Adult-Onset Still’s Disease (AOSD)—On the Basis of Own Cases

Biomedicines, Journal Year: 2024, Volume and Issue: 12(9), P. 2067 - 2067

Published: Sept. 10, 2024

Introduction: Adult-onset Still’s disease (AOSD) is a rare chronic autoinflammatory condition characterized by spiking fever, arthritis, rash, hepatosplenomegaly, lymphadenopathy, leucocytosis, and hyperferritinemia. It sometimes accompanied life-threatening complications like macrophage activation syndrome/hemophagocytic lymphohistiocytosis (MAS/HLH). Treatment options for AOSD include glucocorticoids (GCs), immunosuppressive drugs, biological medications, Janus kinase (JAK) inhibitors. The features that differentiate MAS/HLH from are: in MAS/HLH, different type of which persistent, sharp decrease the number leukocytes thrombocytes, further increase level transaminases ferritin, significant symptoms central nervous system (CNS), disseminated intravascular coagulation (DIC) hemophagocytosis bone marrow. This study aimed to evaluate course AOSD, results MAS/HLD. Patients methods: Nine patients, four whom developed were treated at Rheumatology Clinic Central Clinical Hospital Ministry Interior Affairs 1 January 2015 15 March 2020 National Institute Geriatric, Rehabilitation September 2021 2024. Medical history, clinical data, demographic laboratory imaging Hscore, treatment data collected. Results: All patients with MAS an Hscore above 150 recovered. Discussion: requires rapid diagnosis as well methylprednisolone pulses, cyclosporine A, etoposide. When comparing who those did not, possible risk factors identified: presence pregnancy (two cases) aggressive AOSD. useful tool identifying MAS/HLH.

Language: Английский

Cracking the code: high ferritin load with Salmon-Colored skin episodes

Oxford Medical Case Reports, Journal Year: 2024, Volume and Issue: 2024(8)

Published: Aug. 1, 2024

Abstract A 37-year-old previously healthy male presented to the Emergency Department with a two-week history of intermittent fevers, joint pain, sore throat, and diffuse salmon-colored rash. Examination revealed pruritic rash swelling red spots in oropharynx. Initial sepsis management was instituted, but subsequent investigations, including infectious, hematologic, autoimmune workups, were inconclusive. Notably, elevated ferritin levels prompted consideration life-threatening conditions like Hemophagocytic Lymphohistiocytosis, which ultimately ruled out. Adult-onset Still’s Disease (AOSD) emerged as leading diagnosis following exclusion other potential causes. skin biopsy performed non-specific findings corticosteroid treatment led significant improvement. This case illustrates clinical decision-making process diagnosing AOSD highlights utility novel AI technology dermatologic assessments.

Language: Английский