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https://www.readbyqxmd.com/read/28087478/renal-involvement-in-lysinuric-protein-intolerance-contribution-of-pathology-to-assessment-of-heterogeneity-of-renal-lesions
#1
Emmanuel Estève, Pauline Krug, Aurélie Hummel, Jean-Baptiste Arnoux, Olivia Boyer, Anais Brassier, Pascale de Lonlay, Vincent Vuiblet, Stéphanie Gobin, Rémi Salomon, Christine Piètrement, Jean-Paul Bonnefont, Aude Servais, Louise Galmiche
Lysinuric protein intolerance (LPI) is a rare autosomal recessive disease caused by mutations in the SLC7A7 gene encoding the light subunit of a cationic amino acid transporter. Symptoms mimic primary urea cycle defects but dysimmune symptoms are also described. Renal involvement in LPI was first described in the 1980's. In 2007, it appeared that it could concern as much as 75% of LPI patients and could lead to end stage renal disease. The most common feature is proximal tubular dysfunction and nephrocalcinosis but glomerular lesions are also reported...
January 10, 2017: Human Pathology
https://www.readbyqxmd.com/read/28086993/lack-of-partial-renal-response-by-12%C3%A2-weeks-after-induction-therapy-predicts-poor-renal-response-and-systemic-damage-accrual-in-lupus-nephritis-class-iii-or-iv
#2
Hironari Hanaoka, Hidehiro Yamada, Tomofumi Kiyokawa, Harunobu Iida, Takeshi Suzuki, Yoshioki Yamasaki, Seido Ooka, Hiroko Nagafuchi, Takahiro Okazaki, Daisuke Ichikawa, Sayuri Shirai, Yugo Shibagaki, Junki Koike, Shoichi Ozaki
BACKGROUND: Lupus nephritis class III or IV is associated with a poor prognosis for both patient and renal survival. Recommendations for the management of lupus nephritis have recently been established, and changing therapies is recommended for patients who do not respond adequately to induction therapy. However, it remains a major challenge to determine when to switch the treatment. In this study, we identified early prognostic factors capable of predicting poor renal outcome as well as overall damage accrual in patients with lupus nephritis class III or IV...
January 13, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28079914/intravenous-immunoglobulin-in-pediatric-rheumatology-when-to-use-it-and-what-is-the-evidence
#3
Martha M Rodriguez, Linda Wagner-Weiner
Intravenous immunoglobulin (IVIG) is given to children with a variety of rheumatologic illnesses. The mechanism of action by which it exerts therapeutic effects is not well understood and likely differs in the medical conditions for which it is given. IVIG is approved by the US Food and Drug Administration and is the standard of care for Kawasaki disease, but most IVIG use in pediatric rheumatology is "off-label. " The literature supports the use of IVIG for juvenile dermatomyositis, although it is unclear whether its use should be limited to those children with more severe or refractory disease...
January 1, 2017: Pediatric Annals
https://www.readbyqxmd.com/read/28070763/uveitis-in-childhood-onset-systemic-lupus-erythematosus-patients-a-multicenter-survey
#4
Paola Pinheiro Kahwage, Mariana Paes Leme Ferriani, João M Furtado, Luciana Martins de Carvalho, Gecilmara Salviato Pileggi, Francisco Hugo Rodrigues Gomes, Maria Teresa Terreri, Claudia Saad Magalhães, Rosa Maria Rodrigues Pereira, Silvana Brasilia Sacchetti, Roberto Marini, Eloisa Bonfá, Clovis Artur Silva, Virgínia Paes Leme Ferriani
The aim of this study is to assess uveitis prevalence in a large cohort of childhood-onset systemic lupus erythematosus (cSLE) patients. A retrospective multicenter cohort study including 852 cSLE patients was performed in ten pediatric rheumatology centers (Brazilian cSLE group). An investigator meeting was held and all participants received database training. Uveitis was diagnosed through clinical assessment by the uveitis expert ophthalmologist of each center. Patients with and without uveitis were assessed for lupus clinical/laboratory features and treatments...
