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Most of us have heard of Sjögren’s disease (or Sjögren’s syndrome), an autoimmune condition that targets moisture-producing glands. We associate it with dry eyes and dry mouth (sicca symptoms), and while those symptoms are certainly hallmark features, they are only part of the story.
Sjögren’s disease is a common systemic autoimmune disease, likely affecting about 1 in 300 Australians. This estimate is drawn from international studies, with Australian prevalence data expected in the future. Despite this, it continues to fly under the radar for many clinicians. Sjögren’s disease extends well beyond the eyes and mouth, with the potential to involve multiple organ systems, from the lungs and kidneys to the skin, nervous system, and joints. Left undiagnosed or untreated, it can cause significant morbidity, and in some cases, irreversible organ damage.
More than just sicca symptoms
Part of the problem is that Sjögren’s disease can present in subtle and variable ways. Some patients have the “classic” dryness symptoms; others come with overwhelming fatigue, joint pain, or signs of organ involvement such as lung disease, vasculitis, kidney inflammation, or peripheral neuropathy. The disease can easily be mistaken for other conditions, or dismissed altogether, leading to years of uncertainty and delayed diagnosis.
The average delay from symptom onset to confirmed diagnosis was 5.98 (+/-9.5) years in one study, with some patients waiting decades. In that time, people are often bounced between specialists, mislabelled with unrelated diagnoses, or dismissed entirely. The result is more than just delayed care: it is frustration, isolation, and anxiety for patients; consequences that could be avoided with earlier recognition and intervention.
Why it gets missed
Sjögren’s disease can be deceptively difficult to recognise. Its symptoms often overlap with other connective tissue diseases such as lupus, rheumatoid arthritis, and systemic sclerosis, and patients are often labelled as having undifferentiated connective tissue disease, as Sjogren’s disease hasn’t been considered nor excluded. Common complaints like joint pain, fatigue, and cytopenia are not only shared across these conditions, but are also among the most frequent first presentations of Sjögren’s disease. The usual diagnostic anchor points can’t always be relied on. Around one in four patients do not have a positive antinuclear antibody (ANA) nor the “classic” anti-Ro52/Ro60 (SSA) antibodies, meaning serology alone cannot confirm or exclude the disease. For some, the presentation is subtle or non-specific, with a scatter of symptoms that doesn’t immediately point in one direction. For others, the picture is even more challenging: so-called “occult” Sjögren’s disease, where systemic features such as neuropathy, interstitial lung disease or renal tubular acidosis appear in the absence of any dryness symptoms.
As busy clinicians, it’s easy to focus on more acute health issues in front of us. But if Sjögren’s disease isn’t on our radar, we don’t investigate for it, and we miss the chance to intervene before organ damage occurs.
The Australian response
Until recently, there’s been little coordinated action on Sjögren’s disease in Australia. That’s changing. In late 2024, the Australian Rheumatology Association launched the Sjögren’s Disease Special Interest Group. It’s a forum for rheumatologists, immunologists, ophthalmologists, dentists, researchers, and other interested clinicians to share knowledge, develop resources, and push the science forward.
At the same time, patients have mobilised. Sjögren’s Australia is a new national, patient-led advocacy organisation, the first of its kind here. They’re tackling long-standing gaps in public awareness, patient support, and research. Importantly, they’re partnering with clinicians and researchers to co-design education and care pathways. Their logo, inspired by fluorescein staining of the dry eye, is already sparking conversations in clinics.
Linking in globally
Although the Australian Sjögren’s disease community is still small, we’re plugged into international efforts. We’re contributing to the Asia Pacific League of Associations for Rheumatology, the Outcome Measures in Rheumatology (OMERACT) Sjögren’s Working Group, and the Big Data Sjögren Consortium. These collaborations mean that Australian patients and clinicians will benefit from cutting-edge science, and our local data will inform the global understanding of the disease.
What we can do now
For Australian health care practitioners, there are some straightforward but crucial steps we can take to improve recognition and care for people with Sjogren’s disease.
- Consider Sjögren’s disease on the differential for patients with unexplained sicca symptoms, persistent fatigue, or systemic features.
- Remember: sicca symptoms are important, but the absence of sicca symptoms does not exclude a diagnosis of Sjögren’s disease.
- Take a thorough history, small clues often add up to the bigger picture.
- Look for signs of dryness on examination, including dry tongue, gum recession, dental decay, or dry eye on Schirmer’s test.
- Order targeted investigations, including antibody testing, ocular staining score, salivary flow testing, and where indicated, minor salivary gland biopsy.
- Refer early to rheumatology, especially if systemic manifestations are present.
- Connect patients with support networks, such as Sjögren’s Australia, to provide education, community and advocacy.
Preparing for what’s next
Encouragingly, the treatment landscape for Sjögren’s disease is shifting. In August 2025, Novartis announced positive top-line results from the NEPTUNUS-1 and NEPTUNUS-2 global phase III trials of ianalumab (VAY736). These are the first-ever phase III studies to show a statistically significant reduction in systemic disease activity for Sjögren’s disease, measured by improvements in the EULAR Sjögren’s Syndrome Disease Activity Index (ESSDAI).
Ianalumab is a novel monoclonal antibody with a dual mechanism of B-cell depletion and BAFF-R inhibition, targeting a key driver of Sjögren’s disease pathophysiology. The drug was well tolerated and had a favourable safety profile in both trials. Other targeted therapies are also on the horizon, including agents directed at interferon pathways, blockade of immunoglobulin recycling via inhibition of the neonatal Fc receptor, and co-stimulatory molecule inhibition, signalling a new era in disease-specific treatment.
If approved, ianalumab could become the first targeted disease-modifying therapy for Sjögren’s disease, moving us beyond purely symptomatic management. While regulatory submissions are still ahead, these results highlight why early identification and referral matter: patients diagnosed late may miss the window to benefit from such therapies. For clinicians, this is both a challenge and an opportunity, to think of Sjögren’s disease sooner, act earlier, and be ready to connect our patients with the next generation of treatments when they arrive.
Prof Alberta Hoi is a clinician-researcher and rheumatologist who leads the Monash Sjögren Registry. She brings extensive expertise in longitudinal cohort studies to research focused on predictors, outcomes, and patient-centred care in autoimmune diseases.
Dr Adrian Lee is a clinical immunologist and immunopathologist at Westmead Hospital with strong interests in the diagnosis and management of Sjögren’s disease. He established Sydney’s first Sjögren’s biobank and registry to promote awareness and research into this disease.
Gavin Marshall is the founding director of the not-for-profit patient organisation Sjögren’s Australia and a firefighter, dedicated to advocacy and community service.
Dr Chandra Kirana is a postdoctoral research officer for the rheumatology unit working closely with Prof Maureen Rischmueller on Sjögren’s projects.
Prof Maureen Rischmueller is a rheumatologist who established Australia’s first state-wide Sjögren’s registry and biobank in South Australia, leading research into the disease’s genetic risk factors, systemic manifestations and novel autoantibody associations.
The statements or opinions expressed in this article reflect the views of the authors and do not necessarily represent the official policy of the AMA, the MJA or InSight+ unless so stated.
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