Assessment of Macrophage Activation syndromE in STill's Disease (AMETHYST)

Sobi

Status: Completed

Conditions: Still's Disease, Adult-Onset; Macrophage Activation Syndrome; Stills Disease, Juvenile-Onset

Treatments: Other: Chart review

Study type: Observational

Funder types: Industry

Identifiers

NCT06405152

Sobi.Emapalumab-105

Details and patient eligibility

About

Assessment of Macrophage activation syndrome in STill's disease: retrospective chart analysis of patient History, Symptom resolution and Treatment characteristics

Full description

This is an observational, retrospective cohort study on the treatment utilization and outcomes of Macrophage Activation Syndrome (MAS) refractory to glucocorticoids (GC) in patients with Still's disease. The study will be conducted entirely through medical chart abstraction; all data will be taken from the patient's medical record, with no additional assessments.

Enrollment: 75 patients

Sex: All

Ages: 6 months to 80 years old

Volunteers: No Healthy Volunteers

Inclusion criteria

• Age >6 months and ≤80 years at the beginning of the index MAS episode.
• Diagnosis of Still's disease (sJIA (systemic Juvenile Idiopathic Arthritis) or AOSD (Adult Onset Stills Disease) diagnosis).
• Diagnosis of MAS according to treating physician in the medical record.
• Patients who have received at least 3 consecutive days of GC after diagnosis of MAS and/or are judged by the Investigator to be refractory to GC due to clinical worsening of patient's condition .
• The onset of the index MAS episode occurred between 01 January 2012 and 30 September 2022.

Exclusion criteria

• A diagnosis of primary Hemophagocytic Lymphohistiocytosis (HLH) prior to the beginning of the index MAS episode.
• Confirmed malignancy prior to the beginning of the index MAS episode.
• Patient treated with any investigational product as a part of clinical trial during the index MAS episode.