A HeterozygousRAB27AMutation Associated with Delayed Cytolytic Granule Polarization and Hemophagocytic Lymphohistiocytosis

Interleukin-1 Receptor Blockade Is Associated With Reduced Mortality in Sepsis Patients With Features of Macrophage Activation Syndrome

(2016) Bita Shakoory et al.   CRITICAL CARE MEDICINE

Hemophagocytic lymphohistiocytosis caused by dominant-negative mutations in STXBP2 that inhibit SNARE-mediated membrane fusion

(2015) W. A. Spessott et al.   BLOOD

Clinical features and correct diagnosis of macrophage activation syndrome

(2015) Randy Q Cron et al.   Expert Review of Clinical Immunology

Failed CTL/NK cell killing and cytokine hypersecretion are directly linked through prolonged synapse time

(2015) Misty R. Jenkins et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Inhibition of Natural Killer Cell Cytotoxicity by Interleukin-6: Implications for the Pathogenesis of Macrophage Activation Syndrome

(2015) Loredana Cifaldi et al.   Arthritis & Rheumatology

2013 Update of the 2011 American College of Rheumatology Recommendations for the Treatment of Juvenile Idiopathic Arthritis: Recommendations for the Medical Therapy of Children With Systemic Juvenile Idiopathic Arthritis and Tuberculosis Screening Among C

(2015) Sarah Ringold et al.   ARTHRITIS AND RHEUMATISM

Transcriptional regulation of Munc13-4 expression in cytotoxic lymphocytes is disrupted by an intronic mutation associated with a primary immunodeficiency

(2014) Frank Cichocki et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Genetic Features of Late Onset Primary Hemophagocytic Lymphohistiocytosis in Adolescence or Adulthood

(2014) Yini Wang et al.   PLoS One

IL-6 Amplifies TLR Mediated Cytokine and Chemokine Production: Implications for the Pathogenesis of Rheumatic Inflammatory Diseases

(2014) Ivan Caiello et al.   PLoS One

Genetic Defects in Cytolysis in Macrophage Activation Syndrome

(2014) Mingce Zhang et al.   Current Rheumatology Reports

Development and Validation of the HScore, a Score for the Diagnosis of Reactive Hemophagocytic Syndrome

(2014) Laurence Fardet et al.   Arthritis & Rheumatology

Recurrent Macrophage Activation Syndrome Associated With Heterozygous Perforin W374X Gene Mutation in a Child with Systemic Juvenile Idiopathic Arthritis

(2013) Sule Unal et al.   JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY

Higher-dose Anakinra Is Effective in a Case of Medically Refractory Macrophage Activation Syndrome

(2013) P. J. KAHN et al.   JOURNAL OF RHEUMATOLOGY

A prospective evaluation of degranulation assays in the rapid diagnosis of familial hemophagocytic syndromes

(2012) Y. T. Bryceson et al.   BLOOD

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

(2012) A Ravelli et al.   GENES AND IMMUNITY

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

Familial Hemophagocytic Lymphohistiocytosis May Present during Adulthood: Clinical and Genetic Features of a Small Series

(2012) Elena Sieni et al.   PLoS One

Familial and Acquired Hemophagocytic Lymphohistiocytosis

(2011) G.E. Janka   Annual Review of Medicine

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

(2011) K. Zhang et al.   BLOOD

Hypocomplementemia Associated with Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis and Adult Onset Still's Disease: 3 Cases

(2011) M. GORELIK 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

Screening the PRF1, UNC13D, STX11, SH2D1A, XIAP, and ITK gene mutations in Chinese children with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis

(2011) Huang Zhizhuo et al.   PEDIATRIC BLOOD & CANCER

Review of haemophagocytic lymphohistiocytosis

(2010) H. R. Freeman et al.   ARCHIVES OF DISEASE IN CHILDHOOD

Macrophage Activation Syndrome in Autoimmune Disease

(2010) Sean Deane et al.   INTERNATIONAL ARCHIVES OF ALLERGY AND IMMUNOLOGY

Functional characterization of two RAB27A missense mutations found in Griscelli syndrome type 2

(2010) Norihiko Ohbayashi et al.   Pigment Cell & Melanoma Research

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

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

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

Structural Basis for the Exclusive Specificity of Slac2-a/Melanophilin for the Rab27 GTPases

(2008) Mutsuko Kukimoto-Niino et al.   STRUCTURE

Elucidation of Rab27 Recruitment by Its Effectors: Structure of Rab27a Bound to Exophilin4/Slp2-a

(2008) Leonard M.G. Chavas et al.   STRUCTURE

FOXP3 inhibits HIV-1 infection of CD4 T-cells via inhibition of LTR transcriptional activity

(2008) Nithianandan Selliah et al.   VIROLOGY

A case of macrophage activation syndrome successfully treated with anakinra

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

Efficient In Vivo Targeting of Epidermal Stem Cells by Early Gestational Intraamniotic Injection of Lentiviral Vector Driven by the Keratin 5 Promoter

(2007) Masayuki Endo et al.   MOLECULAR THERAPY