|
| 2nd line treatment | Response rate (patients number: ) | Organ-specific
response rate | Main side effects | References |
|
| Mycophenolate mofetil | 46–75%
–26 | Skin 53% ()
Mouth 67% ()
Liver 54% () | Gastrointestinal disturbances (diarrhea, nausea, and vomiting) | [72–74] |
|
| High-dose cortico-steroids | 48% major responses | Not Reported | Infections, diabetes decompensation, and psychological effects (psychosis and insomnia) | [75] |
|
| Extracorporeal photopheresis | 61%
| Skin 40% ()
Sclerotic skin 67% ()
Mouth 77% ()
Eye 64% ()
Lung 54% () | Anemia, requirement for central IV access | [69, 70] |
|
| Pentostatin | 53% major responses +2% minor responses | Lichenoid skin 69% ()
Sclerotic skin, fascial, and mouth 52%–57% () | Nausea, vomiting, infection, renal dysfunction, rash, and headache | [71] |
|
| Tacrolimus | 46%
| Not Reported | Renal dysfunction, thrombotic microangiopathy, neurotoxicity, and hypertension | [77] |
|
| Rituximab | Meta-analysis of 7 studies: OR 66% (CI: 57–74%) | Skin 60% ()
Oral mucosa 36% ()
Liver 29% ()
Lung 30% () | Infusion reactions, infections, and hepatitis reactivation | [58–68] |
|
| Sirolimus | 63–94%
–35 | Skin 65% ()
Mouth 75% ()
Liver 33% ()
Eye 64% ()
GI 67% () | Hypertriglyceridemia, renal insufficiency, cytopenias, infections | [77, 78] |
|