January 9, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28068928/newly-recognized-cerebral-infarctions-on-postmortem-imaging-a-report-of-three-cases-with-systemic-infectious-disease
#5
Sakon Noriki, Kazuyuki Kinoshita, Kunihiro Inai, Toyohiko Sakai, Hirohiko Kimura, Takahiro Yamauchi, Masayuki Iwano, Hironobu Naiki
BACKGROUND: Postmortem imaging (PMI) refers to the imaging of cadavers by computed tomography (CT) and/or magnetic resonance imaging (MRI). Three cases of cerebral infarctions that were not found during life but were newly recognized on PMI and were associated with severe systemic infections are presented. CASE PRESENTATIONS: An 81-year-old woman with a pacemaker and slightly impaired liver function presented with fever. Imaging suggested interstitial pneumonia and an iliopsoas abscess, and blood tests showed liver dysfunction and disseminated intravascular coagulation (DIC)...
January 10, 2017: BMC Medical Imaging
https://www.readbyqxmd.com/read/28065614/cerebral-venous-sinus-thrombosis-presenting-feature-of-systemic-lupus-erythematosus
#6
Rajesh Kumar Singh, Sanjeev Kumar Bhoi, Jayantee Kalita, Usha Kant Misra
BACKGROUND: CVST (cerebral venous sinus thrombosis) may sometimes be associated with autoimmune disorders that require specific treatment. The clinical and magnetic resonance imaging (MRI) findings of systemic lupus erythematosus (SLE) patients with CVST are presented and contrasted with CVST without SLE. METHODS: Consecutive patients with CVST admitted in neurology service during 2012-2016 were included. The diagnosis of CVST was confirmed by MR venography or digital subtraction angiography...
January 5, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28060988/-neuromyelitis-optica-presenting-concomitantly-with-systemic-lupus-erythematosus-report-of-one-case
#7
Felipe Suárez H, Daniela Urrutia E, Felipe Canales P, Camila Gutiérrez O
Neuromyelitis optica (NMO) is a severe demyelinating disease of the central nervous system, which preferentially attacks the optic nerve and spinal cord. It is associated with antibodies against aquaporin 4. Morbidity and mortality are higher than in multiple sclerosis and its treatment focuses on immunosuppressive drugs. Immunomodulators are contraindicated. We report a previously healthy 35-year-old man, presenting with NMO concomitantly with systemic lupus erythematosus. His evolution was torpid with three outbreaks in the 10 months after the diagnosis, requiring a first-line therapy with methylprednisolone and cyclophosphamide and then a second-line therapy with rituximab...
September 2016: Revista Médica de Chile
https://www.readbyqxmd.com/read/28060446/successful-treatment-of-donor-derived-hepatitis-c-viral-infection-in-three-transplant-recipients-from-a-donor-at-increased-risk-for-bloodborne-pathogens
#8
Ashesh P Shah, Andrew Cameron, Pooja Singh, Adam M Frank, Jonathan M Fenkel
We report here the successful treatment of hepatitis C virus (HCV) transmitted from a nucleic acid testing (NAT)-negative donor to 3 HCV-negative recipients - 2 renal transplants and 1 liver. Both renal recipients underwent standard deceased-donor renal transplantation with immediate graft function. The liver recipient underwent standard orthotopic liver transplantation and recovered uneventfully. The donor was a 39-year-old woman with a terminal serum creatinine of 0.7 mg/dL. She was high risk for bloodborne pathogens, based upon a history of sexual contact with an HCV-infected male partner...
January 6, 2017: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/28059023/the-short-term-efficacy-of-bortezomib-combined-with-glucocorticoids-for-the-treatment-of-refractory-lupus-nephritis
#9
H Zhang, Z Liu, L Huang, J Hou, M Zhou, X Huang, W Hu, Z Liu
Objective The treatment of refractory lupus nephritis (LN) remains challenging for clinicians because these patients either do not respond to conventional therapy or relapse during the maintenance treatment period. The aim of this study was to investigate the efficacy and safety of bortezomib combined with glucocorticoids in refractory lupus patients. Methodology Five refractory LN patients aged 21 to 43 years (four females and one male) with biopsy-proven diagnosis (four with type IV and one with type V+IV) were recruited...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28059019/retrospective-review-of-lupus-nephritis-in-a-new-zealand-multi-ethnic-cohort
#10
E Ly, H Thein, Michael Lam Po Tang
: Increased lupus nephritis has been reported in Pacific Island and Maori populations. Previous studies suggest ethnic variation in response to immunosuppression treatment; however this has not been assessed in Pacific Island and Maori cohorts. This retrospective study reviewed class 3, 4 and 5 lupus nephritis outcomes and response to induction immunosuppression over a 10-year period in a New Zealand multi-ethnic cohort with high Pacific Island representation. This included 49 renal biopsies in 41 patients; by ethnicity Pacific Island 53...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28057097/-assessment-right-atrial-function-in-patients-with-systemic-lupus-erythematosus-by-speckle-tracking-and-three-dimensional-echocardiography
#11
X Y Ge, L Shao, Z L Zheng
Objective: To evaluate right atrial function in systemic lupus erythematosus (SLE) with normal pulmonary pressure by using two dimensional-speckle tracking imaging (2D-STI) and real-time three-dimensional echocardiography(RT-3DE). Methods: A totoal of 40 patients with SLE and 40 control subjects were collected between December 2011 and May 2014 from Ningbo Medical Treatment Center Lihuili Hospital. Right atrial global longitudinal strain (RAGLS) and all kinds of right atrium volume index (RAVI) were detected...
