Lung problems in scleroderma

Many people with scleroderma (also known as systemic sclerosis) have changes in their lungs. But not everyone’s lungs change noticeably. 1-5

Basically, scleroderma can affect your lungs in two different ways: 1, 6

  1. Pulmonary fibrosis (a condition called interstitial lung disease [or ILD]) – the lung tissue thickens, becomes stiff and scarred.
  2. Pulmonary arterial hypertension (or PAH) – the pressure in the blood vessels in the lungs is too high

Patients with scleroderma can get either one, both or none at all.

Shortness of breath is common in scleroderma. This can be unsettling for those affected. However, shortness of breath is not always an alarm sign and does not necessarily mean that pulmonary fibrosis or PAH is present. Be sure to inform your doctor so that your lungs can be examined.

Other terms related to lung problems:

  • Involvement of the lungs
  • Interstitial Lung Disease (or ILD)
  • Pulmonary fibrosis
  • Lung disease
  • Difficulty breathing
  • Pulmonary Arterial Hypertension (or PAH)

Most people don’t notice the changes in their lungs at first. You feel tired and tired. In some cases it is. However, you know your own body best and what you are capable of.

Listen to yourself and try to notice any small changes that could indicate a lung problem. The sooner you know you have lung problems, the sooner you can get tests and support.

It is important to know as soon as possible if your lungs are also affected by scleroderma. Your doctor can then initiate the appropriate treatment for you if necessary.

Understand pulmonary fibrosis (interstitial lung disease)


How many patients get pulmonary fibrosis?

Most patients with scleroderma have pulmonary fibrosis to varying degrees. Only 13–16% have severe symptoms. 1-4, 7, 8


What is happening there?

The lung tissue thickens, becomes stiff and scarred. For some people this means that the lungs no longer work as well as they used to.

With pulmonary fibrosis, the small air sacs in the lungs (also called alveoli) are affected by fibrosis and scarring, which causes stiffening. Air bubbles changed in this way are less able to absorb and release air. The task of these small air bubbles is to allow oxygen from the air you breathe to pass into the blood and to absorb carbon dioxide from the blood, which is then exhaled. The lung tissue in pulmonary fibrosis thickens over time, and the lungs become increasingly immobile and scarred. The lungs can no longer properly expand and carry oxygen into the bloodstream. 9

Some people with pulmonary fibrosis have mild respiratory problems that can only be felt during great exertion. Others may experience severe shortness of breath that interferes with daily activities. 2, 10


Symptoms include: 6, 10

  • Rapid exhaustion from everyday activities
  • Difficulty breathing during simple activities such as climbing stairs
  • Dry, persistent cough
  • Chest tightness
  • Dizziness

How could pulmonary fibrosis affect my life?

Only in some people with lung problems due to scleroderma do the lungs change so much that the disease has a noticeable effect on everyday life. 10

For most people with scleroderma and lung problems, the condition of the lungs remains relatively stable or only slowly deteriorates over many years: 5

  • Seven out of ten people suffer from shortness of breath
  • Six out of ten people reported having problems climbing stairs or walking

Few people have symptoms that worsen quickly after diagnosis:

  • About half of people with lung problems develop these symptoms in the first three years after diagnosis. 2, 4, 11, 12
  • For most people with scleroderma and lung problems, the condition of the lungs remains relatively stable or only slowly deteriorates over many years.

Over time, 1 in 10 people with scleroderma develops severe lung symptoms that affect everyday life. In extremely severe cases, these symptoms will affect lifespan.