Scleroderma is a chronic autoimmune condition that can manifest either locally, often only in the skin (localized scleroderma), or affect multiple organs such as the lungs, intestinal tract, or heart (limited or diffuse systemic scleroderma).

Several types of lung disease may develop in people with systemic scleroderma with lung involvement. Interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are the two most common and severe types of lung disease, and are leading causes of death in scleroderma patients.

Other lung diseases associated with scleroderma are pleural effusion, a build-up of fluid in the lining of the lung, and lung cancer.

Interstitial lung disease (ILD)

Scleroderma is characterized by an abnormal deposition of collagen (the substance that forms scar tissue) in tissues and organs. ILD develops when too much collagen accumulates in the lungs, making the stiff.

ILD can also develop as a side effect of medications used to treat scleroderma, including methotrexate, cyclophosphamide, CellCept (mycophenolate mofetil), and azathioprine. As a study notes, “most of the medications used in the treatment of SSc [systemic scleroderma] have been associated with the development of pulmonary toxicity.”

Patients with ILD are limited in their ability to expand their lungs while breathing. The lungs’ alveoli or air sacs, where oxygen is absorbed into the bloodstream and carbon dioxide removed, have become thickened and damaged, limiting their ability to take in sufficient oxygen.

Symptoms of ILD develop gradually, with patients commonly experiencing shortness of breath and a dry cough. People with diffuse systemic scleroderma are more likely than patients with limited disease to develop ILD.

Pulmonary arterial hypertension (PAH)

PAH is a condition marked by increased resistance to blood flow in the vessels supplying blood to the lungs. It may develop in scleroderma patients as a result of inflammation damaging the lining of the pulmonary arteries, causing blood vessels to narrow increasing resistance. Or  it can develop as a complication of ILD, as thicker lung tissues cause more resistance to blood flow.

People with mild PAH may experience no symptoms, but moderate to severe disease is usually marked by persistent shortness of breath, especially when exercising. PAH places a strain on the right side of the heart, and ca result in right-sided heart failure if untreated.

PAH is develops more frequently in people with limited systemic scleroderma; about 13% to 25% of PAH patients have the disease’s diffuse form.

Other scleroderma-related lung diseases

Pleural effusions occur in scleroderma because of the disease’s underlying inflammatory processes. Patients may experience no symptoms if fluid accumulation is small; others  report chest pain, shortness of breath, and cough.

It is thought that scleroderma patients may be at a slightly higher risk of developing lung cancer. Patients with ILD have been seen to have a higher than average incidence of lung cancer.

Diagnosing lung disease in scleroderma patients

Lung disease in scleroderma may be diagnosed through a series of breathing tests also known as pulmonary function tests, or through echocardiograms, chest X-raysCAT scans, or MRI scans.

A small piece of lung tissue could also be removed, a procedure known as a lung biopsy, and analyzed to confirm or dismiss a suspected diagnosis of ILD or lung cancer.

Patients with pleural effusions may need to undergo thoracentesis, a procedure where a needle is inserted into the chest to collect the accumulated fluid for further tests.

Disease treatment and management

There is currently no cure for ILD or PAH, but available medications can relieve symptoms and help to slow disease progression.

People with pleural effusion may be treated with anti-inflammatory medications, such as NSAIDs or prednisone. The fluid may also be drained via thoracentesis.

Lung cancer patients may be  asked to undergo surgery, and chemotherapy or radiotherapy, depending on their overall health and the site of the cancer, its type, andits stage.

Lifestyle changes, such as not smoking and avoiding exposure to smoke and other air pollutants, and engaging in deep breathing exercises and a graduated aerobic exercise program, may be helpful in making breathing easier for patients with any form of scleroderma-related lung disease.

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