Pathogenesis of macrophage activation syndrome and potential for cytokine-directed therapies

GS Schulert, AA Grom - Annual review of medicine, 2015 - annualreviews.org
Macrophage activation syndrome (MAS) is an acute episode of overwhelming inflammation
characterized by activation and expansion of T lymphocytes and hemophagocytic …

Pathophysiology and epidemiology of hemophagocytic lymphohistiocytosis

CE Allen, KL McClain - Hematology 2014, the American …, 2015 - ashpublications.org
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by extreme
immune activation, resulting in pathologic inflammation. The diagnosis includes a spectrum …

Inhibition of natural killer cell cytotoxicity by interleukin‐6: implications for the pathogenesis of macrophage activation syndrome

L Cifaldi, G Prencipe, I Caiello… - Arthritis & …, 2015 - Wiley Online Library
Objective Systemic juvenile idiopathic arthritis (JIA) is associated with high levels of
interleukin‐6 (IL‐6) in the serum and synovial fluid, and impairment of natural killer (NK) cell …

Macrophage activation syndrome

A Ravelli, S Davì, F Minoia, A Martini… - Hematology …, 2015 - hemonc.theclinics.com
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Evidence-based diagnosis and treatment of macrophage activation syndrome in systemic juvenile idiopathic arthritis

V Boom, J Anton, P Lahdenne, P Quartier… - Pediatric …, 2015 - Springer
Background Macrophage activation syndrome (MAS) is a severe and potentially lethal
complication of several inflammatory diseases but seems particularly linked to systemic …

From bench to bedside and back again: translational research in autoinflammation

D Holzinger, C Kessel, A Omenetti… - Nature Reviews …, 2015 - nature.com
Translational research approaches brought major changes to the understanding and
treatment options of autoinflammatory diseases. Patients with common complex …

How we manage hyperferritinemic sepsis-related multiple organ dysfunction syndrome/macrophage activation syndrome/secondary hemophagocytic …

JA Carcillo, DW Simon, BS Podd - Pediatric Critical Care …, 2015 - journals.lww.com
Whether it is due to changing epidemiology or increased recognition, hyperferritinemic
sepsis is becoming increasingly diagnosed in critically ill children. An acute rise in ferritin is …

Familial hemophagocytic lymphohistiocytosis

B Degar - Hematology/Oncology Clinics, 2015 - hemonc.theclinics.com
Familial hemophagocytic lymphohistiocytosis (FHL) is a rare, life-threatening, inherited
hyperinflammatory syndrome that may be clinically indistinguishable from secondary forms …

New monogenic autoinflammatory diseases—a clinical overview

SW Canna, R Goldbach-Mansky - Seminars in immunopathology, 2015 - Springer
Translating pathogenic insights gained from monogenic defects that cause autoinflammatory
diseases into novel therapies has dramatically improved the lives of patients with these …

The long tail and rare disease research: the impact of next-generation sequencing for rare Mendelian disorders

T Shen, A Lee, C Shen, CJ Lin - Genetics research, 2015 - cambridge.org
There are an estimated 6000–8000 rare Mendelian diseases that collectively affect 30
million individuals in the United States. The low incidence and prevalence of these diseases …