Table 2.
Reported therapies in anti-MDA5 positive DM associated RPILD.
| Therapy | Dose, schedule and route ofadministration |
|---|---|
| Prednisone/prednisolone1 | 0.5–1 mg/kg/day p.o. |
| Pulsed methylprednisolone1 | 500 mg-1 gr/day (×3consecutive days) i.v. |
| Cyclosporine A2 | 2–5 mg/kg/day p.o. or i.v. |
| Tacrolimus3 | 0.06–0.075 mg/kg/day p.o. |
| Cyclophosphamide | 0.5–1 gr/m2/2–4 weeks i.v. |
| Azathioprine4 | 2–3 mg/kg/day p.o. |
| Leflunomide5 | 10–20 mg/day p.o. |
| Methotrexate6 | Up to 25 mg/week p.o. or s.c. |
| Mycophenolate mofetil | 1–3 g/day p.o. |
| Basiliximab | 20 mg/week (×2) i.v. |
| Infliximab | 5 mg/kg i.v. at week 0,2,6 and every 8 weeks |
| Rituximab | 350–375 mg/m2/week (×2–4) i.v. or 1 gr/2 week (×2) i.v. |
| Tofacitinib | 5 mg b.i.d. p.o. |
| Pirfenidone | 267 mg t.i.d. p.o. |
| Immunoglobulin | 0.4 g/kg/5 days i.v. |
| Polymyxin B and plasmapheresis | Hemoperfusion with polymyxin B at a flow rate of 100 ml/h for 3 h/day (×2) and plas mapheresis with 3.51 of 5% seroalbumin replacement followed by intravenous immunoglobulin |
1
Corticosteroids as initial or induction/rescue therapy.
2
To achieve a blood level of 1000 ng/mL during induction therapy, if possible.
3
To achieve a blood level of 10–15 ng/mL during induction therapy, if possible.
4
Depending on thiopurine methyl transferase activity.
5
Dose not reported.
6
Not administered in anti-MDA5 associated RPILD. p.o.: per os. i.v.: intravenous. s.c.: subcutaneous; bid: twice in a day. tid: three in a day.