Assessment of Macrophage Activation syndromE in STill's Disease

Swedish Orphan Biovitrum75 enrolled

Overview

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

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.

Study Type

OBSERVATIONAL

Enrollment

Conditions

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

Interventions

Chart reviewOTHER

Retrospective Chart Review

Eligibility

Sex: ALLMin age: 6 MonthsMax age: 80 Years

Medical Language ↔ Plain English

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.

Locations (11)

Swedish Orphan Biovitrum Research Site

Los Angeles, California, United States

Swedish Orphan Biovitrum Research Site

Atlanta, Georgia, United States

Swedish Orphan Biovitrum Research Site

Cincinnati, Ohio, United States

Outcomes

Primary Outcomes

Overall survival

The proportion of patients alive after 1-year of follow-up

Time frame: 1 year

Time to laboratory value normalization

Time to normalization for the key laboratory values

Time frame: From Index date up to 26 weeks

Time to Macrophage Activation Syndrome (MAS) laboratory remission

Time to normalization for all key laboratory values

Time frame: From Index date up to 26 weeks

Time to partial MAS laboratory remission

Time to normalization for at least 3 key laboratory values

Time frame: From Index date up to 26 weeks

Time to tapering of Glucocorticoids (GCs)

From index date to the last of 7 consecutive days receiving ≤1 mg/kg/day of prednisone (PDN).

Time frame: from index date to the last of 7 consecutive days

Number of recurrent MAS episodes

Occurring any time after the end of the data collection period for the index MAS episode

Time frame: through study completion

Administration of organ support care

Proportion of patients receiving hemodialysis, assisted ventilation, cardiac support, or inotropic drugs

Time frame: from the index date until either the end of hospitalization or 26 weeks, whichever occurs later

Characteristics of MAS treatment

description of treatment patterns of MAS-related treatments for both index and recurrent MAS episodes.

Data from ClinicalTrials.gov

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Assessment of Macrophage Activation syndromE in STill's Disease

Overview

Conditions

Interventions

Eligibility

Locations (11)

Outcomes

Primary Outcomes

Secondary Outcomes