December 20, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28056333/-recommendation-for-the-prevention-and-treatment-of-non-steroidal-anti-inflammatory-drug-induced-gastrointestinal-ulcers-and-its-complications
#12
(no author information available yet)
Non-steroidal anti-inflammatory drugs (NSAIDs) are a broad class of non glucocorticoid drugs which are extensively used in anti-inflammatory, analgesic, and antipyretic therapies. However, NSAIDs may cause many side effects, most commonly in gastrointestinal(GI) tract. Cardiovascular system, kidney, liver, central nervous system and hematopoietic system are also involved. NSAID-induced GI side effects not only endanger the patients' health, increase mortality, but also greatly increase the cost of medical care...
January 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/28056158/mycobacterium-avium-complex-septic-arthritis-presenting-as-osteonecrosis-of-the-femoral-head-in-a-patient-with-systemic-lupus-erythematosus
#13
Alexander B Christ, Elena V Zininberg, Kethy M Jules-Elysee, Michael L Parks
Mycobacterium avium complex is a rare cause of musculoskeletal infection, usually occurring in patients with compromised immune systems. Obtaining the diagnosis requires a high index of suspicion, and treatment can be delayed because of difficulty with isolating the organism. Treatment involves prolonged, targeted combination antibiotic therapy, and it is unclear whether eradication of the infection can occur in the presence of a foreign body, such as antibiotic spacers. The authors report a case of M avium infection presenting as presumed osteonecrosis of the femoral head in a young woman with systemic lupus erythematosus...
January 5, 2017: Orthopedics
https://www.readbyqxmd.com/read/28053559/systemic-lupus-erythematosus-and-pulmonary-arterial-hypertension-links-risks-and-management-strategies
#14
REVIEW
Konstantinos Tselios, Dafna D Gladman, Murray B Urowitz
Systemic lupus erythematosus (SLE) is characterized by the second highest prevalence of pulmonary arterial hypertension (PAH), after systemic sclerosis, among the connective tissue diseases. SLE-associated PAH is hemodynamically defined by increased mean pulmonary artery pressure at rest (≥25 mmHg) with normal pulmonary capillary wedge pressure (≤15 mmHg) and increased pulmonary vascular resistance. Estimated prevalence ranges from 0.5% to 17.5% depending on the diagnostic method used and the threshold of right ventricular systolic pressure in studies using transthoracic echocardiogram...
2017: Open Access Rheumatology: Research and Reviews
https://www.readbyqxmd.com/read/28052948/tb-or-not-to-be-kikuchi-fujimoto-disease-a-rare-but-important-differential-for-tb
#15
C McKenna, T Whitfield, N Patel, A Bonington
A 29-year-old British Pakistani woman presented with a 2-month history of drenching fevers, night sweats, lethargy and tender cervical and axillary lymphadenopathy. Initial investigations, bloods and imaging were unremarkable. Fever persisted during her admission, and treatment for tuberculosis (TB) lymphadenitis was started postbiopsy until histology confirmed a diagnosis of Kikuchi-Fujimoto's disease (KFD). KFD has a non-specific presentation of fever, night sweats and lymphadenopathy and commonly raises a clinical suspicion of a number of other serious conditions such as TB, lymphoma, HIV, systemic lupus erythematous, toxoplasmosis and infectious mononucleosis...
