From the Guidelines
The primary treatment for idiopathic interstitial lung disease (ILD) with non-specific interstitial pneumonia (NSIP) pattern is glucocorticoids, as conditionally recommended by 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. Begin with prednisone 40-60 mg daily for 4-8 weeks, then taper slowly over 3-6 months to the lowest effective dose, often 10-20 mg daily or every other day. Some key points to consider in the treatment of ILD with NSIP pattern include:
- For patients who don't respond adequately to glucocorticoids or to reduce steroid dependence, add immunosuppressive agents such as mycophenolate or azathioprine, as conditionally recommended by the guideline 1.
- Supportive care is crucial, including:
- Supplemental oxygen if needed
- Pulmonary rehabilitation
- Vaccinations (influenza, pneumococcal)
- Treatment of gastroesophageal reflux if present The rationale for this treatment is to suppress the inflammatory and fibrotic processes in the lungs. Glucocorticoids reduce inflammation, while immunosuppressants help modulate the immune response. This approach aims to stabilize or improve lung function and reduce symptoms. Regular follow-up with pulmonary function tests and imaging is essential to assess treatment response and adjust therapy as needed. It is also important to note that the guideline conditionally recommends against certain treatments, such as leflunomide, methotrexate, TNFi, and abatacept, as first-line ILD treatment options 1. Additionally, for people with SARD-ILD progression despite first-line ILD treatment, the guideline conditionally recommends mycophenolate, rituximab, cyclophosphamide, and nintedanib as treatment options, and conditionally recommends against using long-term glucocorticoids 1.
From the Research
Treatment Overview
- The treatment for Idiopathic Interstitial Lung Disease (ILD) with Nonspecific Interstitial Pneumonia (NSIP) is generally instituted in the form of immunosuppression 2.
- Corticosteroids are commonly used as a treatment for NSIP, and the addition of cyclophosphamide to corticosteroids may be a useful treatment for patients with NSIP 3.
- Treatment with corticosteroids and/or immunosuppressants has been shown to improve lung function in iNSIP patients 4.
Factors Affecting Treatment Outcome
- Sero-negative anti-nuclear antibody (ANA) is significantly related to lung function improvement in iNSIP patients 4.
- A shorter duration of respiratory symptoms at diagnosis is significantly associated with a good response to treatment 4.
- Early treatment should be considered in iNSIP patients, even in an early disease stage 4.
Treatment Options
- Systemic steroids, either alone or in combination with other immunosuppressants, are often used to treat iNSIP 5.
- Antifibrotic agents should be considered in cases of progressive pulmonary fibrosis (PPF), where pulmonary fibrosis progressively worsens 5.
- A practical approach to the treatment of idiopathic interstitial pneumonias, including NSIP, involves the use of prognostic markers, clinical trial end points, and an understanding of the pathogenesis of the disease 6.