1. Systemic Sclerosis (SSc)
2. RA
3. IIM (includes anti-synthetase syndromes and Immune mediated necrotising myositis).
4. Sjogren's
5. MCTD
Following is a summary of the recommendations:
For initiation of treatment:
1. MMF as first line choice to commence treatment in all categories. However, this may be tempered by extra-pulonary considerations, for example, rituximab may be preferred in RA with active joint disease.
2. Cyclophosphamide still among first line choices, but Rituximab preferred in all 5 categories due to equivalent results and lower incidence of side effects.
3. Steroids, either short term or long term, to be avoided in SSc.
4. Short term steroids acceptable in other categories, but not long term.
5. Tocilizumab, elevated to one of the first line choices in SSc and MCTD with SSc features.
6. In RA-ILD, Methotrexate, Leflunomide, anti-TNFs and Abatacept should not be used for treatment of ILD. There is discretion to use them for arthritis, but some panelists would withdraw them if incident ILD develops.
7. JAK inhibitors can be used as first line in IIM, and particularly useful in MDA-5 (JAKi include Tofacitinib, Baricitinib and Upadacitinib).
8. Similarly, calcineurin inhibitors, with tacrolimus preferred to ciclosporin, is amongst first line choices for IIM-ILD.
9. Nintedanib is now recommended as a first line choice in SSc, but not others.
10. Azathioprine remains among first line choices for all categories, but is down in the pecking order for SSc, behind MMF, Rituximab and Tocilizumab.
For progression of ILD despite treatment:
1. If any of the first line Rx above has not been used, it may now be used, with the exception of Azathioprine.
2. Nintedanib can be used for all categories, on the grounds of PPF.
3. Tocilizumab may be used for progressive RA-ILD.
4. IVIG may be used for progressive IIM-ILD.
5. AHST may be considered at this point for SSc-ILD.
6. Pirfenidone may be considered, but only for progressive RA-ILD.
7. Lung transplant can be considered in all categories.
For rapidly progressive ILD (RP-ILD), as seen, for example in MDA-5:
1. IV methylprednisolone, 1g for 3 days, plus at least 2 others from Rituximab, Cyclophosphamide, IVIg, MMF, JAKi and Tacrolimus
2. For non MDA-5 conditions, it is IV methylprednisolone and at least one, or sometimes two of the others.
3. Again, avoid steroids in SSc-ILD.
4. Rituximab and Cyclophosphamide preferred ahead of MMF and calcineurin inhibitors in RP-ILD.
5. Rituximab preferred ahead of Cyclophosphamide in MDA-5 associated RP-ILD.
6. JAKi useful for slowly progressive MDA-5 but not for RP-ILD.
6. Consider early referral for lung transplant in all eligible cases of RP-ILD.
https://acrjournals.onlinelibrary.wiley.com/doi/epdf/10.1002/art.42861?af=R
