Treatment Response in Immune-mediated Myositis Associated Rapidly-progressing Interstitial Lung Disease

Sponsor
Singapore General Hospital
Study ID
NCT07775235
Status
Recruiting
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Conditions

  • Interstitial Lung Disease
  • Myositis

Eligibility Criteria

Sex
ALL
Age
21 Years - N/A
Healthy Volunteers
Not accepted

Interventions

  • Immunosuppressant Regimen — DRUG
    Combination treatment regimen lasting 6 months with A) Steroids: Starting with Intravenous methylprednisolone (500mg once daily for three days) followed by tapering dose of prednisolone B) Rituximab (1000mg given at the start of treatment and 1000mg 2 weeks after the first dose) C) Tacrolimus D) Tofacitinib

Study Details

Idiopathic inflammatory myopathies (IIM) are a group of autoimmune conditions characterized by inflammation of muscles with possible extra-muscular manifestations which can include skin and interstitial lung disease (ILD). IIM-associated ILD carries poor prognosis. Particular subtypes of IIM such as anti-melanoma differentiation-associated protein 5 positive (anti-MDA5+) dermatomyositis with ILD are most commonly associated with rapidly progressive-interstitial lung disease (RP-ILD). RP-ILD is defined as worsening dyspnoea on exertion, hypoxaemia, and presence of newly emerging or expanding ground glass opacities on radiographic or computed topography of chest imaging excluding drug or infectious cause. Particularly, patients with anti-MDA5+ dermatomyositis often have RP-ILD with high mortality of over 60% in the first six months of diagnosis. The mainstay of treatment is immunosuppression though there has been no highly efficacious therapy proven to date. Therefore, the overall goal is to improve patient outcomes in IIM-associated RP-ILD including those with anti-MDA5+ dermatomyositis through the development of better treatment regimens. The objective of this research study is to evaluate the efficacy and safety of a combined immunosuppressive regime in patients with IIM-associated RP-ILD. The investigators hypothesize that the simultaneous inhibition of particular targets in the innate and adaptive immune system will improve efficacy and patient survival. The approach involves a combination of four immunosuppressive medications targeting different pathways implicated in IIM associated ILD. If successful, this study could contribute significantly to improving clinical outcomes for patients with IIM-associated RP-ILD.

Key Dates

First listed
Aug 20, 2026
Start date
Dec 26, 2024
Status verified
Sep 2025
Primary completion
Jul 1, 2029
Completion
Jul 1, 2029

Study Design

Enrollment
80 participants (estimated)

Arms

  • Arm: Treatment group
    Patients with immune-mediated myositis-associated rapidly progressive interstitial lung disease undergoing treatment with combined immunosuppressive treatment

Primary Outcome Measure

All-cause mortality at 6 months from treatment initiation [ Time Frame: 6 months ]

Central Contacts

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