Macrophage activation syndrome in systemic lupus erythematosus and Still’s disease: distinct phenotypes shaped by the underlying diseases
{"OBJECTIVES:":[0],"Macrophage":[1],"activation":[2],"syndrome":[3],"(MAS)":[4],"is":[5],"a":[6,47,150],"severe":[7],"complication":[8],"of":[9,26,44,49,80,145,153],"Still's":[10],"disease":[11],"(SD-MAS),":[12],"but":[13,219],"may":[14,243],"also,":[15],"albeit":[16],"rarely,":[17],"complicate":[18],"systemic":[19],"lupus":[20],"erythematosus":[21],"(SLE-MAS).":[22],"The":[23,91],"clinical":[24],"profile":[25],"SLE-MAS":[27,50,92,131,168,194,209,241],"compared":[28,96,196],"with":[29,46,97,111,149,169,213],"SD-MAS":[30,52,109,212],"remains":[31],"unclear,":[32],"as":[33],"do":[34],"the":[35,78,81,112,193,227],"optimal":[36],"treatment":[37],"strategies.":[38],"METHODS:":[39],"A":[40],"retrospective":[41],"multicenter":[42],"cohort":[43],"adults":[45],"history":[48],"or":[51],"was":[53,70,94,164],"gathered":[54],"from":[55,77,211,253],"nine":[56],"tertiary":[57],"referral":[58],"centers.":[59],"MAS":[60,175],"clinical/laboratory":[61],"features,":[62],"treatments":[63],"and":[64,123,187,204,216,224,246],"outcomes":[65],"were":[66],"compared;":[67],"\\"inaugural":[68],"MAS\\"":[69],"defined":[71],"if":[72],"occurring":[73],"within":[74],"6":[75],"months":[76],"onset":[79],"underlying":[82],"disease.":[83],"exploratory":[84],"multivariable":[85],"profiling":[86],"assessed":[87],"for":[88],"disease-specific":[89],"features.":[90],"group":[93],"also":[95],"large":[98],"historical":[99,198],"SLE":[100,199],"cohorts":[101,200],"to":[102,197,231],"identify":[103],"candidate":[104],"risk":[105],"signals.":[106],"RESULTS:":[107],"While":[108],"aligned":[110],"prototypical":[113],"hyperinflammatory":[114],"pattern":[115],"(more":[116],"marked":[117],"hyperferritinemia,":[118],"very":[119],"high":[120],"CRP,":[121],"organomegaly":[122,215],"liver":[124,141,217],"injury;":[125],"100%":[126],"meeting":[127,133],"2016":[128],"ACR/EULAR/PRINTO":[129],"criteria),":[130],"(92%":[132],"criteria)":[134],"more":[135,220],"often":[136],"featured":[137],"CNS":[138,181,222],"dysfunction,":[139],"less":[140,183,214],"injury,":[142],"greater":[143],"degree":[144],"cytopenias":[146],"including":[147],"anemia,":[148],"strong":[151],"signal":[152],"autoimmune":[154,179],"hemolysis":[155],"(73%":[156],"direct":[157],"Coombs":[158],"[DCT]":[159],"positivity).":[160],"Therapeutically,":[161],"IL-1":[162],"antagonism":[163],"used":[165],"in":[166],"7":[167],"mixed":[170],"results":[171],"without":[172],"any":[173],"rapid":[174],"resolution.":[176],"Mucocutaneous":[177],"disease,":[178],"hemolysis,":[180],"involvement,":[182,226],"pronounced":[184,221],"female":[185],"preponderance":[186],"much":[188],"higher":[189],"DCT":[190],"positivity":[191],"dominated":[192],"phenotype":[195],"comprising":[201],"1000,":[202],"2228,":[203],"2055":[205],"cases":[206],"each.":[207],"CONCLUSIONS:":[208],"diverged":[210],"injury":[218],"involvement":[223],"hematological":[225],"latter":[228],"strongly":[229],"linked":[230],"red":[232],"blood":[233],"cell":[234],"directed":[235],"autoantibodies.":[236],"These":[237],"findings":[238],"indicate":[239],"that":[240],"pathogenesis":[242],"be":[244],"divergent":[245],"require":[247],"distinct":[248],"therapeutic":[249],"approaches":[250],"beyond":[251],"extrapolation":[252],"SD-MAS.":[254]}
Authors
- Sinisa Savic (ORCID: https://orcid.org/0000-0001-7910-0554)
- Micaela Fredi (ORCID: https://orcid.org/0000-0002-6511-4936)
- Antonio Tonutti (ORCID: https://orcid.org/0009-0000-9534-6853)
- Franco Franceschini (ORCID: https://orcid.org/0000-0003-3678-6124)
- Aamir Aslam (ORCID: https://orcid.org/0000-0001-9116-3988)
- Francesca Crisafulli (ORCID: https://orcid.org/0000-0003-0319-1538)
- Francesco Caso (ORCID: https://orcid.org/0000-0002-8928-2520)
- Ala Altaie (ORCID: https://orcid.org/0000-0002-9203-1490)
- Addolorata Corrado (ORCID: https://orcid.org/0000-0001-6823-1970)
- Francesca Trunfio
- Maria Gerosa (ORCID: https://orcid.org/0000-0001-5241-5847)
- Maria De Santis (ORCID: https://orcid.org/0000-0002-3196-1336)
- Tom Macleod (ORCID: https://orcid.org/0000-0002-7903-2990)
- Vishal Kakkar (ORCID: https://orcid.org/0000-0003-0513-8677)
- Letizia Pia Di Corcia
- Kerem Abacar
- Edward Vital
- Md Yuzaiful Md Yusof
- Antonio Vitale
- Dina Youssef
- Andrew Barr
- Roberto Giacomelli
- Dennis McGonagle
- Piero Ruscitti
- Micol Frassi
- Ilaria Cavazzana
- Carlo Selmi
Institutions
- University of Siena (IT)
- University of Foggia (IT)
- University of Leeds (GB)
- Humanitas University (IT)
- Università Campus Bio-Medico (IT)
- University of Milan (IT)
- University of L'Aquila (IT)
- Leeds Teaching Hospitals NHS Trust (GB)
- Azienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia (IT)
- Istituto Ortopedico Gaetano Pini (IT)
- NIHR Leeds Musculoskeletal Biomedical Research Unit (GB)
- Federico II University Hospital (IT)
- Azienda Ospedaliera Universitaria Senese (IT)
- IRCCS Humanitas Research Hospital (IT)
- Bradford Teaching Hospitals NHS Foundation Trust (GB)
- University of Naples Federico II (IT)
- University of Brescia (IT)
Publication Details
- Journal
- Lara D. Veeken
- Published
- 2026-09-11
- DOI
- https://doi.org/10.1093/rheumatology/keag490
- Primary Topic
- Autoimmune and Inflammatory Disorders Research
- Type
- article
- Field-Weighted Citation Impact
- 0.00