Table 3.
Doses and monitoring of therapeutic agents in the treatment of M-ILD
| Medication | Highest level of evidence | Recommended use | Dose | Recommended monitoring |
|---|---|---|---|---|
| Glucocorticoids | Meta-analysis of mostly retrospective studies [33] | Initial treatment | PO prednisolone 0.5–1 mg/kg/day [34] | Annual densitometry |
| IV methylprednisolone 1 g/day for 3 days [35] | Glucose monitoring | |||
| Azathioprine | Retrospective studies [39, 40] | First line steroid sparing agent for M-ILD | 2 mg/kg/day PO [39] | FBC and LFT every 2 weeks for 4 weeks, then consider monthly once stable |
| Add on treatment for RP-ILD | Sunscreen | |||
| Mycophenolate Mofetil | Retrospective studies [39, 42] | First line steroid sparing agent for M-ILD | Up-titrate to 2 g/day PO | FBC, LFTS’s every 2 weeks, then consider monthly once stable |
| Add on treatment for RP-ILD | Max 3 gms daily [39, 42] | |||
| Rituximab | Phase IIb randomized, double blind, superiority trial [49] | First line for RP-ILD | 1000 mg IV D1 and D14 [49] | CD19/20 levels |
| Add on treatment for M-ILD | Hepatitis and latent TB screen (QuantiFERON® assay) | |||
| Cyclophosphamide | Phase IIb randomized, double blind, trial [49] | First line for RP-ILD | 600 mg/m2 body surface area every 4 weeks IV for six doses [49] | FBC every 2 weeks initially |
| Add on treatment for M-ILD | ||||
| Tacrolimus | Randomized, open-label comparative trial [61] | Additional treatment option | 0.075 mg/kg PO to achieve trough level of 5–20 ng/ml [54, 61] | FBC, renal profile, drug trough level |
| Cyclosporin A | Randomized, open-label comparative trial [61] | Additional treatment option | 4 mg/kg/day PO, aim for peak levels of 1000 ng/ml [60] or trough level of 100–150 ng/mL [61] | FBC, LFT, renal profile, blood pressure monitoring |
| Tofacitinib | Small open label prospective trial [70] | Additional treatment option | 5 mg PO twice daily [70] | Monitor for signs of infections |
| Nintedanib | Sub-analysis of the INBUILD trial [62] | Additional treatment option | 150 mg twice a day [62] | Monitor LFT |
| Pirfenidone | Small open-label trial [63] | Additional treatment option | 1800 mg daily [63] | Monitor LFT |
| Sunscreen | ||||
| IVIG | Retrospective study [73] | Salvage therapy | 400 mg/kg/day IV × 5 consecutive days per month (period of 6 months) [73] | FBC monthly |
CD cluster of differentiation; FBC full blood count; IV intravenous; IVIG intravenous immunoglobulins; LFT Liver function test; M-ILD Myositis associated interstitial lung disease; PO Orally; TB tuberculosis