Associate Professor of Medicine
Department of Pulmonary and Critical Care Medicine
Director of Outpatient Medical Education
Director of the ILD Clinic
University of Chicago Hospitals and Clinics
Chicago, Illinois

Question: I have a patient with biopsy-proven IPF who has been monitored with a yearly CT scan and has developed a 2 cm solitary pulmonary nodule. It does not appear spiculated. How aggressive should I be in treating this patient?

Answer: Lung cancer in IPF presents a proverbial "rock and a hard place" quandary, and is not uncommon. Le Jeune found that the rate of lung cancer in IPF patients is almost 5 times higher than in the population at large.1

Question: I understand that there is no evidence supporting the use of corticosteroid and cyclophosphamide therapy for patients with confirmed IPF. However, in some cases the diagnosis is not definitive and therefore this therapy might be beneficial for the patient. What are the critical diagnostic findings that should guide the decision on immunosuppressive therapy when IPF is suspected but not confirmed?

Answer: The lack of evidence for efficacy of corticosteroids and additional immunosuppression in IPF suggests that this approach should not be taken in cases of confirmed IPF. The IPFnet is conducting a trial of prednisone/azathioprine/N-acetyl cysteine vs N-acetyl cysteine vs Placebo, which may furnish indirect evidence for prednisone/azathioprine efficacy.1 Your question is about other diseases that present like IPF that might be appropriately treated with immunosuppressive therapy.