Search results for " hyperferritinemic syndrome"

showing 3 items of 3 documents

Macrophage Activation Syndrome in Patients Affected by Adult-onset Still Disease: Analysis of Survival Rates and Predictive Factors in the Gruppo Ita…

2018

Objective.Macrophage activation syndrome (MAS) is a reactive form of hemophagocytic lymphohistiocytosis, which can complicate adult-onset Still disease (AOSD). We investigated AOSD clinical features at the time of diagnosis, to assess predictors of MAS occurrence. Further, we analyzed the outcomes of patients with AOSD who experience MAS.Methods.Patients with AOSD admitted to any Gruppo Italiano di Ricerca in Reumatologia Clinica e Sperimentale center were retrospectively analyzed for features typical of AOSD, MAS occurrence, and their survival rate.Results.Of 119 patients with AOSD, 17 experienced MAS (12 at admission and 5 during followup). Twelve patients with MAS at first admission diff…

AdultMalemusculoskeletal diseases0301 basic medicineAdult onset still diseasemedicine.medical_specialtyAbdominal painMultivariate analysisSurvivalImmunologyStill DiseaseComorbidityGastroenterology03 medical and health sciences0302 clinical medicineRheumatologyInternal medicinePrevalencemedicineHumansImmunology and AllergySurvival rateRetrospective Studies030203 arthritis & rheumatologyFerritinHemophagocytic lymphohistiocytosisbiologybusiness.industryIncidencefungiMiddle AgedHyperferritinemic syndromemedicine.diseaseSurvival RateFerritin030104 developmental biologyMacrophage activation syndromeMacrophage activation syndromeFerritinsCohortbiology.proteinAdult onset still disease; Ferritin; Hyperferritinemic syndrome; Macrophage activation syndrome; Survival; Rheumatology; Immunology and Allergy; ImmunologyFemalemedicine.symptombusinessStill's Disease Adult-OnsetThe Journal of Rheumatology
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Prognostic factors of macrophage activation syndrome, at the time of diagnosis, in adult patients affected by autoimmune disease: Analysis of 41 case…

2016

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 significan…

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 antagonistsAutoimmunity Reviews
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THU0582 Prognostic Factors of Macrophage Activation Syndrome in Adults: Analysis of 40 Cases in 2 Tertiary Referral Centers

2016

Background Macrophage activation syndrome (MAS) is a rare, life-threatening disease in which early diagnosis and aggressive therapeutic strategy may improve the outcome [1]. Due to its rarity, epidemiologic data are still lacking. Hyperferritinemia is frequently associated with MAS and might modulate the cytokines storm the latter contributing to the development of multi-organ failure [2]. Objectives In the current study, we aimed to investigate clinical data, treatments, and outcome of adult MAS patients secondary to autoimmune rheumatic disease. Methods We retrospectively investigated clinical data, treatments, and outcome of 40 adult MAS patients secondary to autoimmune rheumatic disease…

medicine.medical_specialtyImmunologyCatastrophic antiphospholipid syndromeGastroenterologyGeneral Biochemistry Genetics and Molecular Biologychemistry.chemical_compoundTocilizumabRheumatologyInternal medicinemedicineImmunology and AllergyMacrophage activation syndrome adult onset Still‟s disease hyperferritinemic syndromebusiness.industrySeptic shockProportional hazards modelMortality ratehyperferritinemic syndromeadult onset Still‟s diseasemedicine.diseaseComorbiditySurgeryMethylprednisolonechemistryMacrophage activation syndromeMacrophage activation syndromebusinessmedicine.drugAnnals of the Rheumatic Diseases
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