Correct!
1. Anti-nuclear antibody and Scl-70 antibody testing
The NSIP pattern can be seen idiopathically and can be very difficult to differentiate from “early” UIP. In fact, many patients with the thoracic CT interpretation of “possible” UIP using to the American Thoracic Society (ATS) consensus criteria, particularly when the only CT feature lacking for an interpretation of “definite UIP” at thoracic CT is the absence of honeycombing, will be shown to have UIP on surgical biopsy. However, when the NSIP pattern is present on thoracic CT, systemic disorders associated with this pattern should be sought, most notably connective tissue disorders. Inasmuch as possible, the presence of a connective tissue disorder should be excluded before obtaining a surgical biopsy in patients with interstitial lung disease, as the histopathological pattern of lung involvement in patients with connective tissue disorders rarely influences treatment. Therefore, surgical lung biopsy is premature at this point. A repeat thoracic CT using high-resolution technique (HRCT; which implies the use of narrow section width, prone inspiratory imaging, and post-expiratory imaging) can be useful for the assessment of patients with interstitial lung diseases; in particular, prone imaging often proves valuable to determine if the basal lung abnormalities- typical of many interstitial lung disorders, particularly several of the idiopathic interstitial pneumonias- persists on prone imaging, as opposed to resolving on prone imaging, as is typical of reversible basal lung opacity/atelectasis. However, in this patient, the basal lung findings are quite pronounced and are unlikely to reflect reversible basal lung opacity / atelectasis, so repeat HRCT is not required. Endoscopic ultrasound would not be of value for this patient at this point- no target appropriate for biopsy is present. Thoracic MRA could prove useful to assess for vasculitis but would not add useful information to that already obtained with the thoracic CT.
Both the anti-nuclear antibody and Scl-70 antibody tests were positive. Pulmonary function testing showed a forced vital capacity (FVC) of 67% predicted, forced expiratory volume in 1 second (FEV1) 62% predicted, total lung capacity (TLC) 64% predicted, and diffusing capacity for carbon monoxide (DLCO) of 20% predicted. She was capable of 216 meters during her 6-minute walk test. The patient was presumptively started on pirfenidone.
Which of the following statements is the most appropriate next step for the managementof this patient? (Click on the correct answer to proceed to the fifth of eight pages)