Lung decline stays steady despite rise in Sjögren’s ILD treatment
European study tracks shifts in drug use over three decades
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Immunosuppressive treatment for Sjögren’s disease-associated interstitial lung disease (ILD) has become more common in recent years, particularly among patients with greater respiratory symptoms or poorer lung function, a European study found.
Despite the shift toward more frequent and diverse treatment, the proportion of patients whose lung disease worsened after diagnosis remained largely unchanged over nearly three decades of analyzed data.
As researchers noted, “current practice may intervene too late to fully avert progression,” underscoring the need for studies that can identify patients most likely to benefit from earlier treatment.
The study, “Treatment patterns, disease progression and survival in Sjögren disease-associated interstitial lung disease: a multicentre European cohort study,” was published in the Journal of Autoimmunity.
ILD affects about 20% of Sjögren’s patients
Sjögren’s disease is an autoimmune condition that primarily affects the glands producing tears and saliva, leading to symptoms such as dryness in the eyes and mouth. The disease may also affect other parts of the body, including the lungs.
ILD is marked by inflammation and scarring of lung tissue, leading to shortness of breath (dyspnea) and dry cough. It affects about 20% of people with Sjögren’s and can be associated with reduced survival.
However, there is limited evidence guiding treatment decisions for these patients, and treatment approaches remain variable. To learn more, researchers analyzed results from a European study to examine real-world treatment practices in Sjögren’s-associated ILD.
The study included 191 patients, diagnosed at three centers in Norway, Switzerland, and Austria between 1997 and 2025. Participants had a mean age of 59.9 years and a mean time from diagnosis of 7.1 years, and were mainly women (81.2%). Their most common symptoms were dryness in the eyes and mouth (92.6%), joint involvement (66.7%), and fatigue (61.3%).
Corticosteroids, DMARDs most commonly used treatments
At the start of the study, most patients had preserved lung function, with a mean forced vital capacity (FVC), the volume of air a person can forcibly exhale after a deep breath, of 91.3% of what is expected for someone of similar age, sex, and ethnicity.
Most participants (63.9%) received at least one immunosuppressive treatment during the study, a proportion that increased from 52.4% before 2006 up to 2006 to 71.3% after 2017. These findings show that while management of Sjögren’s-related ILD “has evolved towards broader and more consistent use of immunosuppressive therapy … a substantial proportion of patients remained untreated,” the researchers wrote.
The most commonly used treatments included corticosteroids (42.4%) and disease-modifying anti-rheumatic drugs (DMARDs), including conventional DMARDs such as azathioprine and mycophenolate mofetil, and biologic DMARDs such as rituximab. DMARDs are a class of immunosuppressive medications for autoimmune conditions.
The most used DMARD was rituximab (25.1%), followed by azathioprine (17.3%) and mycophenolate mofetil (16.8%). The antifibrotic treatment nintedanib, marketed as Ofev for pulmonary fibrosis and other conditions, was used in seven patients (3.7%).
Looking at treatment sequences, 45.5% of patients received a DMARD or nintedanib as first-line treatment. Rituximab was the most common therapy used as a first-line standalone, followed by conventional DMARDs. Among patients who received first-line treatments, about one-quarter later moved to a second treatment, while only a small number required third- or fourth-line therapies.
[The study shows that] more consistent use of immunosuppressive therapies and, more recently, the introduction of nintedanib may contribute to disease stabilisation and functional improvement.
Patients with more severe dyspnea or poorer lung function (FVC lower than 70%) were about 2.5 to 3.5 times more likely to receive either type of DMARD, while those with a dry cough were about four times more likely to receive biologic DMARDs.
The use of conventional DMARDs was also significantly associated with higher levels of C-reactive protein, a marker of inflammation, and joint or muscle involvement, while the use of biologic DMARDs was associated with younger age, male sex, higher dyspnea burden, lower FVC, and dry cough.
According to the researchers, these findings suggest that “in the absence of robust prognostic markers, clinicians largely rely on respiratory symptoms and FVC decline to guide therapy.”
Within one year, 9.9% of patients showed improved lung function, defined as an increase in FVC of at least 5%. The proportion showing improvement increased over the study period, from 2.4% among patients diagnosed through 2006 to 11.9% among those diagnosed from 2017 onwards.
In contrast, the proportion of patients whose lung function worsened (at least a 10% decline in FVC) remained relatively stable throughout the study. Five-year survival also showed no clear trend over time.
Overall, the study shows that “more consistent use of immunosuppressive therapies and, more recently, the introduction of nintedanib may contribute to disease stabilisation and functional improvement.” However, the researchers noted that “these approaches do not appear to substantially alter short-term progression rates or clearly improve survival.”
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