Sjögren's patients face a significantly increased risk of B-cell non-Hodgkin lymphoma (NHL), particularly in the salivary glands (MALT lymphoma) and other extranodal sites, driven by chronic immune stimulation and B-cell proliferation. While NHL is the main malignancy, solid tumors like oral, breast, thyroid, and nasopharyngeal cancers have also been reported, though the risk for solid tumors is less clear and debated in some studies.
Patients with SS have an elevated risk of developing malignant neoplasms, particularly hematologic malignancies, with most being non-Hodgkin B-cell lymphoma. Other cancers, such as oral cancer, breast cancer, and thymoma, might also occur in patients with SS.
Non-Hodgkin's lymphoma (NHL) occurs in approximately 2.7 - 9.8% of SS patients, with a greater risk than the normal population (Table 1); such patients are also at an increased risk for other autoimmune diseases [4–24].
Infections (35.71%), malignancies (23.8%), and cardiovascular disease (7.14%) were the most common causes of death.
Most lymphomas in pSS are of B-cell lineage and of low- or intermediate-grade malignancy. Lymphoma mainly develops from marginal zone B cells, leading to marginal zone lymphoma and especially mucosa-associated lymphoid tissue (MALT) lymphoma (MALT-L) in 65% of the cases [8, 9].
However, if you have Sjögren's syndrome, you should be aware of the main early symptom of non-Hodgkin lymphoma, which is a painless swelling in a lymph node (gland) – usually in the neck, armpit or groin. Report any swollen lymph nodes to your GP.
Some autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus (SLE or lupus), Sjogren (Sjögren) disease, celiac disease (gluten-sensitive enteropathy), and others have been linked with an increased risk of NHL.
While many patients experience dry eyes, dry mouth, fatigue and joint pain, Sjögren's can also cause dysfunction of organs such as the kidneys, gastrointestinal system, blood vessels, lungs, liver, pancreas, and the central nervous system.
Sjögren's syndrome can cause some complications, including:
Start with five minutes of aerobic exercise daily (e.g. walking, biking, running, elliptical, treadmill) and increase the duration by an additional two-to-three minutes each month up to a maximum of 25 minutes daily. If you have a heart or lung condition, consult your doctor first.
The most common glands that may be noticeably swollen are the parotid (pa-rot`id) glands located on the sides of the cheeks. Other glands less commonly involved are located under the jaw.
Dry mouth.
Sjogren's (SHOW-grins) syndrome is a disorder of your immune system identified by its two most common symptoms — dry eyes and a dry mouth. The condition often accompanies other immune system disorders, such as rheumatoid arthritis and lupus.
Drag moisture into your skin by using products that contain chemicals such as urea, glycerin, lactic or similar “metabolic” or alpha-hydroxy acids, such as AmLactin® cream or Carmol® and Cerave (a dermatologist recommended body cream specifically for severely dry skin).
Final nomenclature consensus was reached as follows: The term Sjögren's Disease should replace Sjögren's Syndrome. Sjögren's without disease is an acceptable way to refer to the disease, especially once it's been cited as Sjögren's Disease. The acronym SjD should be used as an abbreviation for Sjögren's Disease.
It has been reported that more than 400 drugs can exacerbate the dryness symptoms of Sjögren's syndrome, including antihypertensives, diuretics, antidepressants, antiparkinson drugs, antipsychotics, antihistamines, centrally acting analgesics, and anticholinergics [6], [13], [14], [15].
Nipocalimab is the only investigational treatment to be granted Breakthrough Therapy Designation by the U.S. Food and Drug Administration (FDA) for SjD (November 2024). It also received Fast Track Designation by the U.S. FDA earlier this year in April 2025.
There may be times when your symptoms get worse (flare-ups) and times when they're better (remission). It's not known exactly why this happens, but it's thought that certain things, such as overdoing it and not resting enough, may trigger your symptoms or make them worse.
Sjögren's disease can cause numbness or burning of the face, called “trigeminal neuralgia.” Pain in the back of the throat, which may worsen while swallowing, is called “glossopharyngeal neuralgia.” Patients with trigeminal or glossopharyngeal neuralgia can have agonizing mouth and facial pain.
Sjögren's syndrome (SS) is a chronic systemic autoimmune disease, affecting predominantly the exocrine glands, a large array of systemic manifestations and high risk of lymphoma development.
How patients described their first signs of lymphoma
In particular, rheumatoid arthritis, systemic lupus erythematosus, and Sjögren's syndrome are associated with swollen lymph nodes. A lymph node biopsy may be used to confirm whether the swelling is a result of NHL.