Interstitial lung disease (ILD), also called pulmonary fibrosis, is a common and serious complication in people with systemic sclerosis (SSc). When you have SSc-ILD, your lungs get inflamed and scarred. These problems in the lungs can make it harder to breathe over time.

Doctors still don’t know the best way to treat SSc-ILD. But there are a growing number of treatment options.

Starting Treatment

If tests of your lung function and lung scans find ILD, your doctor may recommend watchful waiting. This is most likely if the ILD isn’t too bad and there’s a low risk of it getting worse. In this case, you won’t treat the ILD, but your doctor will monitor you to make sure it doesn’t worsen. Doctors often do this because the treatments come with side effects.

If scans show your ILD is mild to severe and you are having trouble breathing because of it, your doctor might suggest you start treatment. The goal of treatment is to keep the ILD stable or slow its progression. When ILD is getting worse or giving you trouble, it's best to get treatment started early before you’ve lost too much lung function.

Choosing a Treatment

If you and your doctor have decided it’s time to start treatment, you will most likely start with a drug to suppress the immune system. These include:

  • Azathioprine
  • Cyclophosphamide
  • Mycophenolate mofetil (MMF)

Usually, doctors will start with MMF because it is safer than cyclophosphamide. If you can’t take either of these drugs, azathioprine may be an option. It’s not clear how long treatment with these drugs should continue, but people can take MMF for years if it’s working. Cyclophosphamide treatment doesn’t usually work for more than 6 months to a year.

No matter which drug you’re taking, your doctor will monitor you for side effects, which may include:

  • Bone marrow suppression, leading to changes in blood cell counts
  • Gastrointestinal symptoms, including nausea, diarrhea, or cramping
  • Changes in kidney function
  • Miscarriage
  • Infertility

More Treatment Options

If your lung function keeps getting worse despite immunosuppressive treatment, there are other options to consider. These include biologic drugs like:

  • Nintedanib, an anti-fibrotic (which means it prevents scarring)
  • Rituximab, which slows disease progression
  • Tocilizumab, which slows the loss of lung function

Experimental Treatment Options

Studies investigating other ways to treat SSc-ILD are under way. Experimental options include:

  • Pirfenidone, an anti-fibrotic used for other types of pulmonary fibrosis
  • Abatacept, an anti-inflammatory used for rheumatoid arthritis and some other rheumatic conditions
  • Hematopoietic stem cell transplant, which replaces damaged stem cells with healthy ones from your own bone marrow or from a donor

Treatment Outlook

While MMF has been the mainstay of treatment for SSc-ILD, the options are growing. Combination treatments also might help. Doctors are still working to understand the best ways to treat or prevent SSc-ILD.

If medications aren’t working, a lung transplant may be an option. This is most likely if you’re in early respiratory failure. But a lung transplant is a difficult procedure that's only an option for a minority of people with SSc-ILD. If you are interested in exploring more options, ask your doctor if there are any new clinical trials to consider. It’s also a good idea to ask if there are other things you can do to help you feel better and improve your quality of life with SSc-ILD.

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