‘Build a good team; don’t forget cystic disease’: Guidance for lung involvement in Sjögren’s
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While the 2021 consensus guidelines for pulmonary disease in Sjögren’s syndrome may have shed light on this underdiagnosed complication, gaps remain in identification and management of the disease, noted a presenter here.
“Lung involvement in Sjögren’s is more common than I think we currently recognize,” Nancy L. Carteron, MD, associate clinical professor of rheumatology at the University of California, San Francisco, told attendees at the 2021 Congress of Clinical Rheumatology-East. “Our best estimates are that anywhere from 15% to 20% of our Sjögren’s patients – whether they are primary or secondary – will have some type of lung involvement. It often goes unrecognized because, fortunately, most of the time it’s not in the severe category, but it can be.”
Carteron noted that are multiple patterns of pulmonary disease in Sjögren’s, with the “hallmark being mucosal involvement and sicca” that manifests in upper and lower airway disease, interstitial lung disease, pulmonary vasculature, lymphoproliferative disease and amyloidosis.
“It’s been recognized that there is variable management of lung involvement in Sjögren’s in multiple settings,” Carteron said. “There have been limited clinical trials, although certainly with scleroderma and interstitial lung disease related to connective tissue, we are beginning to get more defined information about that as opposed to anecdotal. Up until this point, I think there’s been clearly a lack of consensus on best practices within the pulmonary community as well as rheumatology.”
To this end, Carteron and colleagues released Consensus Guidelines for Evaluation and Management of Pulmonary Disease in Sjögren’s, published in CHEST in February, which provided 52 recommendations with an average consensus agreement of 98%.
Although most of the evidence for the recommendations would be considered “low quality” due to the lack of “good randomized clinical trial data,” Carteron noted that the strength of the recommendations comes from the “clinical expertise and basic science expertise in Sjögren’s from experts in pulmonology, rheumatology and hematology/oncology.”
“Once the guidelines were published, we wanted to begin to share that with the wider community and get feedback with regards to whether these would be helpful in both recognizing pulmonary issues in Sjögren’s as well as management, and importantly in identifying large knowledge gaps to help guide future research,” she said.
Of particular note in the recommendations, Carteron pointed out that patients with Sjögren’s syndrome with cystic lung disease may have a higher risk for pneumothorax and should be educated regarding this risk and the associated signs and symptoms.
“Cystic disease in Sjögren’s is not uncommon and, in fact, what I learned from my pulmonary colleagues is that when they see significant cystic disease in their patients, it triggers them to consider whether could this patient have Sjogren’s if it hasn’t already been diagnosed,” she told attendees. “I don’t think this is a super common occurrence as I have not seen this as a major issue over the years, but I think being aware of it and educating patients about it and what they might be able to do is the important point I want to make.”
Lastly, Carteron highlighted the need for a multidisciplinary team approach – including rheumatologists, oncologists, pulmonologists, primary care physicians and radiologists – to adequately manage patients with Sjögren’s syndrome with pulmonary complications. In addition to “screening and surveillance that requires a high index of suspicion,” building a multidisciplinary team could also “help in getting things authorized through insurance,” Carteron noted.
“Hopefully, I’ve been successful in at least planting the seed that the pulmonary manifestations of Sjögren’s are common, running the whole spectrum from airway disease to parenchymal disease, to lymphoproliferative processes, and these guidelines can help provide a roadmap as well as some support evidence,” she said. “Ultimately, these are just broad guidelines for the individual patient scenario that you’re working through; [to wrap-up] build a good team and don’t forget cystic disease.”
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Carteron NL. Pulmonary Manifestations of Sjogren's. Presented at: Congress of Clinical Rheumatology-East annual symposium; August 12-15, 2021 (hybrid meeting).
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