Table 2.
Treatment | Mechanism | Route and dosing | ILD outcomes (number of RA-ILD patients) |
Treats joints |
Side effects |
---|---|---|---|---|---|
Mycophenolate mofetil | Decreases synthesis of guanine nucleotides and reduces T and B lymphocyte proliferation and antibody formation | Oral: 1.0–1.5 g twice daily | Single-center cohort: FVC and DLCO stability (n = 18)60 Single-center cohort: FVC and DLCO stability (n = 43)61 Multi-center cohort: FVC and DLCO improvement (n = 77)16 |
No62 | GI upset, cytopenia, recurrent infections, elevated liver enzymes |
Azathioprine | Pro-drug of 6-mercaptopurine; inhibits purine synthesis | Oral 1.5–2 mg/kg IBW once daily, not to exceed 200 mg/day | Single-center cohort: FVC and DLCO improvement (n = 54)61 Multi-center cohort: FVC and DLCO improvement (n = 92)16 |
Yes63 | GI upset, elevated liver enzymes, cytopenia, recurrent infections |
Rituximab | Anti-CD20 antibody | IV 1 g once every 2 weeks for 2 doses, repeat every 24 weeks | Multi-center cohort: FVC and DLCO improvement (n = 43)16 RCT: progression-free survival benefit (n = 3)67 |
Yes94 | GI upset, cytopenia, recurrent infections |
Abatacept | T-lymphocyte costimulatory inhibitor | SQ: 125 mg once weekly or IV: 500 mg to 1 g every 4 weeks | Multi-center cohort: FVC and DLCO stability, HRCT stability (n = 263)73 | Yes68,73 | Recurrent or serious infections, cutaneous infusion reaction |
Nintedanib | Tyrosine kinase inhibitor | Oral: 150 mg twice daily | RCT: slow FVC decline (n = 89)80,81 | No | Diarrhea, nausea, vomiting, decreased appetite, elevated liver enzymes, weight loss |
Pirfenidone | Suppresses TGF-β1, modulates fibrogenic growth factors, and downregulates inflammatory pathways100 | Oral: 801 mg 3 times daily | RCT: slow FVC decline (n = 231)15 | No | Nausea, headache, anorexia |
Abbreviations; DLCO, diffusing capacity for carbon monoxide; FVC, forced vital capacity; ILD, interstitial lung disease; IV, intravenous; RA, rheumatoid arthritis; RCT, randomized controlled trial; SQ, subcutaneous; IBW, ideal body weight.