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https://www.readbyqxmd.com/read/27876626/life-threatening-nlrc4-associated-hyperinflammation-successfully-treated-with-interleukin-18-inhibition
#1
Scott W Canna, Charlotte Girard, Louise Malle, Adriana de Jesus, Neil Romberg, Judith Kelsen, Lea F Surrey, Pierre Russo, Andrew Sleight, Eduardo Schiffrin, Cem Gabay, Raphaela Goldbach-Mansky, Edward M Behrens
NLRC4-inflammasome hyperactivity causes infantile-onset Macrophage Activation Syndrome and enterocolitis with extraordinary serum IL-18 elevation (NLRC4-MAS). Herein, we report a critically ill infant with severe, refractory NLRC4-MAS who showed sustained response to treatment with experimental IL-18 inhibition.
November 19, 2016: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/26314501/editorial-21st-century-storm-chasers-defining-macrophage-activation-syndrome
#2
EDITORIAL
Scott W Canna, Peter A Nigrovic
No abstract text is available yet for this article.
March 2016: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/25963521/new-monogenic-autoinflammatory-diseases-a-clinical-overview
#3
REVIEW
Scott W Canna, Raphaela Goldbach-Mansky
Translating pathogenic insights gained from monogenic defects that cause autoinflammatory diseases into novel therapies has dramatically improved the lives of patients with these syndromes. The last 15 years have focused on the central role of IL-1 in driving autoinflammatory phenotypes and on therapies blocking IL-1 signaling. Recent discoveries from patients unresponsive to IL-1 blockade have highlighted other key inflammatory mediators and pathways. New genetic discoveries have confirmed unifying mechanisms of autoinflammation, including dysregulation of danger sensing, cell stress, and immune-receptor signaling...
July 2015: Seminars in Immunopathology
https://www.readbyqxmd.com/read/25706096/molecular-mechanisms-in-genetically-defined-autoinflammatory-diseases-disorders-of-amplified-danger-signaling
#4
REVIEW
Adriana Almeida de Jesus, Scott W Canna, Yin Liu, Raphaela Goldbach-Mansky
Patients with autoinflammatory diseases present with noninfectious fever flares and systemic and/or disease-specific organ inflammation. Their excessive proinflammatory cytokine and chemokine responses can be life threatening and lead to organ damage over time. Studying such patients has revealed genetic defects that have helped unravel key innate immune pathways, including excessive IL-1 signaling, constitutive NF-κB activation, and, more recently, chronic type I IFN signaling. Discoveries of monogenic defects that lead to activation of proinflammatory cytokines have inspired the use of anticytokine-directed treatment approaches that have been life changing for many patients and have led to the approval of IL-1-blocking agents for a number of autoinflammatory conditions...
2015: Annual Review of Immunology
https://www.readbyqxmd.com/read/25217959/an-activating-nlrc4-inflammasome-mutation-causes-autoinflammation-with-recurrent-macrophage-activation-syndrome
#5
Scott W Canna, Adriana A de Jesus, Sushanth Gouni, Stephen R Brooks, Bernadette Marrero, Yin Liu, Michael A DiMattia, Kristien J M Zaal, Gina A Montealegre Sanchez, Hanna Kim, Dawn Chapelle, Nicole Plass, Yan Huang, Alejandro V Villarino, Angelique Biancotto, Thomas A Fleisher, Joseph A Duncan, John J O'Shea, Susanne Benseler, Alexei Grom, Zuoming Deng, Ronald M Laxer, Raphaela Goldbach-Mansky
Inflammasomes are innate immune sensors that respond to pathogen- and damage-associated signals with caspase-1 activation, interleukin (IL)-1β and IL-18 secretion, and macrophage pyroptosis. The discovery that dominant gain-of-function mutations in NLRP3 cause the cryopyrin-associated periodic syndromes (CAPS) and trigger spontaneous inflammasome activation and IL-1β oversecretion led to successful treatment with IL-1-blocking agents. Herein we report a de novo missense mutation (c.1009A > T, encoding p...
October 2014: Nature Genetics
https://www.readbyqxmd.com/read/24470448/editorial-interferon-%C3%AE-friend-or-foe-in-systemic-juvenile-idiopathic-arthritis-and-adult-onset-still-s-disease
#6
EDITORIAL
Scott W Canna
No abstract text is available yet for this article.
