Lung Complications and Treatments

Pulmonary Arterial Hypertension (PAH)

Although skin lesions are the most visible effect of scleroderma, most major body systems are also affected—especially the circulatory system. One of the most common and dangerous complications of vessel damage is PAH. This condition occurs when high blood pressure develops in the arteries that carry blood between the heart and the lungs.

Shortness of breath and reduced exercise capacity can result from the development of scar tissue (fibrosis) in the lungs, due to collagen production and other cellular changes.

Shortness of breath and reduced exercise capacity can result from the development of scar tissue (fibrosis) in the lungs, due to collagen production and other cellular changes.

Because scleroderma also causes thickening in the walls of blood vessels, the vessels that carry blood to and from the lungs are less able to flow smoothly. This can lead to a life-threatening condition, pulmonary arterial hypertension, or PAH.

Regular screening for pulmonary lung function, echocardiography, chest radiography or CT scans can help identify lung problems. You may be referred to a pulmonologist for further assessment and treatment recommendations.

Between 8% and 12% of those who have systemic sclerosis also have PAH. It is the leading cause of disability and death among these patients, accounting for 30% of deaths.

PAH has been considered to be a later complication of scleroderma, but because symptoms are often not recognized, it actually may develop much earlier.

Part of the problem is that there are few very early symptoms of PAH, and these are often mistaken for a general lack of fitness. Symptoms that develop later are often typical of other conditions (such as asthma), which can cause a further delay in diagnosis. Patients may experience:

  • Shortness of breath during normal activities such as climbing stairs
  • Tiredness or fatigue
  • Chest pain
  • Rapid heartbeat
  • Pain in the upper right side of the abdomen
  • Decreased appetite

As PAH worsens, other symptoms may appear, including light-headedness during activity, fainting, swelling in the legs and ankles, and a bluish color in the lips and skin.

Since people with scleroderma are known to be at high risk for PAH, regular screening can identify the condition in earlier stages. However, because patients rarely report early symptoms such as shortness of breath and fatigue, they may be severely affected by the time their PAH is diagnosed.

The discovery that endothelial cell dysfunction was a key trigger for the development of PAH, and since then, several treatment options have been developed over the past 20 years. Although there is no cure for PAH, treatment can help to relieve symptoms, improve function, and slow the progression of the disease.

Medications for scleroderma-related PAH may be injected, inhaled, or taken by mouth. The goal of all these treatments is to relax the blood vessels in the lungs and reduce excess cell growth, so that blood can flow more easily.

  • Prostaglandin Derivatives
  • Epoprostenol
  • Treprostinil
  • Iloprost
  • Endothelial Receptor Antagonists
  • IP Receptor Agonists
  • Soluble Guanylate Cyclase Stimulators
  • Phosphodiesterase type 5 (PDE5) inhibitors

Interstitial Lung Disease (ILD)

ILD is another complication of systemic sclerosis which may affect breathing. ILD occurs when there is inflammation and scarring to the lung tissue that supports the alveoli—the air sacs within the lungs. Over time, this scarring makes it difficult to breathe in and out, and also reduces the amount of oxygen that can reach the bloodstream.

There are treatments that can improve ILD symptoms, slow the progression of the disease, and improve patients’ quality of life.

There are several factors that are thought to trigger ILD development, or cause it to worsen:

  • Inflammation in lung tissue, which can have many causes
  • Tissue damage and lung fibrosis
  • Viruses and bacteria that may cross-react with collagen, a key component in lung tissue and produced in abnormal amounts in scleroderma
  • Occupational factors, including asbestos exposure, coal or grain dust, mold, or silica dust
  • Medications such as chemotherapy or immunomodulating drugs, heart medications, and some antibiotics

Even though the scarring of lung tissue caused by ILD cannot be reversed once it has occurred, there are treatments that can improve symptoms, slow the progression of the disease, and improve patients’ quality of life.

It is important that those with scleroderma receive regular screening for pulmonary lung function, to identify ILD and other potential lung problems early. Patients may be referred to a pulmonologist for further assessment and treatment.

Supplementary oxygen may be prescribed if oxygen levels are low when measured at rest or with activity.

For patients with severe ILD who have experienced significant scarring, lung transplantation may be required.