Scleroderma can affect both lung tissue and blood vessels in the lungs, raising the risk to a patient of developing complications like interstitial lung disease (ILD), the name given a group of lung disorders that generally involve progressive scarring of lung tissue, and pulmonary arterial hypertension (PAH).

What is ILD?

Interstitial lung disease is caused by inflammation and the buildup of scar tissue in the lungs. The scar tissue affects breathing and hampers the lungs’ ability to transfer oxygen to the bloodstream.

How is ILD diagnosed?

ILD may first be detected when the lungs make crackling sounds that can be heard by an examining physician using a stethoscopic. If such sounds are heard, the patient may be asked to undergo a complete lung function test, which includes measures of lung volume and breathing mechanics. Lung function will be reduced if inflammation and fibrosis are present.

If ILD is suspected based on a patient’s medical history and lung function tests, the next step will be to determine the extent of the inflammation and fibrosis. This is usually done with a high-resolution CT scan (HRCT) of the lungs.

Other tests to assess the extent of lung inflammation include bronchoalveolar lavage (BAL) and/or a lung biopsy. BAL is done by a specialist who introduces a slender flexible telescope into the lungs and washes up a sample of fluid from the base of the lungs, which is then studied under a microscope. A lung biopsy is an invasive surgical procedure done by introducing an operating telescope through an incision made in the rib cage. Tissue obtained in this way is also studied under a microscope and undergoes extensive laboratory testing.

How is ILD treated?

There are a number of therapies available to treat ILD.

For example, the anti-fibrotic agent Esbriet (pirfenidone) inhibits a chemical mediator that plays a key role in fibrosis and a cytokine that is active in inflammation, while Ofev (nintedanib), a tyrosine kinase inhibitor, targets growth factor receptors that are involved in the mechanisms of fibrosis. Both medicines have been approved for the treatment of a rare form of ILD called idiopathic pulmonary fibrosis (IPF).

Additionally, medications that suppress the immune system, such as Imuran (azathioprine) and CellCept (mycophenolate mofetil), and alkylating agents that are used to slow or stop the growth of cancer can also be used to treat scleroderma-related ILD.

Lifestyle changes such as quitting smoking and avoiding exposure to air pollutants can also help reduce ILD symptoms. Regular use of deep breathing exercises and a graduated aerobic exercise program may also help patients breathe easier.

Early diagnosis and early use of preventive treatments for ILD are key, as once lung damage has occurred, it cannot be reversed.

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