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https://www.readbyqxmd.com/read/29239931/a-case-of-amyloidosis-caused-by-sporadic-muckle-wells-syndrome-response-to-treatment-with-anakinra
#1
Ufuk İlgen, Orhan Küçükşahin, Şiyar Erdoğmuş, Gökhan Nergizoğlu
No abstract text is available yet for this article.
December 12, 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/29239928/aspergillus-pneumonia-in-a-patient-with-adult-onset-still-disease-successfully-treated-with-anakinra
#2
Emre Bilgin, Abdulsamet Erden, Levent Kilic, Alper Sari, Berkan Armagan, Umut Kalyoncu, Ömer Karadag
No abstract text is available yet for this article.
December 12, 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/29239128/efficacy-of-anakinra-in-a-patient-with-systemic-amyloidosis-presented-with-amyloidoma
#3
Hulya Nalcacioglu, Ozan Ozkaya, Gurkan Genc, Suat Ayyildiz, Mehmet Kefeli, Murat Elli, Oguz Aydin, Meltem Ceyhan Bilgici
Amyloidosis is a heterogeneous group of disorders characterized by extracellular deposition of unique protein fibrils. The least common presentation of an amyloid deposition is as a discrete mass called amyloidoma or amyloid tumor. We report a case of a soft tissue amyloidoma in the abdomen of a 16-year-old girl who was diagnosed as having systemic amyloidosis. A girl aged 16 years was referred to our hospital with a pre-diagnosis of a retroperitoneal mass documented with abdominal ultrasonography and tomography...
December 13, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/29225799/kidney-involvement-in-the-schnitzler-syndrome-a-rare-disease
#4
REVIEW
Carlo Basile, Luigi Rossi, Francesco Casucci, Annalisa Teutonico, Pasquale Libutti, Piero Lisi, Carlo Lomonte, Raffaele Manna
The Schnitzler syndrome (SS) is a rare and underdiagnosed entity that associates a chronic urticarial rash, monoclonal IgM (or sometimes IgG) gammopathy and signs and symptoms of systemic inflammation. During the past 45 years, the SS has evolved from an elusive little-known disorder to the paradigm of a late-onset acquired auto-inflammatory syndrome. Though there is no definite proof of its precise pathogenesis, it should be considered as an acquired disease involving abnormal stimulation of the innate immune system, which can be reversed by the interleukin-1 receptor antagonist anakinra...
December 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/29214791/a-case-of-schnitzler-s-syndrome-without-monoclonal-gammopathy-associated-chronic-urticaria-treated-with-anakinra
#5
Min Joo Ahn, Ji Eun Yu, Jiung Jeong, Da Woon Sim, Young Il Koh
Chronic urticaria may often be associated with interleukin (IL)-1-mediated autoinflammatory disease, which should be suspected if systemic inflammation signs are present. Here, we report a case of Schnitzler's syndrome without monoclonal gammopathy treated successfully with the IL-1 receptor antagonist anakinra. A 69-year-old man suffered from a pruritic urticarial rash for 12 years. It became aggravated episodically and was accompanied by high fever, arthralgia, leukocytosis, and an elevated C-reactive protein and erythrocyte sedimentation rate...
January 2018: Yonsei Medical Journal
https://www.readbyqxmd.com/read/29166924/experience-with-etanercept-tocilizumab-and-interleukin-1-inhibitors-in-systemic-onset-juvenile-idiopathic-arthritis-patients-from-the-biker-registry
#6
Gerd Horneff, Anna Carina Schulz, Jens Klotsche, Anton Hospach, Kirsten Minden, Ivan Foeldvari, Ralf Trauzeddel, Gerd Ganser, Frank Weller-Heinemann, Johannes Perter Haas
BACKGROUND: Treatment of systemic onset juvenile idiopathic arthritis JIA (sJIA), although dramatically improved, remains a challenge. Experience from clinical practice will be presented using data from the German Biologics register (BiKeR) for evaluation of efficacy and safety of treatment with etanercept (ETA), tocilizumab (TOC) and the interleukin-1 inhibitors anakinra and canakinumab (IL-1i) in sJIA. METHODS: Patients with sJIA documented in the BIKeR register, who were exposed to ETA, TOC or IL-1i were identified...
