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RESEARCH PRODUCT
Prognostic factors of macrophage activation syndrome, at the time of diagnosis, in adult patients affected by autoimmune disease: Analysis of 41 cases collected in 2 rheumatologic centers
Francesco CicciaVasiliki LiakouliFrancesco CarubbiGiovanni TrioloPaola Di BenedettoPaola CiprianiRoberto GiacomelliOnorina BerardicurtiMarco ValentiFrancesco MaseduPiero RuscittiGiuliana GugginoSalvatore Di Bartolomeosubject
AdultMalemusculoskeletal diseases0301 basic medicinemedicine.medical_specialtyImmunologyAdult onset Still's disease; Hyperferritinemic syndrome; Macrophage activation syndrome; Adult; Ambulatory Care Facilities; Autoimmune Diseases; Female; Humans; Immunosuppressive Agents; Macrophage Activation Syndrome; Male; Middle Aged; Prognosis; Retrospective Studies; Treatment Outcome; Immunology and Allergy; ImmunologyDiseaseAmbulatory Care FacilitiesAutoimmune Diseases03 medical and health sciences0302 clinical medicineAdult onset Still's diseaseInternal medicineHumansImmunology and AllergyMedicineRetrospective Studies030203 arthritis & rheumatologyAutoimmune diseaseAdult patientsbusiness.industryMortality ratefungiRetrospective cohort studyMiddle AgedPrognosisHyperferritinemic syndromemedicine.diseasebody regionsSettore MED/16 - ReumatologiaTreatment Outcome030104 developmental biologyMacrophage activation syndromeMacrophage activation syndromeCohortImmunologyFemalelipids (amino acids peptides and proteins)businessCytokine stormImmunosuppressive Agentshormones hormone substitutes and hormone antagonistsdescription
Macrophage activation syndrome (MAS) is a rare, life-threatening disease in which early diagnosis and aggressive therapeutic strategy may improve the outcome. Due to its rarity, epidemiologic data are still lacking. Hyperferritinemia is frequently associated with MAS and might modulate the cytokine storm, which is involved in the development of multiple organ failure. In this paper, we investigated clinical data, treatments, and outcome of a homogeneous cohort of 41 adult MAS patients, complicating autoimmune rheumatic diseases. MAS-related death occurred in 17 patients (42.5%) during the follow-up, and older age and increased serum ferritin levels, at the time of diagnosis, were significantly associated with mortality. In conclusion, adult MAS is associated with high mortality rate. Some clinical features at diagnosis may be predictive of MAS-associated death.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2016-01-01 | Autoimmunity Reviews |