From the Guidelines
Fibrotic NSIP should be treated with corticosteroids as the first line of therapy, with the majority of patients showing improvement after treatment, as evidenced by the American Thoracic Society statement 1. The clinical presentation of fibrotic NSIP is similar to IPF, with cough and dyspnea present for months to years, and chest radiographic findings showing primarily lower zone reticular opacities.
- Key features of fibrotic NSIP include:
- Homogeneous appearance of either inflammation or fibrosis on histology
- Temporally uniform changes, but the process may be patchy with intervening areas of unaffected lung
- Rare honeycomb areas
- Treatment with corticosteroids, such as prednisone at 0.5-1 mg/kg/day, is typically effective, with most patients showing improvement after treatment 1.
- For patients who do not respond adequately to steroids, immunosuppressive agents like mycophenolate mofetil or azathioprine may be added, and antifibrotic medications such as nintedanib or pirfenidone can be considered in progressive disease.
- Supportive care, including supplemental oxygen, pulmonary rehabilitation, vaccination against respiratory pathogens, and management of gastroesophageal reflux disease, is essential for improving quality of life and reducing morbidity and mortality.
- Regular monitoring with pulmonary function tests, chest imaging, and clinical assessment every 3-6 months is recommended to evaluate treatment response and adjust therapy as needed, with a focus on reducing the estimated 15-20% mortality in 5 years 1.
From the Research
Fibrotic NSIP Treatment Options
- The treatment of fibrotic NSIP may involve the use of corticosteroids, immunosuppressants, and antifibrotic drugs 2, 3, 4.
- A study published in 2002 found that the addition of cyclophosphamide to corticosteroids may be a useful treatment for patients with NSIP 2.
- Another study published in 2019 found that antifibrotic drugs such as pirfenidone and nintedanib may be a valuable treatment option for patients with progressive fibrosing non-IPF ILD, including those with fibrotic NSIP 3.
Prognostic Factors
- A study published in 2022 found that the factors associated with disease progression and relapse in fibrotic NSIP include diffusing capacity for carbon monoxide (DLco) <60%, bronchoalveolar lavage (BAL) lymphocyte >15%, and treatment with corticosteroid and azathioprine 5.
- The same study found that disease progression or relapse was associated with mortality, and that preserved lung function, BAL lymphocytosis, and treatment with corticosteroids and azathioprine were associated with lower risks of disease progression and relapse 5.
Management of Fibrotic NSIP
- A review published in 2016 found that the management of patients with fibrotic, progressive, and immunosuppressive treatment-refractory NSIP remains uncertain, and that further studies are needed to address the role of antifibrotic drugs in this setting 4.
- The same review found that oxygen therapy, pulmonary rehabilitation, and lung transplantation are important in the current management of severe, progressive, and refractory NSIP patients 4.
- A study published in 2016 found that azathioprine may be a useful treatment option for patients with fibrotic connective tissue disease-associated interstitial lung disease, including those with CTD-associated usual interstitial pneumonia (UIP) 6.