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Table 5 Systemic sclerosis treatments based on affected organ

From: French recommendations for the management of systemic sclerosis

Manifestations Treatment
Peripheral vascular damage Calcium channel blockers
Prostacyclin analogs
Endothelin A and B receptor antagonists: bosentan (prevention of occurrence of new digital ulcers)
Phosphodiesterase type 5 inhibitors: sildenafil (healing of digital ulcers)
Platelet aggregation inhibitors in case of macroangiopathy
Skin damage Methotrexate
Therapeutic intensification and autologous hematopoietic stem cell graft in case of severe and progressive diffuse form after validation of indication in MCM (see Appendix 4)
Articular damage Nonsteroidal antiinflammatory drugs if no upper digestive damage
Low-dose corticosteroids (≤ 10 mg/day)
Targeted biologic treatments only in case of refractory arthritis: abatacept, rituximab, tocilizumab
Inflammatory myopathy Oral corticosteroid therapy
Intravenous immunoglobulins
Diffuse infiltrative lung disease MMF
Intravenous cyclophosphamide followed by azathioprine or MMF
Low-dose corticosteroid therapy (10–15 mg/day) (discussed)
Oxygen therapy
Lung transplant
Pulmonary arterial hypertension Oxygen therapy
Endothelin receptor antagonists: bosentan, ambrisentan
Phosphodiesterase 5 inhibitors: sildenafil, tadalafil
Prostacyclin receptor agonists: selexipag
Prostacyclin analogs: epoprostenol, treprostinil
Atrial septostomy
Lung or heart–lung transplant
Heart Calcium channel blockers
Angiotensin II converting enzyme inhibitors or angiotensin II receptor blockers or neprilysin inhibitors and angiotensin II receptor blockers,
Diuretics–mineralocorticoid receptor antagonists
Antiarrhythmia drugs
Implantable defibrillator/stimulator
Sometimes immunosuppressants if myocarditis
Heart transplant
Renal Crisis Angiotensin-converting enzyme inhibitors
Intravenous calcium channel blockers
Kidney transplant
Digestive damage Esophagus: proton pump inhibitors, prokinetics (metoclopramide, domperidone)
Stomach: proton pump inhibitors, erythromycin (125–250 mg × 2/day), clavulanic acid, prokinetic (metoclopramide, metopimazine)
Small bowel: in case of motility disorders and/or intestinal pseudo-obstruction, octreotide (50–100 µg/day)
Colon: in case of constipation, balanced diet with fibers and mucilages, adequate hydration, regular physical activities, laxatives and cleansing enemas, prokinetics for a limited period (metoclopramide, domperidone)
Enteral and parenteral feeding: in case of severe small bowel damage or deglutition disorders
Small intestinal bacterial overgrowth: sequential antibiotic therapy (three antibiotics among amoxicillin, metronidazole, fluoroquinolones, gentamycin, etc.)
  1. MCM multidisciplinary coordination meeting, MMF mycophenolate mofetil