From the Guidelines
Early intravenous immunoglobulin (IVIG) is conditionally recommended as a treatment option for people with myositis-associated interstitial lung disease (ILD) progression despite first ILD treatment. The use of IVIG in this context is supported by observational studies and expert opinion, as noted in the 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) guideline for the treatment of interstitial lung disease in people with systemic autoimmune rheumatic diseases 1. Key points to consider when using IVIG for myositis-associated ILD include:
- IVIG may be useful when rapid onset of action is desired, such as in the presence of severe respiratory muscle weakness.
- The decision to use IVIG should be made on a case-by-case basis, taking into account the individual patient's disease severity, response to initial treatment, and potential risks and benefits.
- IVIG should be used as part of a comprehensive treatment plan that includes conventional immunosuppressive therapy and other supportive measures as needed.
- Patients should be monitored closely for potential adverse effects of IVIG, including headache, thrombotic events, and renal dysfunction. The conditional recommendation for IVIG in myositis-associated ILD reflects the limited but growing evidence base for this treatment, as well as its potential benefits in selected cases 1.
From the Research
Early IVIG in Myositis ILD
- The role of early intravenous immunoglobulin (IVIG) in the treatment of myositis-associated interstitial lung disease (ILD) is being explored as a potential therapeutic option.
- Studies have shown that IVIG can be effective in treating refractory myositis and ILD associated with polymyositis/dermatomyositis (PM/DM) 2, 3, 4.
- A study published in 2009 found that IVIG treatment was safe and could be an effective salvage therapy for refractory ILD-PM/DM in certain cases 3.
- Another study published in 2009 found that IVIG as an add-on treatment with mycophenolate mofetil (MMF) was effective in severe and refractory myositis, and was a safe and steroid-sparing agent 5.
- A recent study protocol published in 2023 proposes a phase-2 double-blind placebo-controlled randomized trial to investigate the efficacy of early add-on IVIg in patients with newly diagnosed myositis, compared to prednisone monotherapy 6.
Mechanism and Efficacy
- The exact mechanism of IVIG in treating myositis and ILD is not fully understood, but it is thought to involve modulation of the immune system and reduction of inflammation.
- Studies have shown that IVIG can improve symptoms and muscle strength in refractory myositis patients, and can also improve outcomes in treatment-naive patients 2, 4, 5.
- The efficacy of IVIG in treating ILD associated with PM/DM is still being investigated, but preliminary results suggest that it may be a useful therapeutic option in certain cases 3.