May 2014: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/24470386/brief-report-alternative-activation-of-laser-captured-murine-hemophagocytes
#7
Scott W Canna, Patrícia Costa-Reis, William E Bernal, Niansheng Chu, Kathleen E Sullivan, Michele E Paessler, Edward M Behrens
OBJECTIVE: Hemophagocytes (HPCs) are activated macrophages that have engulfed other hematopoietic cells. Although HPCs are rarely identified in normal spleen tissue and bone marrow, an excess of these macrophages characterizes many cytokine storm syndromes, particularly macrophage activation syndrome and hemophagocytic lymphohistiocytosis. This study was undertaken to assess the functions of HPCs and their significance in acute inflammatory conditions. METHODS: HPCs were generated in wild-type mice using repeated stimulation with Toll-like receptor 9 (TLR-9) and interleukin-10 receptor blockade...
June 2014: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/23553372/interferon-%C3%AE-mediates-anemia-but-is-dispensable-for-fulminant-toll-like-receptor-9-induced-macrophage-activation-syndrome-and-hemophagocytosis-in-mice
#8
Scott W Canna, Julia Wrobel, Niansheng Chu, Portia A Kreiger, Michele Paessler, Edward M Behrens
OBJECTIVE: Macrophage activation syndrome (MAS) is a devastating cytokine storm syndrome complicating many inflammatory diseases and characterized by fever, pancytopenia, and systemic inflammation. It is clinically similar to hemophagocytic lymphohistiocytosis (HLH), which is caused by viral infection of a host with impaired cellular cytotoxicity. Murine models of MAS and HLH illustrate that interferon-γ (IFNγ) is the driving stimulus for hemophagocytosis and immunopathology. This study was undertaken to investigate the inflammatory contributors to a murine model of Toll-like receptor 9 (TLR-9)-induced fulminant MAS...
July 2013: Arthritis and Rheumatism
https://www.readbyqxmd.com/read/23302539/a-17-year-old-with-isolated-proximal-tibiofibular-joint-arthritis
#9
Scott W Canna, Nancy A Chauvin, Jon M Burnham
The proximal tibiofibular joint (TFJ) is rarely affected in rheumatic diseases, and we frequently interpret pain of the lateral knee as the result of overuse or trauma. Nonetheless, the TFJ is a synovial joint that communicates with the tibiofemoral joint in a proportion of patients. While proximal TFJ arthritis has been rarely associated with existing spondyloarthritis, isolated TFJ arthritis as the presenting manifestation of spondyloarthritis has not yet been described. Here, we report the clinical and radiographic presentation of an adolescent with chronic proximal TFJ arthritis heralding spondyloarthritis highly suggestive of ankylosing spondylitis...
2013: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/22560573/making-sense-of-the-cytokine-storm-a-conceptual-framework-for-understanding-diagnosing-and-treating-hemophagocytic-syndromes
#10
REVIEW
Scott W Canna, Edward M Behrens
Cytokine storm syndromes (CSS) are a group of disorders representing a variety of inflammatory causes. The clinical presentations of all CSS can be strikingly similar, creating diagnostic uncertainty. However, clinicians should avoid the temptation to treat all CSS equally, because their inciting inflammatory insults vary widely. Failure to identify and address this underlying trigger results in delayed, inoptimal, or potentially harmful consequences. This review places the hemophagocytic syndromes hemophagocytic lymphohistiocytosis and macrophage activation syndrome within a conceptual model of CSS and provides a logical framework for diagnosis and treatment of CSS of suspected rheumatic origin...
April 2012: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/22089101/not-all-hemophagocytes-are-created-equally-appreciating-the-heterogeneity-of-the-hemophagocytic-syndromes
#11
REVIEW
Scott W Canna, Edward M Behrens
PURPOSE OF REVIEW: The deadly macrophage activation syndrome (MAS) constitutes one of the few rheumatologic emergencies. MAS is part of a larger group of diseases referred to as hemophagocytic syndromes that are seen in infections, malignancies, or genetic immunodeficiencies. Because of the clinical similarity of these diseases, many clinicians are tempted to approach them all similarly, both in diagnostic criteria and treatment paradigms. New work in the field suggests that not all hemophagocytic syndromes are equal...
