Table 2 Notable differences in ratings between specialties

Which of the following would you likely perform to screen the general scleroderma population for ILD?

 6MWD

1.31 (2.69)

2.83 (2.21)

When deciding whether to treat patients for ILD do you consider

 Potential contribution of reflux?

1.92 (1.71)

3.17 (1.19)

In deciding to initiate treatment for SSc-ILD, how important are other parameters besides HRCT and PFTs?

 6MWD

1.39 (1.94)

2.67 (1.50)

“At initial presentation in patients with SSc, this condition would cause me enough concern about near-term ILD that I would start treatment right away”

 FVC and/or DL_CO < LLN

3.15 (1.52)

2.25 (2.22)

What initial therapy do you use once you have decided to treat SSc-ILD?

 Methotrexate

–3.31 (2.50)

–2.33 (2.46)

What is your typical/target dose for MMF?

 2000 mg daily

3.08 (0.95)

2.25 (2.42)

What is your typical/target dose for rituximab?

 1 g on days 0 and 15

3.69 (1.70)

0.17 (2.82)

 I do not utilize rituximab

–3.31 (2.36)

–2.17 (3.01)

Use of antifibrotic drugs

 I see antifibrotic drugs fitting into the management of SSc-ILD after TCZ*

2.73 (1.56)

1.27 (2.33)

Use of nintedanib [following publication of SENSCIS and INBUILD trial results]

 Based on lack of effective response or improvement with immunosuppressive agents (MMF/CYC/TCZ) as defined by lack of improvement of lung function

1.21 (1.89)

2.09 (2.26)*

2.45 (1.37)

3.00 (0.63)*

 Any patient with CTD with clinically significant or worsening ILD

1.64 (1.91)

1.45 (1.97)*

2.36 (1.69)

2.82 (1.25)*

 Based on lack of effective response or improvement with immunosuppressive agents (MMF/CYC/TCZ) as defined by a combination of no improvement in symptoms; ILD on HRCT; and/or PFTs

2.14 (1.92)

2.55 (2.46)*

3.00 (1.41)

3.55 (0.69)*

 Nintedanib fits into the management of SSc-ILD as add-on therapy to TCZ*

2.64 (1.75)

1.73 (2.69)

Use of TCZ [following publication of focuSSced trial results]*

 Patients with early SSc and ILD with anti-topoisomerase antibodies

2.55 (0.93)

2.36 (1.12)

 Based on active worsening of patient condition as defined by a combination of worsening symptoms; ILD on HRCT; and lung function

2.73 (1.95)

2.00 (2.49)

 Based on inability to continue CYC/MMF/antifibrotics due to adverse effects

2.91 (0.94)

2.27 (2.65)

 Based on inability to continue CYC/MMF/antifibrotics due to lack of achievement of effective dose with CYC/MMF/antifibrotics

2.82 (1.40)

2.09 (2.81)

Based on the following response to SSc-ILD treatment, please indicate your likely course of action:

 With progression/worsening of ILD, I would switch to another agent

3.77 (1.01)

1.42 (3.58)

 With progression/worsening of ILD, I would add another agent

3.46 (2.15)

2.33 (3.11)

What circumstances would prompt you to consider weaning a patient from therapy?

 Lack of efficacy

4.23 (1.17)

2.08 (2.64)

How do you wean patients from therapy?

 Stop therapy quickly (over weeks or “cold turkey”)

–4.08 (1.19)

–2.42 (3.26)

What is success to you?

 6MWD stabilization/improvement

2.46 (2.11)

3.42 (1.00)