Indications for treatment of interstitial lung disease in systemic sclerosis

It can be difficult to identify which people with interstitial lung disease associated with systemic sclerosis require treatment. If interstitial lung disease is suspected, refer the patient to a specialist to ensure the diagnosis is accurate and not delayed.

Note: If interstitial lung disease is suspected, refer the patient to a specialist to ensure the diagnosis is accurate and not delayed.

Treatment of interstitial lung disease is indicated for people who are likely to develop rapidly progressive or life-threatening disease. People who may benefit from treatment include those with:

  • respiratory symptoms and recent disease onset
  • restrictive disease on pulmonary function tests or more than 20% lung involvement on high-resolution computed tomography (CT) scan
  • progressive decline in lung volume or diffusing capacity of the lungs for carbon monoxide (DLCO) on pulmonary function tests
  • other features of systemic sclerosis that warrant immunomodulatory treatment.

If treatment is indicated, a trial of immunomodulatory therapy (eg mycophenolate, cyclophosphamide, rituximab, tocilizumabKhanna, 2020) may be used, with or without low-dose oral corticosteroids. Avoid prednisolone (or prednisone) doses of more than 10 mg daily in patients with systemic sclerosis because it can precipitate scleroderma renal crisis.

Note: Avoid prednisolone (or prednisone) doses of more than 10 mg daily in patients with systemic sclerosis because it can precipitate scleroderma renal crisis.

Stem-cell transplantation may be considered for patients with interstitial lung disease in poor-prognosis systemic sclerosisSullivan, 2018 if the patient has single system disease.

For more details on management for interstitial lung disease see the separate topic in the Respiratory guidelines.