What you should know about Sjogren's lung disease- (rev. 05-11-2023)

Research shows that lung (pulmonary) disease impacts more than half of people with Sjogren's (102). Because many doctors do not know that Sjogren's lung disease is common, patients might need to request testing for this. Lung disease may occur without obvious symptoms. Early diagnosis, monitoring, and management of lung disease are key to better outcomes. The Sjogren's Foundation's Pulmonary Clinical Practice Guidelines (CPGs) are an essential resource for rheumatologists and pulmonologists. I recommend sharing a copy of the CPGs with rheumatologists, primary care clinicians, ENTs, and pulmonologists along with a printout of the PDF version of the entire journal article (explains the CPGs in detail). Why screen patients who don't have obvious pulmonary (lung) disease symptoms? Many asymptomatic Sjogren's patients have "silent" lung disease.

“Normal lung physiology features inherent functional reserves and inevitably significant pathology exists before patients become symptomatic. This is common in many lung diseases, partially accounting for disappointing outcomes in chronic respiratory conditions including ILD.” (200) (ILD- interstitial lung disease)

Many patients considered "asymptomatic" actually do have symptoms. Lung disease symptoms include: -chronic cough -shortness of breath -exercise intolerance -chest tightness and burning Chronic cough may be a sign of lung disease and should not be automatically attributed to dryness.

Symptomatic patients should undergo a thorough evaluation as described in the Pulmonary Clinical Practice Guidelines. A plain chest X-ray will not pick up most types of Sjogren's lung disease. Lung disease must be ruled out, even if the clinician suspects symptoms are caused by dryness, gastroesophageal reflux disease (GERD),asthma, allergies, obesity, or being deconditioned. Covid and Long Covid may cause lung disease and most be considered in patients with a history of infection.

Sjogren’s lung disease is common but often remains undiagnosed.

“Up to 65 % of asymptomatic patients have abnormal pulmonary imaging.” This statement in the CPG introduction is confusing because it follows this one: "Approximately 16% of Sjögren’s patients demonstrate pulmonary complications." So how many patients have lung disease?

We know that Sjogren's lung disease is underdiagnosed. It is far more prevalent than 16%. The lower estimate of 16% reflects the fact that pulmonary evaluation in Sjogren's is typically limited to patients with severe and longstanding symptoms. The abnormal imaging in 65% includes both clinical and subclinical lung disease (102). Lung disease is often overlooked.

Subclinical lung disease includes patients with no symptoms, mild symptoms, or symptoms that were not noted by a clinician. Just because the symptoms weren’t noted or measured does not mean that lung disease is not there.

-Moderate, or even severe, interstitial lung disease (ILD) may be present in asymptomatic patients (89). -Chronic obstructive pulmonary disease (COPD) is also common. In a series of 51 unselected (not chosen based on symptoms) Sjogren's patients, 41% overall, and 34% of never-smokers, met the GOLD criteria for COPD (158).

There are many types of Sjogren’s lung disease: Interstitial lung disease, COPD, cystic lung disease, bronchiolitis, bronchiectasis, and less common problems such as lymphoma may be caused by Sjogren's. Some patients have more than one type of lung disease. Pulmonary hypertension and pulmonary arterial hypertension (PH and PAH) impact the blood vessels in the lungs and are discussed in the CPGs. PH and PAH are life-threatening. Once thought of as rare in Sjogren's, more patients are being diagnosed with these conditions, especially PAH. From the Pulmonary Hypertension Association Sjogren's brochure:

"Pulmonary hypertension should move to the top of the possible causes

for people with shortness of breath, swelling of the legs, chest pain and

fainting and normal pulmonary function tests."

Learn more about Pulmonary Hypertension in Sjogren's

Sjogren’s lung disease demonstrates a wide range of severity. Lung disease may follow a mild course, in which case it may not need treatment. Regardless of severity, it must be monitored for progression because it can lead to serious breathing problems and lower quality of life. It is the direct cause of death in a small but significant number of Sjogren’s patients. Early diagnosis and management are key to better outcomes.

Who should get tested for lung disease?

• All Sjogren’s patients should be routinely screened with a lung symptom history and physical examination, regardless of symptoms. See ACTION STEPS below.

• Decisions about evaluation for lung disease should be the same regardless of serologic markers such as SS-A, ANA, etc. (Table 1 journal article, Pulmonary CPGs)

SS-A negative patients should be evaluated in the same manner as SS-A positive patients. It is a common misperception that SS-A negative patients don't get serious systemic features. They can and they do. Learn more here.

What tests are used to look for lung disease?

Asymptomatic patients should have a baseline chest X-ray (CXR) and pulmonary function tests (PFTs). Note that even these two screening tests will miss some patients with lung disease. Ideally, every Sjogren's patient would get a baseline HRCT, but this is rarely done because of cost and concern about radiation exposure. Screening of asymptomatic patients was a controversial topic during CPG development. According to the authors, the PFT and CXR were not recommended more strongly (as a "must") for asymptomatic patients because “the burden and expense of obtaining PFTs as well as an improbability of non-pulmonologists ordering such tests due to inadequate awareness of and appreciation for pulmonary manifestations in Sjogren’s.” In other words, they did not think the rheumatologists would follow the recommendations because of their unfamiliarity with Sjogren's lung disease. Comment: Like some of the authors, I do not agree with this approach because it puts the burden on patients to advocate for the recommended baseline screening.

Symptomatic patients, especially those with chronic cough, shortness of breath, or "asthma" should have a full lung disease evaluation. Complete PFTs and high resolution CT scan (HRCT) are recommended for any Sjogren’s patient with pulmonary symptoms or findings on examination. A plain chest X-ray will not pick up most types of Sjogren's lung disease.

Chronic cough may have many causes, including dry trachea, gastroesophageal reflux, asthma and others (See Table 3, journal article CPGs). Keep in mind that Sjogren’s lung disease may co-occur with other conditions.

Lung disease treatments

See the Consensus Guidelines journal article for detailed treatment recommendations. This is a complex topic that should be managed by a pulmonologist (lung specialist) familiar with Sjogren’s lung disease.

ACTION STEPS for Sjogren’s patients

1. Always tell your rheumatologist if you are experiencing possible lung disease symptoms, even if they are mild. Examples include: chronic cough, wheezing, chest burning/ tightness, and shortness of breath, especially on exertion.

2. Be sure that your rheumatologist or primary care doctor listens to your lungs with a stethoscope at routine visits. It is especially important that they listen to the base of your lungs (near the bottom of the rib cage). This is tricky to bring up if they are not already doing this: You might ask, “Would you mind listening to my lungs today?”

3. Copy the Flow Chart (Figure 1, page 687 of PDF version of journal article) (click the PDF icon at the top.) Take this handy one-page summary to use for discussion with your clinicians.

4. If you are asymptomatic, ask for baseline screening tests, and share the recommendation on Table 1, item 1 (page 686) of the journal article) (click the PDF icon at the top of the link).

5. Offer printouts of the entire CPG document and journal article to your rheumatologist, pulmonologist, ENT and PCP.

REMINDER: It is best to share primary sources such as peer-reviewed articles, Printable Handouts for Clinicians, Clinical Practice Guidelines, and Sjogren’s Foundation brochures and resource sheets with your doctors. See the Handouts for Clinicians page for tips and strategies.