Guidelines Issued for Managing Lung Symptoms

The Sjögren’s Foundation has developed clinical practice guidelines for healthcare providers to improve the diagnosis, evaluation, and care of Sjögren’s syndrome patients with lung manifestations.

The guidelines, which include 52 recommendations for Sjögren’s evaluation and management in patients with pulmonary symptoms, are based on the strength of supporting evidence and practical recommendations.

Pulmonary manifestations are a frequent and potentially serious complication of Sjögren’s syndrome, affecting about 16% of patients, many of whom may be asymptomatic.

These manifestations may include airway disorders like chronic obstructive pulmonary disease, interstitial lung diseases (ILD) that cause scarring of the lung tissue, or lymphoproliferative disorders — such as blood cancers and other diseases caused by the overproduction of immune cells called lymphocytes.

Overall, the new guidelines recommend a multidisciplinary approach to identify and manage pulmonary complications in Sjögren’s patients, including evaluation by a rheumatologist, primary care physician, pulmonologist, pathologist, and radiologist, and an oncologist when necessary.

The guidelines encourage doctors to obtain a detailed respiratory-related medical history and consider a chest X-ray for all Sjögren’s patients, especially if lung involvement is suspected.

When evaluating patients with pulmonary symptoms such as difficulty breathing and suspected pulmonary hypertension (elevated blood pressure in blood vessels supplying the lungs) or cardiac involvement, the guidelines recommend an echocardiogram.

Lung function tests and other imaging scans, such as computed tomography (CT) or an angiogram that take pictures of lung blood vessels, also may be requested to more accurately evaluate pulmonary manifestations.

For Sjögren’s patients with suspected ILD, the guidelines recommend high-resolution CT scans and a test that measures blood oxygen level. For patients with confirmed ILD, there also is a strong recommendation for immunization against the flu and pneumococcal infection in accordance with the U.S. Centers for Disease Control and Prevention guidelines.

The guidelines also recommend that healthcare providers and patients be aware of the potential risks associated with the use of common treatments for ILD and related disorders in Sjögren’s patients, including systemic steroids, azathioprine, mycophenolate mofetil, and rituximab.

According to the guidelines, all Sjögren’s patients should be monitored for signs of lymphoproliferative diseases. Patients with symptoms suggestive of lymphomas, such as unexplained weight loss, fever, and swollen lymph nodes, should receive further evaluation. 

In addition to the recommendations, the guidelines include checklists to screen for pulmonary involvement in Sjögren’s patients, as well as for Sjögren’s syndrome in patients with lung manifestations.

They also have information about drug maintenance therapy for patients with Sjögren’s and symptomatic ILD, and schematics for the evaluation and management of Sjögren’s patients with pulmonary complications.