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

…, C Bracaglia, K Hayward, C Wouters… - … : Official Journal of …, 2009 - Wiley Online Library
Objective To describe the clinical and laboratory features of macrophage activation syndrome
as a complication of juvenile systemic lupus erythematosus (SLE). Methods Cases of …

Targeting interferon-γ in hyperinflammation: opportunities and challenges

F De Benedetti, G Prencipe, C Bracaglia… - Nature Reviews …, 2021 - nature.com
… This leads to recruitment of signal transducer and activator of transcription 1 (STAT1),
which binds to IFNGR1 and is phosphorylated in the C terminus by JAK1 and JAK2. …

[HTML][HTML] Macrophage activation syndrome: different mechanisms leading to a one clinical syndrome

C Bracaglia, G Prencipe, F De Benedetti - Pediatric Rheumatology, 2017 - Springer
… In these mice, administration of Toll-like receptor (TLR) ligands, including lipopolysaccharides
(LPS) and poly(I:C), induces the development of MAS by mimicking an acute infection on …

EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis …

…, E Unsal, S Oliveira, C Bracaglia… - Annals of the …, 2010 - ard.bmj.com
… HSP, 150 c-PAN, 60 c-WG, 87 c-TA and 59 c-other. Prevalence, signs/symptoms,
laboratory, biopsy and imaging reports were consistent with the clinical picture of the four c-vasculitides…

Elevated circulating levels of interferon-γ and interferon-γ-induced chemokines characterise patients with macrophage activation syndrome complicating systemic …

C Bracaglia, K de Graaf, DP Marafon… - Annals of the …, 2017 - ard.bmj.com
Objectives Interferon-γ (IFNγ) is the pivotal mediator in murine models of primary haemophagocytic
lymphohistiocytosis (pHLH). Given the similarities between primary and secondary …

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

…, G Prencipe, I Caiello, C Bracaglia… - Arthritis & …, 2015 - Wiley Online Library
… B and C, Expression of perforin and granzyme B (B) and production of IFNg (C),
determined as the MFI and percentage of IFNg1DX51CD3 2 NK cells. In A (right), B, and …

Prednisone versus prednisone plus ciclosporin versus prednisone plus methotrexate in new-onset juvenile dermatomyositis: a randomised trial

…, F Corona, F Dressler, V Gerloni, MT Apaz, C Bracaglia… - The Lancet, 2016 - thelancet.com
Background Most data for treatment of dermatomyositis and juvenile dermatomyositis are
from anecdotal, non-randomised case series. We aimed to compare, in a randomised trial, the …

Safety and efficacy of infliximab and adalimumab for refractory uveitis in juvenile idiopathic arthritis: 1-year followup data from the Italian Registry

…, I Pontikaki, MP Paroli, C Bracaglia… - The Journal of …, 2013 - jrheum.org
… Laboratory monitoring included white blood cell (WBC) count, hemoglobin, platelet count,
erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), alanine transaminase (ALT), …

A novel disorder involving dyshematopoiesis, inflammation, and HLH due to aberrant CDC42 function

…, K Ramme, V Rosti, C Bracaglia… - Journal of Experimental …, 2019 - rupress.org
… R186C amino acid substitution occurs in a unique region at the C-terminus, within the …
impact of amino acid substitutions in regions far from the C-terminal tail on RhoGDI binding, which …

[HTML][HTML] Autoantibody-mediated impairment of DNASE1L3 activity in sporadic systemic lupus erythematosus

…, A Tsirigos, I Caiello, C Bracaglia… - Journal of Experimental …, 2021 - rupress.org
… was subjected to an initial denaturation of 8 min at 95C followed by 40 amplification cycles
of denaturation for 30s at 95C, annealing for 40s at 55C, and extension for 1 min at 72C. …