November 22, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/29150554/cancer-cells-induce-interleukin-22-production-from-memory-cd4-t-cells-via-interleukin-1-to-promote-tumor-growth
#7
Cornelia Voigt, Peter May, Adrian Gottschlich, Anamarija Markota, Daniel Wenk, Inga Gerlach, Sebastian Voigt, Georgios T Stathopoulos, Kristina A M Arendt, Constanze Heise, Felicitas Rataj, Klaus-Peter Janssen, Melanie Königshoff, Hauke Winter, Isabelle Himsl, Wolfgang E Thasler, Max Schnurr, Simon Rothenfußer, Stefan Endres, Sebastian Kobold
IL-22 has been identified as a cancer-promoting cytokine that is secreted by infiltrating immune cells in several cancer models. We hypothesized that IL-22 regulation would occur at the interface between cancer cells and immune cells. Breast and lung cancer cells of murine and human origin induced IL-22 production from memory CD4+ T cells. In the present study, we found that IL-22 production in humans is dependent on activation of the NLRP3 inflammasome with the subsequent release of IL-1β from both myeloid and T cells...
December 5, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29141858/interleukin-1-blockade-in-recently-decompensated-systolic-heart-failure-results-from-redhart-recently-decompensated-heart-failure-anakinra-response-trial
#8
RANDOMIZED CONTROLLED TRIAL
Benjamin W Van Tassell, Justin Canada, Salvatore Carbone, Cory Trankle, Leo Buckley, Claudia Oddi Erdle, Nayef A Abouzaki, Dave Dixon, Dinesh Kadariya, Sanah Christopher, Aaron Schatz, Jessica Regan, Michele Viscusi, Marco Del Buono, Ryan Melchior, Pranav Mankad, Juan Lu, Robin Sculthorpe, Giuseppe Biondi-Zoccai, Edward Lesnefsky, Ross Arena, Antonio Abbate
BACKGROUND: An enhanced inflammatory response predicts worse outcomes in heart failure (HF). We hypothesized that administration of IL-1 (interleukin-1) receptor antagonist (anakinra) could inhibit the inflammatory response and improve peak aerobic exercise capacity in patients with recently decompensated systolic HF. METHODS AND RESULTS: We randomly assigned 60 patients with reduced left ventricular ejection fraction (<50%) and elevated C-reactive protein levels (>2 mg/L), within 14 days of hospital discharge, to daily subcutaneous injections with anakinra 100 mg for 2 weeks, 12 weeks, or placebo...
November 2017: Circulation. Heart Failure
https://www.readbyqxmd.com/read/29111302/-cryopyrin-associated-periodic-syndromes
#9
P Quartier, F Rodrigues, S Georgin-Lavialle
Cryopyrin-associated periodic syndromes (CAPS) are linked to one single gene mutations, however they are associated with 3 syndromes, which are, from the mildest to the most severe phenotype familial cold urticaria, Muckle-Wells syndrome and chronic, infantile, neurologic, cutaneous, articular (CINCA) syndrome also called neonatal-onset multisystem inflammatory disease (NOMID). Autosomic dominant inheritance is present in most cases but in CINCA/NOMID syndrome where neomutations are more common. Mutations in the gene encoding cryopyrin, NLRP3, are associated with deregulation of caspase-1 activity, excessive interleukin-1 production and an autoinflammatory syndrome, which in familial cold urticaria and Muckle-Wells syndrome may be triggered or worsened by exposure to coldness...
October 27, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/29110911/blau-syndrome-with-good-reponses-to-tocilizumab-a-case-report-and-focused-literature-review
#10
Li Lu, Min Shen, Dongbin Jiang, Yanmin Li, Xiaolong Zheng, Yao Li, Zhixun Li, Leilei Zhang, Jungen Tang, Yanke Guo, Shengyun Liu, Zhaohui Zheng, Guanmin Gao, Quancheng Kan
OBJECTIVES: Blau syndrome (BS), a rare auto-inflammatory granulomatous disease, is a progressive disorder. Usually the maintenance dose of glucocorticoid may not be tapered below 15 mg per day while immunosuppressives is used. There has been some experience with biologic agents in refractory BS patients. The objective of this study is to describe the case of a BS patient benefiting from Tocilizumab, a humanized monoclonal antibody against interleukin 6 receptor. METHODS: We report the first Chinese patient with BS who was resistant to currently available therapies but had rapid quiescence after using Tocilizumab...
September 25, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/29098911/targeted-treatments-for-hidradenitis-suppurativa-a-review-of-the-current-literature-and-ongoing-clinical-trials
#11
Melody Maarouf, Ashley K Clark, Dylan E Lee, Vivian Y Shi
PURPOSE: Targeted, immune-modulating drugs are at the forefront of therapy for HS, and a comprehensive clinical trial registry is needed to facilitate data pooling and clinical efficacy comparison. MATERIALS AND METHODS: A systematic review of the ClinicalTrials.gov database was searched for planned, in-progress, completed, or terminated trials investigating the effect of targeted biologic therapies for hidradenitis suppurativa (HS). When results of RCTs were not available, case reports or series were included...
