Sjögren's syndrome (SS) is strongly associated with an increased risk of non-Hodgkin B-cell lymphoma (NHL), particularly salivary gland MALT (mucosa-associated lymphoid tissue) lymphoma, with some studies showing a 6- to 9-fold higher risk than the general population, though risks for other solid tumors (like thyroid, oral, breast) are less consistent but also observed in some populations.
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.
Clinical History
Dryness of the eyes (xerophthalmia) and mouth (xerostomia) almost always occurs in patients with Sjögren syndrome, though symptom severity ranges from very mild to debilitating.
Comorbidities: Immune System
A second autoimmune or autoinflammatory disease is present in about half of Sjogren's patients. These include rheumatological diseases such as rheumatoid arthritis or spondylitis, as well as other autoimmune and immune-mediated diseases.
Death (Mortality)
While most people with Sjogren's live a normal lifespan, an estimated 10% die from direct Sjogren's complications such as lung disease or lymphoma (3, 47).
Infections (35.71%), malignancies (23.8%), and cardiovascular disease (7.14%) were the most common causes of death.
Nipocalimab is the first and only investigational therapy granted U.S. FDA breakthrough therapy designation for the treatment of adults living with moderate-to-severe Sjögren's disease [news release]. Johnson & Johnson. 2024 Nov 11.
Inflammation can cause pneumonia, bronchitis or other problems in your lungs; lead to problems with kidney function; and cause hepatitis or cirrhosis in your liver. Lymph nodes. A small percentage of people with Sjogren's syndrome develop cancer of the lymph nodes (lymphoma).
Graves' disease and Hashimoto's disease are two very common thyroid disorders. Both are caused by dysfunction of the immune system (autoimmune condition), but each is different in numerous ways.
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.
Dry mouth.
The most common blood tests that point towards Sjogren's disease are a positive anti-nuclear antibody (ANA), anti-SSA (Ro) and SSB (La) antibodies, and rheumatoid factor (RF) tests. However, there are also patients with negative antibodies, where a salivary gland biopsy is needed to confirm the diagnosis.
The occurrence of non-Hodgkin's lymphoma (NHL) is the most serious complication of Sjögren's syndrome (SS). We performed a study of 16 NHLs occurring in patients with an underlying SS.
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).
Sjögren's syndrome rarely coexisted with MM, a chronic autoimmune disease affecting exocrine glands and presenting very unique diagnostic and management challenges to the physician.
In addition to dryness, you might have other symptoms, including: Joint pain. Muscle pain or weakness. Swollen lymph nodes.
Many of the autoimmune diseases that overlap with Sjögren's can cause the same symptoms. For example, overlapping diseases like lupus and rheumatoid arthritis can also cause fatigue or joint pain — symptoms that are common in Sjögren's. These similarities can make it challenging to diagnose overlapping diseases.
The "worst" autoimmune diseases are subjective but often ranked by severity, impact on life expectancy, and organ damage, with top contenders including Giant Cell Myocarditis (deadly heart inflammation), Vasculitis (blood vessel inflammation like GPA), Systemic Lupus Erythematosus (multi-organ attacks), Multiple Sclerosis (nervous system damage), and Type 1 Diabetes (pancreas destruction). These conditions can severely affect quality of life, cause permanent disability, and reduce lifespan if not managed effectively, though rare ones like Giant Cell Myocarditis are acutely fatal.
Although anyone can develop Hashimoto's disease, it's most common among middle-aged women. The primary treatment is thyroid hormone replacement. Hashimoto's disease is also known as Hashimoto's thyroiditis, chronic lymphocytic thyroiditis and chronic autoimmune thyroiditis.
In our perspective, Sjögren's is an under-researched disorder, a situation that is even worse for patients with so called “secondary” disease. This stands in sharp contrast to the potential impact of Sjögren's on quality of life, even when compared to other systemic rheumatological disorders.
SARS-CoV-2 may trigger the development or progression of autoimmune diseases, as evidenced by increased autoantibodies in patients and the presentation of cardinal symptoms of SjD.
Because your body has trouble producing saliva, you may experience difficulty swallowing when symptoms flare up. Your mouth may feel chalky, and you may have trouble swallowing food. Many people with Sjogren's develop cavities and oral infections.
The International Sjögren's community has officially changed the disease name Sjögren's Syndrome in favor of Sjögren's Disease to better indicate the serious and systemic nature of the disease. The term “secondary Sjögren's” was also discarded.
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.
Immunosuppressive agents (methotrexate, cyclosporine) may improve the symptoms of Sjogren's syndrome, although they do not alter the decreased lacrimal and salivary flow rates or the histologic markers of the disease.