Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment

Munc13-4 Regulates Granule Secretion in Human Neutrophils

(2014) C. Pivot-Pajot et al.   JOURNAL OF IMMUNOLOGY

Rapid and Sustained Remission of Systemic Juvenile Idiopathic Arthritis-Associated Macrophage Activation Syndrome Through Treatment With Anakinra and Corticosteroids

(2012) Normi Bruck et al.   JCR-JOURNAL OF CLINICAL RHEUMATOLOGY

Gene expression profiling of peripheral blood mononuclear cells from children with active hemophagocytic lymphohistiocytosis

(2011) J. Sumegi et al.   BLOOD

Disruption of MyD88 signaling suppresses hemophagocytic lymphohistiocytosis in mice

(2011) P. Krebs et al.   BLOOD

Perforin is a critical physiologic regulator of T-cell activation

(2011) J. E. Lykens et al.   BLOOD

Hypomorphic mutations in PRF1, MUNC13-4, and STXBP2 are associated with adult-onset familial HLH

(2011) K. Zhang et al.   BLOOD

Efficacy and safety of repeat courses of rituximab treatment in patients with severe refractory juvenile idiopathic arthritis

(2011) Ekaterina I. Alexeeva et al.   CLINICAL RHEUMATOLOGY

Repeated TLR9 stimulation results in macrophage activation syndrome–like disease in mice

(2011) Edward M. Behrens et al.   JOURNAL OF CLINICAL INVESTIGATION

Hemophagocytosis causes a consumptive anemia of inflammation

(2011) Erin E. Zoller et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Association of IRF5 Polymorphisms with Susceptibility to Macrophage Activation Syndrome in Patients with Juvenile Idiopathic Arthritis

(2011) M. YANAGIMACHI et al.   JOURNAL OF RHEUMATOLOGY

An International Consensus Survey of Diagnostic Criteria for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis

(2011) S. DAVI et al.   JOURNAL OF RHEUMATOLOGY

Combination Therapy of Abatacept and Anakinra in Children with Refractory Systemic Juvenile Idiopathic Arthritis: A Retrospective Case Series

(2011) J. L. RECORD et al.   JOURNAL OF RHEUMATOLOGY

Adult-onset Still’s disease with macrophage activation syndrome successfully treated with a combination of methotrexate and etanercept

(2011) Keisuke Maeshima et al.   Modern Rheumatology

IL1-receptor antagonist anakinra provides long-lasting efficacy in the treatment of refractory adult-onset Still's disease

(2010) L Naumann et al.   ANNALS OF THE RHEUMATIC DISEASES

Anakinra as first-line disease-modifying therapy in systemic juvenile idiopathic arthritis: Report of forty-six patients from an international multicenter series

(2010) Peter A. Nigrovic et al.   ARTHRITIS AND RHEUMATISM

Munc13-4 is a limiting factor in the pathway required for platelet granule release and hemostasis

(2010) Q. Ren et al.   BLOOD

XIAP deficiency: a unique primary immunodeficiency best classified as X-linked familial hemophagocytic lymphohistiocytosis and not as X-linked lymphoproliferative disease

(2010) R. A. Marsh et al.   BLOOD

Nucleic acid sensing Toll-like receptors in autoimmunity

(2010) Sarah E Ewald et al.   CURRENT OPINION IN IMMUNOLOGY

Exacerbation of adult-onset Still’s disease, possibly related to elevation of serum tumor necrosis factor-alpha after etanercept administration

(2010) Kaichi KANEKO et al.   International Journal of Rheumatic Diseases

Visceral leishmaniasis and macrophagic activation syndrome in a patient with rheumatoid arthritis under treatment with adalimumab

(2010) Anna Moltó et al.   JOINT BONE SPINE

Macrophage Activation Syndrome in the Acute Phase of Kawasaki Disease

(2010) Giuseppe A. Latino et al.   JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY

Macrophage Activation Syndrome Treated with Anakinra

(2010) M. DURAND et al.   JOURNAL OF RHEUMATOLOGY

Benefit and a possible risk of tocilizumab therapy for adult-onset Still’s disease accompanied by macrophage-activation syndrome

(2010) Masafumi Kobayashi et al.   Modern Rheumatology

Macrophage activation syndrome/hemophagocytic lymphohistiocytosis and Kawasaki disease

(2010) Gabriele Simonini et al.   PEDIATRIC BLOOD & CANCER

Chronic Murine Typhoid Fever Is a Natural Model of Secondary Hemophagocytic Lymphohistiocytosis

(2010) Diane E. Brown et al.   PLoS One

Successful treatment of severe paediatric rheumatic disease-associated macrophage activation syndrome with interleukin-1 inhibition following conventional immunosuppressive therapy: case series with 12 patients

