Table 2.
Treatment and outcome of anti-MDA5 Ab+ DM/CADM patients with myocardial involvement.
| Patient | P1 | P2 | P3 | P4 | P5 | P6 | P7 | P8 | P9 | P10 | P11 | P12 |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Complications | ||||||||||||
| Respiratory failure | + | – | + | – | – | + | + | + | + | + | – | – |
| Heart failure | + | + | + | – | – | + | + | – | – | + | – | – |
| CMV infection | – | + | – | + | – | NA | NA | + | – | + | – | + |
| EBV infection | NA | + | + | – | – | NA | NA | – | – | – | – | – |
| PCP | – | – | + | – | – | – | – | – | + | – | – | – |
| Bacterial infection | + | + | + | – | – | – | – | – | + | – | – | + |
| Fungal infection | + | – | – | – | – | – | – | – | + | – | – | – |
| Treatment | ||||||||||||
| GC | + | + | + | + | + | + | + | + | + | + | + | + |
| Pulse therapy | + | + | + | + | + | – | – | – | – | – | – | – |
| Immunosuppressor | TAC CTX |
CTX | – | TAC CTX |
CTX | TAC | TAC MMF |
– | TAC CTX |
– | TAC CTX |
TAC CTX |
| IVIG | + | – | + | + | – | – | + | + | – | + | + | + |
| Other | PE | – | – | – | – | – | – | – | – | – | T | T |
| Antiviral | + | + | – | + | – | – | – | + | – | + | – | + |
| Prophylactic SMZ | + | + | – | + | + | + | + | + | – | + | + | + |
| Therapeutic SMZ | – | – | + | – | – | – | – | – | + | – | – | – |
| Antibiotics | + | + | + | – | – | + | + | + | + | + | – | + |
| Antifungal | + | – | – | – | – | – | – | – | + | – | – | – |
| Short-term outcome | ||||||||||||
| Deceased | + | + | + | – | – | + | + | + | + | + | – | – |
| Cause of death | INF | HF | INF | NA | NA | RF | RF | RF | INF | RF | NA | NA |
+, yes; -, no; NA, not applicable; CMV, cytomegalovirus; EBV, Epstein–Barr virus; PCP, pneumocystis pneumonia; GC, glucocorticoids; IVIG, intravenous immunoglobulin; SMZ, sulfamethoxazole; CTX, cyclophosphamide; TAC, tacrolimus; MMF, mycophenolate mofetil; PE, plasmapheresis; T, tocilizumab; INF, infection; HF, heart failure; RF, respiratory failure.