Sjögren’s syndrome is an autoimmune condition affecting the exocrine glands, notably the lacrimal and salivary glands, causing symptoms of dry mouth, eyes and vagina. Dry eyes and dry mouth can be called sicca symptoms.
It can also cause dry skin, joint pain and stiffness. It is more common in women and typically presents in middle age.
Primary Sjögren’s is where the condition occurs in isolation. Secondary Sjögren’s is where it occurs due to other diseases such as systemic lupus erythematosus and rheumatoid arthritis.
Antibodies associated with Sjögren’s are:
- Anti-SS-A antibodies (also called anti-Ro)
- Anti-SS-B antibodies (also called anti-La)
Schirmer Test
The Schirmer test involves inserting folded filter paper under the lower eyelid with the end hanging out. Moisture from the eye will travel by diffusion along the filter paper. After 5 minutes, the distance that the moisture travels along the filter paper is measured. In a healthy young adult, 15mm is expected. Less than 10mm is significant.
Management
The diagnosis is typically made based on the clinical features and presence of antibodies. Salivary gland biopsy may be used to confirm the diagnosis but is not usually necessary.
Management involves rheumatology and ophthalmology. Treatment options include:
- Artificial tears (e.g., polyvinyl alcohol eye drops during the day and carbomer gel at night)
- Artificial saliva
- Vaginal lubricants
- Pilocarpine (oral) can be used to stimulate tear and saliva production
- Hydroxychloroquine may be considered, mainly in patients with associated joint pain
Pilocarpine stimulates muscarinic receptors, stimulating the parasympathetic nerves and promoting salivary and lacrimal gland secretion.
Complications
Complications related to exocrine gland dysfunction include:
- Eye problems, such as keratoconjunctivitis sicca and corneal ulcers
- Oral problems, such as dental cavities and candida infections
- Vaginal problems, such as candida infection and sexual dysfunction
Sjögren’s can rarely affect other organs, causing complications such as:
- Pneumonia
- Bronchiectasis
- Non-Hodgkins lymphoma
- Peripheral neuropathy
- Vasculitis
- Renal impairment
Last updated August 2023