep Singh Shergill, Hari Janardanan Pillai
Gelatinous transformation of the bone marrow (GTBM) is a rare hematologic entity, which was first described by Paul Michael in 1930. GTBM is mostly associated with caloric intake/anorexia nervosa, although it also has been described accompanying other pathologic conditions, such as malignancy, systemic lupus erythematosus, HIV infections. Even though the diagnostic features of the hematopoietic tissue, such as hypoplasia, adipose cell atrophy, and deposition of a gelatinous substance in the bone marrow (which stains with Alcian blue at pH 2...
October 2017: Autopsy & case reports
https://www.readbyqxmd.com/read/29259241/computational-detection-and-quantification-of-human-and-mouse-neutrophil-extracellular-traps-in-flow-cytometry-and-confocal-microscopy
#20
Brandon G Ginley, Tiffany Emmons, Brendon Lutnick, Constantin F Urban, Brahm H Segal, Pinaki Sarder
Neutrophil extracellular traps (NETs) are extracellular defense mechanisms used by neutrophils, where chromatin is expelled together with histones and granular/cytoplasmic proteins. They have become an immunology hotspot, implicated in infections, but also in a diverse array of diseases such as systemic lupus erythematosus, diabetes, and cancer. However, the precise assessment of in vivo relevance in different disease settings has been hampered by limited tools to quantify occurrence of extracellular traps in experimental models and human samples...
December 19, 2017: Scientific Reports
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