The standard approach to treating pulmonary fibrosis involves first determining if the pattern is typical of IPF or if the pattern is not typical. This assessment is primarily based on high resolution CT characteristics in combination with patient characteristics (age greater than 50, cough and shortness of breath, crackles and clubbing on lung exam). Patients whose CT scans were not typical of IPF then often proceeded to surgical lung biopsy.
A New Study in New England Journal of Medicine
A recently published study in the New England Journal of Medicine now challenges this approach. Patients with pulmonary fibrosis were included. 60% of patients had a CT pattern typical of IPF and 40% of patients had a CT pattern that was not typical of IPF. Patients had to have progressive lung disease as measured by worsening pulmonary function tests. These patients were then randomized to either OFEV (nintedanib) or placebo for 52 weeks. Patients who received OFEV had substantially slower progression of their lung disease compared to the placebo group. Importantly, patients were excluded from this study if they were taking azathioprine, mycophenolate, rituximab, cyclophosphamide, or prednisone at doses greater than 20mg per day.
OFEV Treatment in Idiopathic Pulmonary Fibrosis
The results of this study now challenge the standard approach to pulmonary fibrosis. Whereas previously we have tried hard to separate IPF from non-IPF, we may no longer need to do that. OFEV appears to be equally effective in patients with imaging that is not typical for IPF. It may be that many of the patients with imaging that was not diagnostic of IPF actually had IPF. In clinical practice, it is common to have a patient with imaging not diagnostic for IPF who proceeds to lung biopsy and the pathologic diagnosis is IPF.
This new study provides support for a new approach to patients with pulmonary fibrosis. Perhaps the new approach will involve asking the following questions: Does the CT scan look like definite or possible IPF? Or does the CT scan look like another specific pattern (sarcoidosis, hypersensitivity pneumonitis, recurrent aspiration pneumonia)? If the CT scan is consistent with definite or possible IPF then no lung biopsy is needed. Proceed with treating the lung disease with OFEV. In patients with lung disease that does not fit into the possible or definite IPF category, the next question is whether medicines that suppress the immune system may be beneficial.
Combined with the recent study showing OFEV is beneficial in scleroderma associated pulmonary fibrosis, we are now armed with a new approach in many of our patients. I suspect that over the next few years we will learn that OFEV is beneficial in not just scleroderma associated pulmonary fibrosis but also pulmonary fibrosis associated with other connective tissue diseases such as rheumatoid arthritis, dermato/polymyositis, mixed connective tissue disease and even some patients with lupus associated pulmonary fibrosis. We now have reason to consider OFEV in a broad group of patients not just IPF and scleroderma. Patients with fibrotic hypersensitivity pneumonitis and fibrotic NSIP (non-specific interstitial lung disease) are also likely to benefit based on the new data.
Many important questions remain. In patients with autoimmune lung disease, when should we use medicines to suppress the immune system and when should we use antifibrotic therapy with OFEV? Perhaps many of these patients will benefit from both therapies. Stay tuned….