January 4, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28050729/acute-lupus-choroidopathy-multimodal-imaging-and-differential-diagnosis-from-central-serous-chorioretinopathy
#16
Murat Hasanreisoglu, Gokcen D Gulpinar Ikiz, Hamit Kucuk, Ozkan Varan, Sengul Ozdek
IMPORTANCE: Systemic lupus erythematosus (SLE) is a thoroughly examined multi-organ disease whose common ocular complications are also well documented. However, SLE choroidopathy, being a rare ocular complication, is needed to be better understood, in order to make the differential diagnosis from clinical conditions such as central serous chorioretinopathy and provide adequate prompt treatment. OBJECTIVE: To provide a clear understanding for the clinical course of SLE choroidopathy by the help of enhanced depth imaging optical coherence tomography, fluorescein angiography, and indocyanine angiography...
January 3, 2017: International Ophthalmology
https://www.readbyqxmd.com/read/28042125/lupus-myocarditis-initial-presentation-and-longterm-outcomes-in-a-multicentric-series-of-29-patients
#17
Guillemette Thomas, Fleur Cohen Aubart, Laurent Chiche, Julien Haroche, Miguel Hié, Baptiste Hervier, Nathalie Costedoat-Chalumeau, Karine Mazodier, Mikael Ebbo, Philippe Cluzel, Nadège Cordel, David Ribes, Jean Chastre, Nicolas Schleinitz, Véronique Veit, Jean-Charles Piette, Jean-Robert Harlé, Alain Combes, Zahir Amoura
OBJECTIVE: Cardiac involvement during systemic lupus erythematosus (SLE) may include the pericardium, myocardium, valvular tissue, and coronary arteries. The aim of this study was to describe the clinical, biological, and radiological presentation of lupus myocarditis (LM) as well as the treatment response and longterm outcomes. METHODS: We conducted a multicentric retrospective study of LM from January 2000 to May 2014. RESULTS: Twenty-nine patients (3 men and 26 women) fulfilled the inclusion criteria (median age at the diagnosis of SLE: 30 yrs, range 16-57)...
January 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28040536/th9-cells-and-il-9-in-autoimmune-disorders-pathogenesis-and-therapeutic-potentials
#18
REVIEW
Yaxiong Deng, Zijun Wang, Christopher Chang, Liwei Lu, Chak Sing Lau, Qianjin Lu
Naïve CD4(+) T cells are pleiotropically divided into various T helper (Th) cell subsets, according to their pivotal roles in the regulation of immune responses. The differentiation of Th9 cells, an interleukin (IL)-9 producing subset, can be impacted by specific environmental cues, co-stimulation with transforming growth factor β (TGF-β) and IL-4, and other regulatory factors. Although IL-9 has been recognized as a classical Th2-related cytokine, recent studies have indicated that IL-9-producing cells contribute to a group of autoimmune disorders including systemic lupus erythematosus (SLE), multiple sclerosis (MS), inflammatory bowel diseases (IBD), rheumatoid arthritis (RA) and psoriasis...
December 28, 2016: Human Immunology
https://www.readbyqxmd.com/read/28039412/cervical-neoplasia-in-systemic-lupus-erythematosus-a-nationwide-study
#19
Hjalmar Wadström, Elizabeth V Arkema, Christopher Sjöwall, Johan Askling, Julia F Simard
OBJECTIVE: The aim was to examine the risk of cervical neoplasia in women with SLE, overall and with respect to treatment, compared with women from the general population. METHODS: By linking national Swedish registers, we assembled a cohort including women with SLE (n = 4976) and matched general population comparators (n = 29 703). Two subcohorts of treated SLE patients were defined on the basis of treatment with antimalarials (n = 1942) and other immunosuppressants (AZA, CYC, ciclosporin, MTX, MMF or rituximab; n = 2175)...
December 30, 2016: Rheumatology
https://www.readbyqxmd.com/read/28036035/serum-interleukin-34-levels-are-elevated-in-patients-with-systemic-lupus-erythematosus
#20
Hongxu Wang, Ju Cao, Xiaofei Lai
Interleukin-34 (IL-34) was initially identified as an alternative ligand for the colony-stimulating factor-1 receptor (CSF-1R) to mediate the biology of mononuclear phagocytic cells. Recently, IL-34 was found to be associated with chronic inflammation, such as in rheumatoid arthritis (RA). Both RA and systemic lupus erythematosus (SLE) are multifactorial autoimmune diseases and are characterized by excessive immune and inflammatory responses. Thus, we investigated whether IL-34 is involved in the pathogenesis of SLE...
December 28, 2016: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
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