Sjögren’s disease
Evidence: Antibody
Names
Sjögren’s disease
Sjögren's syndrome
Sicca syndrome (generally refers to the non-autoimmune form of this condition)
Keratoconjunctivitis sicca (a symptomatic diagnosis not specific to autoimmune disease)
Description
Sjögren’s disease is a systemic autoimmune disease that can affect many parts of the body. Traditionally, it was thought to be focused on the moisture-secreting glands of the eyes and mouth, but that has since been debunked. Its name was changed from Sjögren's syndrome to Sjögren's disease to reflect the systemic nature of the disease. Sjögren's patients are also predominantly female, with a female to male ratio of roughly 10 to 1.
Sjögren's can impact many different areas of the body, including the lungs, brain, gastrointestinal tract, sinus tract, nerves, joints, blood vessels, and kidneys. Symptoms vary significantly among patients, ranging from dry eyes and dry mouth to systemic fatigue, pain and brain fog. Depression and anxiety are also common comorbidities to the disease. Patients’ quality of life can be significantly impacted, with some evidence that fatigue and pain are the greatest predictors of poor quality of life.
Patients with Sjögren's can be diagnosed through positive antibodies (SSA or SSB), seropositive, or by diagnostic criteria without the presence of antibodies, seronegative. Seronegative patients report greater difficulty in obtaining a diagnosis and it is believed that, in general, Sjögren's disease is under-diagnosed.
Sjögren's disease is also often accompanied by other immune system disorders, such as rheumatoid arthritis and lupus. In such cases, physicians tend to refer to the patients as having Secondary Sjögren's, as opposed to Primary Sjögren's when patients only have one autoimmune disease. There has been a movement among patients and advocates to remove the label of “Secondary” as it trivializes Sjögren's as less important than the other autoimmune diseases.
Patient Groups
Bloggers
Prevalence
US Cases: 480,000 - 1,376,000 [Narváez 2020] for Primary Sjogren's. Many patients with other rheumatic autoimmune disease have Secondary Sjogren’s.
Typical Age of onset
The average of diagnosis is around 45, although many patients report significant delays before obtaining a diagnosis.
Symptoms
Prolonged fatigue
Systemic pain / fibromyalgia
Brain fog / cognitive impairment
Dry mouth
Swollen salivary glands
Dry eyes
Gritty or burning sensation in the eyes
Persistent dry cough
Sinus inflammation / infections
Joint pain
Joint swelling
Joint stiffness
Neuropathy
Vasculitis
Raynaud’s syndrome
Rashes
Dry skin
Vaginal dryness / recurrent yeast infections
Acid reflux / gastritis
Shortness of breath
Dizziness
Irritable bowl symptoms
Depression
Anxiety
References
Famous People
American College of Rheumatology Johns Hopkins Sjögren's Center Negrini et al. (2022) Dias et al. (2021) Luppi et al. (2020) Miyamoto et al. (2019) Chang et al. (2019) Chang et al. (2016) Segal et al. (2013)
Venus Williams (tennis player) Halsey (singer)
July 23rd is World Sjögren's Day
Profile by Ingrid He
Updated Nov. 2022