What is Sjögren’s?

Symptoms

Sjögren’s can cause mild to severe dryness of the eyes, mouth, nose, skin, digestive tract and vagina. The reduced moisture in these parts of the body can result in tooth decay, oral infections, difficulty eating, swollen glands, vision problems, eye pain, sinus pain, difficulty sleeping, swallowing problems, constipation, and sexual dysfunction. 

In addition, Sjögren’s can affect any part of the body, causing profound fatigue, skin rashes, chronic joint and muscle pain, an increased risk of lymphoma, headaches and migraines, brain fog, Postural Orthostatic Tachycardia Syndrome (POTS), and pregnancy complications. It can also result in damage to the kidneys, blood vessels (vasculitis), liver, pancreas, digestive system, nerves (peripheral neuropathies & dysautonomia), and lungs. These complications can range from mild to severe, for example the lung complications range from mild reduction in lung function to severe complications such as idiopathic pulmonary fibrosis (Barendregt et al 1998; Ramos-Casals et al 2020). 

Diagnosis

Diagnosing Sjögren’s can be difficult due to lack of awareness of the symptoms amongst the public and healthcare professionals. As Sjögren’s can affect the whole body, often healthcare professionals and patients don’t ‘connect the dots’ between the various symptoms. Diagnosing Sjögren’s can also be tricky because the most common symptoms can mimic other autoimmune diseases, menopause and drug side effects. 

Unfortunately, there is no single test for Sjögren’s. Diagnosis can be based on the results of a range of tests (of the eyes, mouth and blood), combined with the person’s symptoms.  

Common tests for people with suspected Sjögren’s include: 

• Blood tests – a range of blood tests are used to check for autoimmune diseases. Anti-Ro/SSA & Anti-La/SSB antibodies in particular are associated with Sjögren’s. It’s important to note that Anti-Ro & Anti-La antibodies are only present in approximately 60% of cases. So, it’s possible to have Sjögren’s, even if this test is negative. This is known as seronegative Sjögren’s. 

• Ocular surface staining – a special dye is dropped into your eye so that any dry patches can be seen. 

• Schirmer’s test – a special strip of paper is placed under your eyelid for 5 minutes and then the amount of moisture is measured. 

• Lip biopsy – a small sample of salivary (labial) gland tissue is taken from your lip. An expert looks at the tissue for any signs of Sjögren’s under a microscope. 

• Salivary gland ultrasound or MRI – changes to the large salivary glands beside your ear or under your chin, may show up in these less invasive scans. 

• Salivary flow test (sialometry) – you are asked to spit into a container for 5 mins and afterwards the amount of saliva is measured. 

• Urine test – this test may show if there are any changes to your kidney function.

Early diagnosis is important for improving outcomes, yet some people with Sjögren’s report waiting over a decade for diagnosis (Greenan et al 2023). There is evidence that long delays from first symptoms to diagnosis can result in irreversible gland and organ damage (Retamozo et al 2020).

Treatment

Management of Sjögren’s has not changed much in recent decades and is primarily based on managing symptoms of dryness or uses systemic drugs that mostly treat other conditions. Treatment protocols, which are not specific for Sjögren’s, can be ineffective, burdensome and costly (Seror et al 2021). Many people with Sjögren’s have other conditions that can make treatment more challenging, as drug interactions occur which can aggravate symptoms.  

Each person’s treatment is decided on a case-by-case basis and depends on many factors. Your doctor may take into consideration whether the disease is affecting other parts of the body (such as organs or joints), the risks versus benefits of a medication, and what side effects it may cause. 

The British Society for Rheumatology in their 2024 Sjögren’s disease guidelines recommended the following: 

• Start preservative-free lubricating eye drops 4 times per day. 

• Use warm eye compress for 10 mins daily. 

• Try saliva substitutes. 

• Regularly attend the dentist. 

• Long term monitoring of the condition by a doctor, especially of those at high risk of lymphoma. 

• Wear glasses to reduce tear evaporation. 

• Maximise Omega 3 through diet or supplements. 

• Avoid dry, smoky environments. 

• Humidify your environment – turn down heating, put saucers of water on radiators. 

• Reduce sugar consumption. 

• Wash and floss teeth carefully. 

• Stay hydrated by drinking plain water. 

• Keep active. 

Living with Sjögren's

“It wasn’t taken seriously by doctors and…it was having a significant impact on my day-to-day life. I would go to bed at 8pm and wake up the next morning feeling like I hadn’t slept in days. I was taking time off work just to sleep…I went to my GP repeatedly for years about various symptoms…I never got any significant relief or diagnosis…There is a serious lack of knowledge in relation to Sjögren’s…Patients turn to the healthcare system to be looked after, not to act as educators and advocates…I see five different doctors to manage my different symptoms. It’s complicated, exhausting, and expensive…Had it been diagnosed earlier, it may not have progressed so far…There have been times when my joint pain has been so bad that I’ve been unable to tie my own shoes, hold a pen or climb the stairs…I use eye drops 30 times a day and am limited to one hour of computer use a day…Half the battle with Sjögren’s is getting diagnosed at all.”

— Extract from article published in the Irish Times (a national Irish newspaper) featuring Deirdre Collins (co- applicant) in 2020. 

Sjögren’s can have a considerable impact on the quality of life of those affected, often impairing their ability to carry out daily activities and negatively affecting their work and social lives (Ng et al 2016). The reduction in quality of life is similar to that seen in other chronic condition such as lupus and rheumatoid arthritis (Miyamoto et al, 2021). 

Management of Sjögren’s is costly, and although we have no figures available for Ireland, international research has estimated the average annual direct cost ranging from £2,200 (UK) to $20,000 (US) (Ramos-Casals et al 2020).

Key Terminology

Patient communities around the world have highlighted the impact language can have on the understanding of Sjögren’s disease. There is a movement away from the old use of “syndrome”, as this may give rise to incorrect perceptions of nuisance symptoms or a non-serious condition. We prefer to describe Sjögren’s as a disease, as this more accurately describes it’s serious and systemic nature. We use ‘Sjögren’s’ here because it was identified by our PPI contributors as the preferred term. 

Similarly, we don’t use the primary/secondary classification as it may give the mistaken idea that some autoimmune diseases are more important than others (Baer & Hammitt, 2021; Kollert & Fisher 2020). Instead, we use the term “overlap conditions” to describe when someone has both Sjögren’s and other autoimmune diseases. 

Disclaimer This website is for education and information purposes only and does not substitute for medical diagnosis and treatment. Please consult with your clinician about your medical care.