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Hi everyone, this is Chiha Li reporting for RheumNow from APLAR 2026 in Seoul, Korea.
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Today I want to talk about interstitial lung disease, or ILD, particularly in Sjögren's disease, which came up in a day 2 session as part of the conversation around extra-glandular involvement.
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When Sjögren's patients have airway or respiratory symptoms, there are three main things to think about: xerotrachea, which shows up as cough or hoarseness; lymphoproliferative
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disease like lymphocytic interstitial pneumonitis, follicular bronchiolitis, nodular lymphoid hyperplasia, and MALT lymphoma; and then there's ILD, which is going to be our focus today.
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So why does this matter? ILD has gotten more attention at many of the national conferences like ACR and EULAR since the 2023 ACR guidelines have come out, but most of the data and conversation focuses on scleroderma and RA.
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For this talk that focused on Sjögren's, a Korean multicenter cohort showed that the prevalence of ILD is about 6% among those with primary Sjögren's, and that 9% of mortality can be attributed to acute exacerbation of ILD.
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Now how do you predict acute exacerbation?
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They showed that neutrophil-to-lymphocyte ratio above 1.8 flagged exacerbation with about 80% sensitivity.
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So for those who already have the diagnosis, that can be a clue.
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But taking a step back, first you have to figure out who has ILD or is at risk of developing it, because early identification matters.
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The talk was interesting because they focused more on the newer EULAR recommendation guidelines that were published, I believe, earlier this year — like January 2026 — in Annals of Rheumatic Diseases.
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So although this session itself focused on Sjögren's disease, the overview of the guideline pertaining to ILD will be applicable across other connective tissue diseases.
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And where I can, I will try to draw some comparison between the 2023 ACR guidelines versus the 2026 EULAR guidelines.
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To start off, ACR covers five diseases: scleroderma, RA, myositis, Sjögren's, and undifferentiated connective tissue disease.
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The EULAR one differs because they also bring in lupus, and both guidelines cover treatment.
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So the first question: who to screen? Both guidelines are risk-based.
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EULAR strongly recommends screening everyone with scleroderma or mixed connective tissue disease and myositis.
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For RA and Sjögren's, it's a little bit softer — a conditional suggestion to screen only those with risk factors — whereas ACR agrees but is more universally recommending screening.
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The risk factor overlap: so in Sjögren's, EULAR lists older age, male sex, extra-pulmonary involvement, higher CRP, and anti-Ro antibody as risk factors to initiate screening.
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ACR has a shorter list where they highlight just Ro-52 and positive ANA.
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One thing I want to point out when it comes to older age and male sex is that that's not your typical Sjögren's patient, right?
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We're used to seeing patients with Sjögren's in their 40s and 60s with a female predominance.
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So think about that when you see your next patient for screening.
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Second, how to screen? ACR conditionally recommends lung function testing plus HRCT over lung function testing alone.
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Whereas EULAR is firmer — they say HRCT is the screening test of choice and lung function testing shouldn't replace it.
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There's also a split on rescreening. ACR suggests repeating lung function testing every year for at-risk patients, whereas EULAR makes no such recommendation on how often to screen, citing that there's very little evidence outside of scleroderma.
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For baseline assessment once ILD is found, EULAR suggests a baseline severity assessment that includes a six-minute walk test and patient-reported outcomes.
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Now this is different from ACR, which advises against the six-minute walk test and favors ambulatory desaturation testing instead.
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Some of these differences may stem from available resources, but it's interesting to see that patient-reported outcomes are now part of the EULAR guidelines.
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Next question: how often to monitor? ACR suggests lung function testing every 3 to 12 months in the first year for RA and Sjögren's, and less often once patients stabilize.
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EULAR has a tighter recommendation of every 3 to 6 months in the first year for Sjögren's, and the first one to two years for RA — so a longer monitoring period — then at least every 6 to 12 months thereafter.
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It also includes FEV1 because of a possible airway component, which fits Sjögren's disease.
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For repeat HRCT, ACR really doesn't provide intervals but recommends it be more clinically indicated.
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EULAR suggests repeating it every one to two years in Sjögren's, especially in higher-risk patients, and anytime you suspect progression.
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So what counts as high risk? It actually means two different things.
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For screening, it's a higher chance of developing ILD — the risk factors we just covered.
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For monitoring, it's a higher chance that ILD already found will progress, and the guidelines are less specific there on how to define or look for that.
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In RA, EULAR points to a UIP pattern, more extensive disease on HRCT, FVC, and DLCO.
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In scleroderma, it adds autoantibodies like anti-topoisomerase I, skin involvement, and inflammatory markers.
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In myositis, it's anti-MDA5, anti-synthetase, and Ro-52 antibodies.
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For Sjögren's — the focus of this talk — EULAR couldn't make a recommendation.
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ACR suggests closer follow-up for worsening symptoms, a UIP pattern, antibodies like MDA5 and anti-synthetase, and failing lung function, and notes that Sjögren's patients with Ro-52 can progress quickly.
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So in Sjögren's, high risk is largely a clinical judgment.
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So here's my take. Both guidelines agree on the big picture: screen for ILD depending on risk factors, use HRCT in the workup, and watch closely early on.
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They differ on the details that set your clinical schedule — regarding HRCT intervals, choice of tests regarding the walk test or patient-reported outcomes, and rescreening.
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But when it comes to Sjögren's patients with risk factors, a reasonable approach would be a baseline HRCT and lung function, then lung function testing every 3 to 6 months in the first year, and a repeat HRCT.
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The open question is how often to rescreen someone whose first scan is clear and yet where you have concerns, and I think that's where clinical judgment still holds a greater degree of reliance than our guidelines.
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Thank you for listening. This is Chiha Li for RheumNow from APLAR 2026 in Seoul.
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