`

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

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