Table II.
Abnormality | Medication | Strength of recommendation |
---|---|---|
Raynaud’s phenomenon | Calcium channel antagonists (dihydropyridine derivatives) such as nifedipine | A |
Phosphodiesterase type 5 inhibitors – sildenafil | A | |
Iloprost (i.v. infusions/p.o.) | A | |
Alprostadil (i.v. infusions) | A | |
Fluoxetine | C | |
Fingertip lesions | Iloprost (i.v. infusions) | A |
Phosphodiesterase type 5 inhibitors – sildenafil, tadalafil | A | |
Endothelin receptor antagonist – bosentan | A | |
Pulmonary hypertension | Endothelin receptor antagonist – bosentan, ambrisentan, macitentan; PDE-5 inhibitors; riociguat | B |
Epoprostenol (i.v. infusions) | A | |
Iloprost, treprostinil | B | |
Skin involvement/internal organ Fibrosis | Methotrexate | A |
Cyclophosphamide | A | |
Mycophenolate mofetil | A | |
Scleroderma renal crisis | ACE inhibitors | C |
Gastrointestinal involvement | Proton pump inhibitors | B |
Prokinetic agents | C | |
Antibiotics – quinolones, amoxicillin + clavulanic acid, metronidazole, doxycycline | D | |
Autologous stem cells, transplantation | A | |
Perspectives Skin involvement and internal organ fibrosis |
B-cell depletion – rituximab (anti-CD20) anti-interleukin-6 – tocilizumab Tyrosine kinase inhibitors (e.g. nintedanib) TGFβ inhibitors (e.g. fresolimumab) Anti-interleukin-13 (e.g. tralokinumab) Human cytotoxic T-lymphocyte-associated antigen 4 (CTLA4) – abatacept Cannabinoids Organ transplantations (e.g. lungs) |