Table 2.
Treatments and Outcomes of All 16 Patients (previously Reported Patients and Our Patients) with Thrombotic Microangiopathies Accompanied with Polymyositis/dermatomyositis.
| Pt | Age/ Sex |
PM/ DM |
Treatment | Outcome | Cause of death | Reference | |||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| GC | PE | CAPD | Antiplatelet | VCR | HD | IVIG | RTX | MTX | |||||||
| Treatment-effective group | |||||||||||||||
| 1 | 57/F | PM | (+) | PSL 100 mg | (+) | (+) | (-) | (-) | (-) | (-) | (-) | (-) | death | electrocyte inbalances and fluid overload | 6 |
| 2 | 42/M | DM | (+) | PSL 200 mg | (-) | (-) | (-) | (+) | (-) | (+) | (-) | (+) | alive | 7 | |
| 3 | 50/F | DM | (+) | N/A | (-) | (-) | (-) | (-) | (-) | (-) | (-) | (-) | alive | 8 | |
| 4 | 70/F | PM | (+) | PSL 40 mg | (+) | (-) | (-) | (-) | (-) | (-) | (-) | (-) | death | miliary tuberculosis | 9 |
| 5 | 69/F | DM | (+) | N/A | (+) | (-) | (-) | (-) | (-) | (-) | (-) | (-) | alive | Present case (Patient1) | |
| 6 | 75/M | DM | (+) | N/A | (+) | (-) | (-) | (-) | (-) | (+) | (+) | (-) | death | cholangitis, sepsis | Present case (Patient2) |
| Treatment-ineffective group | |||||||||||||||
| 7 | 65/F | DM | (+) | mPSL 2 g q4 h | (+) | (-) | (+) | (-) | (-) | (-) | (-) | (-) | death | pneumonia | 10 |
| 8 | 62/F | DM | (+) | N/A | (+) | (-) | (-) | (-) | (-) | (-) | (-) | (-) | death | respiratory fairure | 11 |
| 9 | 73/F | PM | (+) | N/A | (+) | (-) | (-) | (-) | (+) | (-) | (-) | (-) | death | heart failure, respiratory failure | 12 |
| 10 | 59/F | PM | (+) | N/A | (+) | (-) | (+) | (-) | (+) | (-) | (-) | (+) | death | sepsis | 13 |
| 11 | 60/M | DM | (+) | mPSL pulse 1g | (+) | (-) | (+) | (-) | (-) | (+) | (-) | (-) | death | heart failure, pneumonia | 14 |
| 12 | 50/F | PM | (+) | (+) | (-) | (-) | (-) | (+) | (-) | (-) | (-) | death | veno-occlusive disease | 15 | |
| 13 | 53/F | PM | (+) | (-) | (-) | (-) | (-) | (-) | (-) | (-) | (-) | death | acute renal failure | 16 | |
| 14 | 48/F | DM | (+) | mPSL | (-) | (-) | (-) | (-) | (+) | (+) | (-) | (-) | death | ventricular asystole | 17 |
| 15 | 57/M | DM | (+) | mPSL pulse 1g | (-) | (-) | (-) | (-) | (-) | (+) | (-) | (-) | death | diffuse alveolar hemorrhage | 18 |
| 16 | 65/F | DM | (+) | N/A | (+) | (-) | (-) | (-) | (+) | (-) | (-) | (-) | death | sepsis | Present case (Patient3) |
PM: polymyositis, DM: dermatomyositis, TMA: thrombotic microangiopathies, GC: glucocorticoid, PE: plasma exchange, CAPD: continuous ambulatory peritoneal dialysis, VCR: vincristine, HD: hemodialysis, IVIG: intravenous immunoglobulin, RTX: rituximab, MTX methotrexate, (+) positive, (-) negative, N/A: not available