What you should know about Sjogren's lung disease- (rev. 01-14-23)

Most clinicians remain unaware that more than half of Sjogren's patients have evidence of lung disease based on pulmonary imaging, evaluation, including asymptomatic patients (102).

This page summarizes what patients should know about lung (pulmonary) disease and why they may need to advocate for screening and /or a full evaluation.

The Sjogren's Pulmonary Clinical Practice Guidelines (CPGs), are an essential resource for clinicians, especially rheumatologists and pulmonologists. The first page of these guidelines provides an excellent overview of Sjogren's lung disease. I suggest giving rheumatologists, PCPs, ENTs, and pulmonologists a copy of the CPGs along with a print out of the PDF version of the entire journal article. We are indebted to the Sjogren’s Foundation .and the many expert clinicians who volunteered their time to create the CPGs and the journal article which goes into great practical detail.

TIP: You can "market" these documents as recent guidelines created by a team of experts in rheumatology, pulmonology, and oncology (or you can just say top experts). Why would you need to promote a document of such obvious importance? The short answer: Rheumatologists are often unaware that Sjogren’s Clinical Practice Guidelines exist. The American College of Rheumatology (ACR) does not publish and promote Clinical Practice Guidelines (CPGs) that they did not create. The ACR is the main source of clinical guidance for most U.S. rheumatologists, but they do not give Sjogren's its due. The Sjogren’s Foundation stepped up to the plate to create Sjogren’s CPGs because the ACR would not.

Why screen patients who don't have obvious pulmonary (lung) symptoms? Many asymptomatic Sjogren's patients have "silent" lung disease. Early diagnosis and intervention improves outcomes.

“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)

Many patients with Sjogren's lung disease are considered asymptomatic. Many of them actually do have symptoms that are blamed on dryness, asthma, allergies, or being deconditioned.

Symptomatic patients (e.g., chronic cough or shortness of breath) should undergo a thorough evaluation as described in the Pulmonary Clinical Practice Guidelines. A plain chest Xray will not pick up most types of Sjogren's lung disease.

Chronic cough, often blamed on dryness, may be a sign of lung disease. Other symptoms thta may indicate lung disease include shortness of breath, exercise intolerance, chest tightness or burning.

Pulmonary hypertension is a rare but potentially life-threatening pulmonary disease that may occur in Sjogren's. 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."

Sjogren’s lung disease is very common and often remains undiagnosed.

“Up to 65 % of asymptomatic patients have abnormal pulmonary imaging.” This statement in the introduction to the CPGs can be confusing because it follows this one: “Approximately 16% of Sjögren’s patients demonstrate pulmonary complications." So which is it? Lung disease is actually far more prevalent than 16 %. The abnormal imaging in 65% does indicate lung disease, often subclinical (102). Subclinical means no symptoms, mild symptoms, or symptoms that were not noted by a clinician. But just because the symptoms weren’t noted or measured does not mean that lung disease is not there. The lower estimate reflects past studies done almost exclusively in patients with severe and often longstanding symptoms.

My comments:

Moderate, or even severe, interstitial lung disease (ILD) may be present in asymptomatic

patients (89) or chronic obstructive pulmonary disease (COPD). In a series of 51 unselected 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 others. Some patients have more than one type of lung disease. More details are covered in the CPGs, including a section on lymphoma of the lung (uncommon).

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)

In other words, 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.

Specific tests

Asymptomatic patients In an ideal world, every Sjogren’s patient, regardless of symptoms, would have a baseline chest X-ray (CXR) and pulmonary function tests (PFTs). This was the most controversial topic during CPG development. According to the authors, these tests were not recommended more strongly for asymptomatic patients because of “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.”

Note that even these two screening tests would miss some patients with lung disease. This is why it is so important to be evaluated if you have even mild symptoms that might be caused by lung disease.

Symptomatic patients, especially those with chronic cough and or shortness of breath. Every Sjogren's patient diagnosed with asthma should be considered symptomatic. While evaluation should be tailored to individual circumstances. 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 Xray will not pick up most types of Sjogren's lung disease.

Chronic cough may have causes other than Sjogren’s lung disease. Examples include 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 complicated topic that must be managed by a pulmonologist familiar with Sjogren’s lung disease.

ACTION STEPS for Sjogren’s patients

1. Always tell your doctor if you are experiencing possible lung 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 doctors listen to your lungs with a stethoscope at every routine visit. 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, “Do 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).

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

6. Sjogren’s Advocate is written with patients and PCPs in mind. It is best to share primary sources such as peer-reviewed articles, Clinical Practice Guidelines, and other Sjogren’s Foundation materials with rheumatologists and other specialists. I do not recommend printing out Sjogren's webpages and blog posts. See the Clinician Handouts page for tips and strategies.