January 2012: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/21576823/repeated-tlr9-stimulation-results-in-macrophage-activation-syndrome-like-disease-in-mice
#12
Edward M Behrens, Scott W Canna, Katharine Slade, Sheila Rao, Portia A Kreiger, Michele Paessler, Taku Kambayashi, Gary A Koretzky
Hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) are 2 similar diseases characterized by a cytokine storm, overwhelming inflammation, multiorgan dysfunction, and death. Animal models of HLH suggest that disease is driven by IFN-γ produced by CD8⁺ lymphocytes stimulated by persistent antigen exposure. In these models and patients with "primary" HLH, the antigen persists due to genetic defects, resulting in ineffective cytotoxic responses by CD8⁺ T cells and poor pathogen clearance...
June 2011: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/20028520/acute-hepatitis-in-three-patients-with-systemic-juvenile-idiopathic-arthritis-taking-interleukin-1-receptor-antagonist
#13
Scott Canna, Jennifer Frankovich, Gloria Higgins, Michael R Narkewicz, S Russell Nash, J Roger Hollister, Jennifer B Soep, Leonard L Dragone
PURPOSE: We investigated the etiology of acute hepatitis in three children with systemic Juvenile Idiopathic Arthritis (sJIA) taking Interleukin-1 receptor antagonist (IL1RA). METHODS: Laboratory and clinical data for three children with sJIA diagnosed at ages 13 months to 8 years who developed acute hepatitis during treatment with IL1RA were reviewed for evidence of sJIA flare, infection, macrophage activation syndrome (MAS), malignancy, and drug reaction. RESULTS: In all patients, hepatitis persisted despite cessation of known hepatotoxic drugs and in absence of known infectious triggers, until discontinuation of IL1RA...
2009: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/17136361/arthropathy-of-neonatal-onset-multisystem-inflammatory-disease-nomid-cinca
#14
Suvimol Chirathivat Hill, Madjimbaye Namde, Andrew Dwyer, Andrew Poznanski, Scott Canna, Raphaela Goldbach-Mansky
BACKGROUND: Neonatal onset multisystem inflammatory disease (NOMID), an autoinflammatory disease, is characterized by fever, chronic urticarial rash, CNS manifestations, and arthropathy. Approximately 50% of patients with NOMID have de novo missense mutations in CIAS1, which is associated with modulation of the IL-1b and apoptotic pathways. Approximately 60% of NOMID patients have prominent arthropathy, most commonly involving the knees, the cause of which remains poorly understood. OBJECTIVE: To more fully describe the findings of NOMID arthropathy on MRI and radiography and to provide a better understanding of the origin of the bony lesions...
February 2007: Pediatric Radiology
https://www.readbyqxmd.com/read/16899778/neonatal-onset-multisystem-inflammatory-disease-responsive-to-interleukin-1beta-inhibition
#15
Raphaela Goldbach-Mansky, Natalie J Dailey, Scott W Canna, Ana Gelabert, Janet Jones, Benjamin I Rubin, H Jeffrey Kim, Carmen Brewer, Christopher Zalewski, Edythe Wiggs, Suvimol Hill, Maria L Turner, Barbara I Karp, Ivona Aksentijevich, Frank Pucino, Scott R Penzak, Margje H Haverkamp, Leonard Stein, Barbara S Adams, Terry L Moore, Robert C Fuhlbrigge, Bracha Shaham, James N Jarvis, Kathleen O'Neil, Richard K Vehe, Laurie O Beitz, Gregory Gardner, William P Hannan, Robert W Warren, William Horn, Joe L Cole, Scott M Paul, Philip N Hawkins, Tuyet Hang Pham, Christopher Snyder, Robert A Wesley, Steven C Hoffmann, Steven M Holland, John A Butman, Daniel L Kastner
BACKGROUND: Neonatal-onset multisystem inflammatory disease is characterized by fever, urticarial rash, aseptic meningitis, deforming arthropathy, hearing loss, and mental retardation. Many patients have mutations in the cold-induced autoinflammatory syndrome 1 (CIAS1) gene, encoding cryopyrin, a protein that regulates inflammation. METHODS: We selected 18 patients with neonatal-onset multisystem inflammatory disease (12 with identifiable CIAS1 mutations) to receive anakinra, an interleukin-1-receptor antagonist (1 to 2 mg per kilogram of body weight per day subcutaneously)...
August 10, 2006: New England Journal of Medicine
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