Outcomes in Idiopathic Inflammatory Myopathies
Predictors of Long-term Outcomes in Idiopathic Inflammatory Myopathies
King’s College Hospital NHS Foundation Trust
Duration of Study in the UK
1 years, 8 months, 3 days
The objective of this study is to identify how auto-antibody profile predicts progression and mortality in a large multi-centre UK cohort of IIM-ILD patients.
The idiopathic inflammatory myopathies (IIMs) are a group of related autoimmune conditions that result in a variety of different clinical phenotypes including inflammation of the muscles, skin, joints and lungs to varying degrees. Interstitial lung disease (ILD) can occur and may be rapidly progressive and immediately life-threatening or slow and indolent.
Introducing immunosuppressive therapies early in the course of the disease before irreversible damage occurs may result in better long-term outcomes. Therefore, identifying which patients are likely to progress is vital. A number of studies have attempted to identify predictors of outcome, however they are often limited by small sample sizes of these rare diseases, or by short follow up times. Conflicting results between studies may also be due to population differences between these single-centre studies.
This retrospective cohort study will use medical records across 4 specialist centres to collect and analyse baseline disease characteristics of patients with IIM, including age at diagnosis, sex, ethnicity, smoking status, pre-existing comorbidity, auto-antibody profile, CK, CRP, ESR, muscle biopsy and radiological findings.
Long-term follow up outcomes will be observed as time to deterioration in lung function or death. Secondary outcomes would include radiological changes, treatment escalation, functional decline, need for ambulatory oxygen, referral for lung transplantation, and cause of death.
The demographic and clinical characteristics of patients will be defined by antibody subtype. A survival method using Cox proportional hazards will be used to compare time to deterioration according to antibody subtype. Analysis of change in lung function over time will be analysed using a mixed effects regression model.
London - Brent Research Ethics Committee
Date of REC Opinion
2 Jun 2021