November 10, 2017: Journal of Dermatological Treatment
https://www.readbyqxmd.com/read/29081616/biological-response-modifiers-in-rheumatoid-arthritis-systematic-review-and-meta-analysis-of-safety
#12
Nitishkumar D Tank, Bharti N Karelia, Bhavisha N Vegada
OBJECTIVE: To analyze available evidence on the safety of different biological response modifiers which are used for a treatment of rheumatoid arthritis (RA). MATERIALS AND METHODS: We searched systematically for randomized controlled clinical trials on treatment of RA with different biological response modifiers, followed by a systematic review with meta-analysis. Trials were searched from MEDLINE and Cochrane Library databases. The following safety parameters reported in the selected trials were analyzed: number of patients suffering any adverse event (AE), withdrawal due to AEs, serious AE (SAEs), infections, serious infections, infusion reactions, injection site reactions, malignancies, and overall mortality...
July 2017: Journal of Pharmacology & Pharmacotherapeutics
https://www.readbyqxmd.com/read/29034454/successful-treatment-of-sapho-syndrome-with-apremilast
#13
S Adamo, J Nilsson, A Krebs, U Steiner, A Cozzio, L E French, A G A Kolios
Synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome is a rare disease with inflammatory osteoarticular and skin involvement. The pathogenesis of SAPHO syndrome remains unclear, but evidence suggests it may be an autoinflammatory disease triggered upon exposure to infectious agents in genetically predisposed individuals. Induction of the IL-23/Th17 axis as well as neutrophil activation seem to play a key role, and therapies targeting these immunological pathways, including TNF-inhibitors, ustekinumab, secukinumab and the IL-1 inhibitor anakinra are potential treatment options that need further investigation...
October 16, 2017: British Journal of Dermatology
https://www.readbyqxmd.com/read/28993312/mmp-14-overexpression-correlates-with-the-neurodegenerative-process-in-familial-amyloidotic-polyneuropathy
#14
Diana Martins, João Moreira, Nádia Pereira Gonçalves, Maria João Saraiva
Levels of matrix metalloproteases (MMPs) can be differentially regulated in response to injury or neurological diseases. For instance, it is known that selective and short-term inhibition of MMP-14, a membrane-type 1 MMP, accelerates axon regeneration. Because axon growth and regeneration is impaired in familial amyloidotic polyneuropathy (FAP), a neurodegenerative disorder characterized by misfolding and deposition of mutant transthyretin (TTR) in the peripheral nervous system (PNS), we presently investigated the expression levels and the potential role for MMP-14 in this condition...
October 1, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28992387/a-nationwide-experience-with-the-off-label-use-of-interleukin-1-targeting-treatment-in-familial-mediterranean-fever-patients
#15
Servet Akar, Pınar Cetin, Umut Kalyoncu, Omer Karadag, Ismail Sari, Muhammed Cınar, Sedat Yilmaz, Ahmet Mesut Onat, Bunyamin Kisacik, Abdulsamet Erten, Ayse Balkarli, Orhan Kucuksahin, Sibel Yilmaz Oner, Soner Senel, Abdurrahman Tufan, Haner Direskeneli, Ferhat Oksuz, Yavuz Pehlivan, Ozun Bayindir, Gokhan Keser, Kenan Aksu, Ahmet Omma, Timucin Kasifoglu, Ali Ugur Unal, Fatih Yildiz, Mehmet Ali Balci, Sule Yavuz, Sukran Erten, Metin Ozgen, Mehmet Sayarlıoglu, Atalay Dogru, Gozde Yildirim, Fatma Alibaz Oner, Mehmet Engin Tezcan, Omer Nuri Pamuk, Fatos Onen
OBJECTIVE: Around 30-45% of patients with familial Mediterranean fever (FMF) have been reported to have attacks despite colchicine treatment. Currently, data on the treatment of colchicine-unresponsive or colchicine-intolerant FMF patients are limited; the most promising alternatives seem to be anti-interleukin-1 (anit-IL1) agents. Herein we report our experience with the off-label use of anti-IL1 agents in a large group of FMF patients. METHODS: In all, 21 centers from different geographical regions of Turkey were included in the current study...
