Correct!
4. 1 and 3

A thoracic CT scan was performed (Figure 4).

Figure 4. Thoracic CT scans from 2011, January 2018 and April 2018.

The CT scan suggests progression as well as increased ground glass consistent with an exacerbation of his underlying NSIP. In NSIP exacerbations occur in 4-5% of patients per year and are likely to respond to steroid therapy. In contrast, exacerbations occur in 10-15% of IPF patients per year; occur most often in patients with an FVC < 70% predicted; are less likely to respond to corticosteroids; and prognosis is poor with >50% mortality.

Our patient was treated with high dose corticosteroids and clinically improved. He is currently on prednisone 60 mg/day and being evaluated for lung transplantation.

References

1. Visscher DW, Myers JL. Histologic spectrum of idiopathic interstitial pneumonias. Proc Am Thorac Soc. 2006 Jun;3(4):322-9. [CrossRef] [PubMed]
2. Travis WD, Hunninghake G, King TE Jr, et al. Idiopathic nonspecific interstitial pneumonia: report of an American Thoracic Society project. Am J Respir Crit Care Med. 2008 Jun 15;177(12):1338-47. [CrossRef] [PubMed]
3. Park IN, Kim DS, Shim TS, et al. Acute exacerbation of interstitial pneumonia other than idiopathic pulmonary fibrosis. Chest. 2007 Jul;132(1):214-20. [CrossRef] [PubMed]

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