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 MMF Cyclophosphamide 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) Methotrexate Leflunomide Targeted biologic treatments only in case of refractory arthritis: abatacept, rituximab, tocilizumab |
Inflammatory myopathy | Oral corticosteroid therapy Methotrexate Intravenous immunoglobulins |
Diffuse infiltrative lung disease | MMF Intravenous cyclophosphamide followed by azathioprine or MMF Low-dose corticosteroid therapy (10–15 mg/day) (discussed) Rituximab Nintedanib Oxygen therapy Lung transplant |
Pulmonary arterial hypertension | Oxygen therapy Diuretics 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, Beta-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 Dialysis 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.) |