(2010) P. M. Miettunen et al.   RHEUMATOLOGY

Successful use of infliximab in macrophage activation syndrome with severe CNS involvement

(2010) A Sellmer et al.   SCANDINAVIAN JOURNAL OF RHEUMATOLOGY

A 21-year-old man with Still's disease with fever, rash, and pancytopenia

(2010) Bijal M. Mehta et al.   ARTHRITIS CARE & RESEARCH

Macrophage activation syndrome revealing familial Mediterranean fever

(2010) L. Rossi-Semerano et al.   ARTHRITIS CARE & RESEARCH

Familial Hemophagocytic Lymphohistiocytosis Type 5 (FHL-5) Is Caused by Mutations in Munc18-2 and Impaired Binding to Syntaxin 11

(2009) Udo zur Stadt et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Macrophage activation syndrome in juvenile systemic lupus erythematosus: A multinational multicenter study of thirty-eight patients

(2009) Alessandro Parodi et al.   ARTHRITIS AND RHEUMATISM

Macrophage activation syndrome: Serological markers and treatment with anti-thymocyte globulin

(2009) Andreea Coca et al.   CLINICAL IMMUNOLOGY

Neutralization of IFNγ defeats haemophagocytosis in LCMV-infected perforin- and Rab27a-deficient mice

(2009) Jana Pachlopnik Schmid et al.   EMBO Molecular Medicine

Anakinra for Systemic Juvenile Arthritis

(2009) Andrew Zeft et al.   JCR-JOURNAL OF CLINICAL RHEUMATOLOGY

The Development and Function of Regulatory B Cells Expressing IL-10 (B10 Cells) Requires Antigen Receptor Diversity and TLR Signals

(2009) K. Yanaba et al.   JOURNAL OF IMMUNOLOGY

Acute hepatitis in three patients with systemic juvenile idiopathic arthritis taking interleukin-1 receptor antagonist

(2009) Scott Canna et al.   Pediatric Rheumatology

Mutations in the perforin gene can be linked to macrophage activation syndrome in patients with systemic onset juvenile idiopathic arthritis

(2009) Sebastiaan J. Vastert et al.   RHEUMATOLOGY

A rare trigger for macrophage activation syndrome

(2009) Shikhar Agarwal et al.   RHEUMATOLOGY INTERNATIONAL

A 36-year-old woman with recurrent high-grade fevers, hypotension, and hypertriglyceridemia

(2009) Richard C. Chou et al.   ARTHRITIS CARE & RESEARCH

Hemophagocytic lymphohistiocytosis (HLH) and related disorders

(2009) A. H. Filipovich   Hematology-American Society of Hematology Education Program

Mutations of the hemophagocytic lymphohistiocytosis–associated geneUNC13D in a patient with systemic juvenile idiopathic arthritis

(2008) Melissa M. Hazen et al.   ARTHRITIS AND RHEUMATISM

Macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis is associated with MUNC13-4 polymorphisms

(2008) Kejian Zhang et al.   ARTHRITIS AND RHEUMATISM

Macrophage activation syndrome in rheumatic disease: What is the role of the antigen presenting cell?

(2008) Edward M. Behrens   AUTOIMMUNITY REVIEWS

A Griscelli syndrome type 2 murine model of hemophagocytic lymphohistiocytosis (HLH)

(2008) Jana Pachlopnik Schmid et al.   EUROPEAN JOURNAL OF IMMUNOLOGY

A review of the current use of rituximab in autoimmune diseases

(2008) Hakan M. Gürcan et al.   INTERNATIONAL IMMUNOPHARMACOLOGY

Efficacy of Anakinra in a Case of Refractory Still Disease

(2008) Severine Debiais et al.   JCR-JOURNAL OF CLINICAL RHEUMATOLOGY

Efficacy of Infliximab in Cogan's Syndrome

(2008) RAWAN GHADBAN et al.   JOURNAL OF RHEUMATOLOGY

Efficacy and safety of tocilizumab in patients with systemic-onset juvenile idiopathic arthritis: a randomised, double-blind, placebo-controlled, withdrawal phase III trial

(2008) Shumpei Yokota et al.   LANCET

The role of the initial bone marrow aspirate in the diagnosis of hemophagocytic lymphohistiocytosis

(2008) Abha Gupta et al.   PEDIATRIC BLOOD & CANCER

Mice lacking asparaginyl endopeptidase develop disorders resembling hemophagocytic syndrome

(2008) C.-B. Chan et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Macrophage activation syndrome in 13 children with systemic-onset juvenile idiopathic arthritis

(2008) Hua-Song Zeng et al.   World Journal of Pediatrics

A case of macrophage activation syndrome successfully treated with anakinra

(2008) Alison Kelly et al.   Nature clinical practice. Rheumatology