October 9, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/28984997/nlrp3-il-1%C3%AE-mediates-denervation-during-bladder-outlet-obstruction-in-rats
#16
Robin Lütolf, Francis M Hughes, Brian M Inouye, Huixia Jin, Jennifer C McMains, Elena S Pak, Johanna L Hannan, J Todd Purves
AIMS: Denervation of the bladder is a detrimental consequence of bladder outlet obstruction (BOO). We have previously shown that, during BOO, inflammation triggered by the NLRP3 inflammasome in the urothelia mediates physiological bladder dysfunction and downstream fibrosis in rats. The aim of this study was to assess the effect of NLRP3-mediated inflammation on bladder denervation during BOO. METHODS: There were five groups of rats: (i) Control (no surgery); (ii) Sham-operated; (iii) BOO rats given vehicle; (iv) BOO rats given the NLRP3 inhibitor glyburide; and (v) BOO rats given the IL-1 receptor antagonist anakinra...
October 6, 2017: Neurourology and Urodynamics
https://www.readbyqxmd.com/read/28980900/one-year-in-review-2017-beh%C3%A3-et-s-syndrome
#17
REVIEW
Gulen Hatemi, Emire Seyahi, Izzet Fresko, Rosaria Talarico, Vedat Hamuryudan
A meta-analysis showed that methodological differences in prevalence studies such as a sample survey design or census design may be responsible for some of the variance in BS prevalence reported across countries, in addition to a true geographic variation. Efforts towards developing a data driven core set of outcome measures for clinical trials is continuing. Multimodal imaging using color fundus photography, fluorescein angiography, and optical coherence tomography is essential in visualising diagnostic features, detecting structural changes, and monitoring disease activity and response to treatment in Behçet's uveitis...
September 29, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28978596/safe-usage-of-anakinra-and-dexamethasone-to-treat-refractory-hemophagocytic-lymphohistiocytosis-secondary-to-acute-disseminated-histoplasmosis-in-a-patient-with-hiv-aids
#18
Anthony J Ocon, Birju D Bhatt, Cynthia Miller, Ruben A Peredo
Hemophagocytic lymphohistiocytosis (HLH) is a serious life-threatening disease if not recognised early. In patients with HIV/AIDS, this association has been reported following acute opportunistic infections, including histoplasmosis. However, optimal treatment is not known. We describe a male aged 46 years with AIDS who developed HLH following acute disseminated histoplasmosis. Presenting symptoms included fever, hepatosplenomegaly and pancytopenia. Bone marrow biopsy confirmed HLH. Initially, he was refractory to the treatment with amphotericin B, antiretroviral therapy and intravenous immunoglobulin (IVIG)...
October 4, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28968868/international-multi-centre-study-of-pregnancy-outcomes-with-interleukin-1-inhibitors
#19
Taryn Youngstein, Patrycja Hoffmann, Ahmet Gül, Thirusha Lane, Rene Williams, Dorota M Rowczenio, Huri Ozdogan, Serdal Ugurlu, John Ryan, Len Harty, Sean Riminton, Alex P Headley, Joachim Roesler, Norbert Blank, Jasmin B Kuemmerle-Deschner, Anna Simon, Adrian S Woolf, Philip N Hawkins, Helen J Lachmann
Objective: To provide outcome data concerning pregnancies exposed to the Interleukin-1 (IL-1) inhibitors prior to conception in both men and women, during pregnancy and breast feeding. Methods: Retrospective data were collected from members of the International Society for Systemic Autoinflammatory diseases and collated in a single centre. A uniform data collection sheet was used to obtain standardized data including maternal age and diagnosis, type, duration of and response to IL-1 blockade, pregnancy duration, delivery, mode of feeding and neonatal development...
August 23, 2017: Rheumatology
https://www.readbyqxmd.com/read/28957335/fetal-skin-as-a-pro-inflammatory-organ-evidence-from-a-primate-model-of-chorioamnionitis
#20
Suppawat Boonkasidecha, Paranthaman Senthamarai Kannan, Suhas G Kallapur, Alan H Jobe, Matthew W Kemp
BACKGROUND: Intrauterine infection is a primary cause of preterm birth and fetal injury. The pro-inflammatory role of the fetal skin in the setting of intrauterine infection remains poorly characterized. Whether or not inflammation of the fetal skin occurs in primates remains unstudied. Accordingly, we hypothesized that: i) the fetal primate skin would mount a pro-inflammatory response to preterm birth associated pro-inflammatory agents (lipopolysaccharides from Escherichia coli, live Ureaplasma parvum, interleukin-1β) and; ii) that inhibiting interleukin-1 signaling would decrease the skin inflammatory response...
2017: PloS One
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