From the Guidelines
Corticosteroids are not recommended as the primary treatment for nonspecific interstitial pneumonia (NSIP) due to the lack of survival benefit and substantial long-term morbidity associated with their use. The typical regimen for corticosteroids in NSIP is oral prednisone starting at 0.5-1 mg/kg/day (usually 40-60 mg daily) for 4-8 weeks, followed by a gradual taper over 3-6 months, as suggested by older studies 1. However, more recent guidelines suggest that corticosteroid monotherapy is associated with substantial long-term morbidity and no survival benefit has been demonstrated in controlled trials 1.
Some patients may still require corticosteroids to alleviate symptoms, and oral corticosteroid therapy up to 10 mg of prednisone per day may be proposed to alleviate incapacitating cough 1. High-dose corticosteroid therapy may also be proposed to treat patients with acute exacerbation of IPF 1.
- Monitor for clinical improvement and adjust the taper accordingly
- Some patients may require long-term low-dose steroids (5-10 mg/day) to maintain remission
- Add steroid-sparing agents like azathioprine or mycophenolate if prolonged treatment is needed
- Regular follow-up with pulmonary function tests and imaging is essential to assess response and guide treatment duration
- Be aware of potential side effects of long-term steroid use and manage accordingly
The use of corticosteroids in NSIP should be carefully considered, weighing the potential benefits against the risks of long-term morbidity and lack of survival benefit, as suggested by the most recent and highest quality study 1.
From the Research
Role of Corticosteroids in Nonspecific Interstitial Pneumonia
- Corticosteroids are effective for nonspecific interstitial pneumonia (NSIP) as reported in many studies 2.
- The addition of cyclophosphamide to corticosteroids might be a useful treatment for patients with NSIP, as demonstrated in a case study where interstitial infiltrates on chest computed tomography improved dramatically after adding intravenous cyclophosphamide followed by oral cyclophosphamide 2.
- Treatment of idiopathic NSIP is generally instituted in the form of immunosuppression, with corticosteroids being a common treatment option, although the evidence supporting its effectiveness is limited 3, 4.
- Corticosteroid treatment has been shown to improve lung function in iNSIP patients, with a significant improvement in forced vital capacity (FVC), forced expiratory volume (FEV1), and diffusing capacity of the lung for carbon monoxide (DLco) 5.
- Factors affecting treatment response to corticosteroids in iNSIP patients include sero-negative anti-nuclear antibody (ANA) and shorter duration of respiratory symptoms at diagnosis, which are associated with a good response to treatment 5.
Treatment Outcomes and Prognosis
- The prognosis for iNSIP is generally more favorable than for idiopathic pulmonary fibrosis (IPF), with many studies reporting a 5-year survival rate above 70% 4.
- Early treatment with corticosteroids and/or immunosuppressants is recommended, even in an early disease stage, as it can improve lung function in iNSIP patients 5.
- Antifibrotic agents should be considered in cases of progressive pulmonary fibrosis (PPF), where pulmonary fibrosis progressively worsens 4.