In non-specific interstitial pneumonia, the distinction between the early inflammatory phase and the late stage characterised by fibrosis is of great importance for the choice of appropriate therapy. It is now well established that the inflammation that dominates the early stage of NSIP can be effectively treated by anti-inflammatory therapy(immunosuppression). The earlier such therapy is given, the better the treatment results. In contrast, anti-inflammatory immunosuppression is largely ineffective in advanced disease with only fibrosis. While intensive immunosuppression with cyclophosphamide was initially the treatment of choice, more recently the less intensive and often better tolerated mycophenolate mofetil has been similarly effective. Studies on several other anti-inflammatory drugs have been started. Exclusive cortisone therapy does not produce good long-term results in this disease and is no longer considered appropriate.
As previously outlined, two new drugs are available, pirfenidone and nintedanib, which act primarily on fibrosis rather than inflammation. In parallel to the nintedanib trial in idiopathic pulmonary fibrosis mentioned above, another large trial was published in May 2019 showing that nintedanib also significantly slows the deterioration of lung disease due to scleroderma. Nintedanib is the first drug for which such an effect in a rheumatic disease is well established. A large trial of pirfenidone in this situation has been started.
