Table 4 Combination of keywords, used for each question, to perform the systematic literature search
CategoryCombination of keywords
Cyclophosphamide“Cyclophosphamide OR Endoxan” AND “scleroderma [MAJR]”
Prostacyclins“Prostanoids OR iloprost OR beraprost OR cisaprost OR teprostenil OR epoprostenol” AND “scleroderma [MAJR]”
Endothelin receptor antagonists“Endothelin receptor antagonists [tw]” OR “bosentan” OR “sitaxsentan” AND “scleroderma [MAJR]” AND “digital ulcers” OR “Raynaud phenomenom” OR “necrosis”
“Endothelin receptor antagonists [tw] OR bosentan OR sitaxsentan” AND “scleroderma [MAJR]”
Selective PDE inhibitors“Phosphodiesterase Inhibitors OR sildenafil OR tadalafil OR vardenafil” AND “scleroderma [MAJR]”
Corticosteroids“Steroids or prednisone OR prednisolone OR dexamethasone OR methylprednisolone” AND “scleroderma [MAJR]”
Stem cell transplantation“Autologous stem cells transplantation” AND “scleroderma [MAJR]”
ACE inhibitors and angiotensin receptor antagonists (sartans)“Angiotension converting enzyme inhibitors” AND “scleroderma [MAJR]”
“Angiotensin receptor antagonists [tw]” OR “Losartan” AND “scleroderma [MAJR]”
Immunosuppressive drugs“Cyclosporin OR cyclosporine” AND “scleroderma [MAJR]”
“Mycophenolate mofetil OR mycophenolic acid OR CellCept” AND “scleroderma [MAJR]”
“Metothrexate” AND “scleroderma [MAJR]”
“Azathyoprine OR Imuran” AND “scleroderma [MAJR]”
Calcium channel blockers“Calcium channel blockers OR Nitrendipine OR Nisoldipine OR Nimodipine OR Nifedipine OR Nicardipine OR Isradipine OR Felodipine OR Amlodipine OR Dihydropyridines or verapamil OR diltiazem” AND “scleroderma [MAJR]”
Proton pump inhibitors“Proton pump inhibitors” AND “lung fibrosis” AND “scleroderma [MAJR]”
Other treatments“Prokinetic OR clebopride OR mosapride OR erythromycin OR octreotide OR misoprostol OR metoclopramide OR domperidone OR cisapride OR prucalopride” AND “scleroderma” [MAJR]”
“Calcinosis OR calcinosis treatment” AND “scleroderma [MAJR]”
“Antibiotics” AND “gastrointestinal” OR “malabsorption” OR “anorectal” AND “scleroderma [MAJR]”
“NSAIDs OR “Non-steroidal antiinflammatory drugs” or “cyclooxygenase inhibitors” or “COX inhibitors” AND “scleroderma [MAJR]”
  • Search was also performed replacing scleroderma by systemic sclerosis and then by CREST. Medical subject heading (MeSH) search was used for all databases unless stated otherwise (text word, [tw] or major [MAJR]).

  • ACE, angiotensin converting enzyme; PDE, phosphodiesterase.