Table II.

Current systemic sclerosis treatment and further perspectives

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)