Sjorgren's Sydrome

Overview

Sjögren's syndrome is an autoimmune disorder. The body’s immune system attacks glands that secrete fluid, such as the tear and salivary glands.

The effects of Sjögren's syndrome can be widespread. Certain glands become inflamed, which reduces the production of tears and saliva, causing the main symptoms of Sjögren's syndrome, which are dry eyes and dry mouth (1)

Symptoms

The main symptoms are dry eye (keratoconjunctivitis sicca) and dry mouth (xerostomia) and resulting complications. Please see a full list of symptoms and their sequelae that can be associated with Sjorgren's:

Sjögren's syndrome - Symptoms| NHS inform

Diagnosis

Sjögren's syndrome can be difficult to diagnose because the symptoms are similar to those of other health conditions.

Before proceeding to investigations consider other causes for dry eyes and mouth.

• Aging – tear and unstimulated saliva production decline with age due to age-related histological alterations in the lacrimal and salivary glands.
• Medications – Multiple medications are well recognised to cause sicca syndrome including, anti-cholinergic drugs, sympathomimetic drugs, benzodiazepines, SSRIs, tricyclic antidepressants, phenothiazines, antihistamines, nicotine, opioids, α-1 antagonists, α-2 antagonists, β-blockers and diuretics.

Reassuringly patients with symptoms secondary to age or medications typically lack histological changes seen in Sjogren’s Syndrome and anti-SSA/Ro and anti-SSB/La are generally negative

History

If the above causes of symptoms have been considered and a patients answers "yes" to most of the questions below, they may have Sjögren's syndrome and further tests should be considered:

• Have you had daily, persistent, troublesome dry eyes for more than three months?
• Do you keep having a sensation of grit in your eye?
• Do you need to use eye drops containing tear substitutes more than three times a day?
• Have you had a daily feeling of dry mouth for more than three months?
• Do you keep getting swollen salivary glands (located between your jaw and your ears)?
• Do you frequently drink liquids to help you swallow food?

Investigations

Bloods

• FBC  - is usually normal, although anaemia of chronic disease may be a feature. Abnormal white cell count may suggest a lymphoma.
• Inflammatory markers - such as CRP or ESR may be raised but are nonspecific.
• Rheumatoid factor - is more often positive in Sjögren's syndrome than it is in rheumatoid disease.
• Antinuclear antibodies (ANA)-  are often positive even without SLE. There may be positive antiphospholipid antibodies. (To request: In NBT ICE go to the Immunology tab - Autommunity tests - Anti-Nuclear antibodies; In UHB ICE go to Haem and Immuno - Autoimmune liver disease which includes ANA).
• [Autoantibodies - the best-described autoantibodies in primary Sjögren's syndrome are the anti-Ro and anti-La antibodies. About two thirds of patients with primary Sjögren's syndrome have anti-Ro antibodies and/or anti-La antibodies. However, these tests cannot be requested directly by GPs and will only be done dependent on result of the ANA]

Schirmer test

A bent piece of filter paper is placed into the lower conjunctiva and left there for five minutes. In normal people the paper will be wet to 15 mm or beyond after five minutes, whereas a definitive positive result is less than 5 mm after five minutes. This test can be useful to help exclude or confirm significant dryness of the eyes but it is not specific for the disease.

Imaging

USS of the salivary glands can show inflammation and support a diagnosis.

Biopsy

In cases of uncertainty, biopsy of a salivary gland may be required. Usually one of the minor glands from the inner lip is preferred to a parotid. Histology will reveal gland infiltration.

Management

Haematology - USC (2WW)There is no cure for Sjögren's syndrome, but treatments help relieve symptoms such as eye and mouth dryness.

Patient support groups such as British Sjorgren's Syndrome Association (BSSA) can be helpful.

Patients with mild symptoms can often be managed in primary care and referral should be reserved for patients with more severe symptoms or complications not responding to these treatments.

Eye Care - see also the Dry Eye page

Mild to moderate cases of dry eye can usually be successfully treated with eye drops containing artificial tears – a liquid that mimics tears. These eye drops are available from a pharmacist, without a prescription.

Please see the BNSSG formulary for occular lubricants.

Special types of spectacles can be provided by local opticians.

If there are complications from dry eye then consider referral to Opthalmology cornea clinic via eRS. Topical steroids can be used for short periods but usually only on the advice of a specialist.

Mouth Care

Patients should be directed towards self care and oral hygiene which can also be supported by their dentist.

Saliva substitutes are available as a spray, lozenge, gel, or gum. See the BNSSG formulary for treatments of dry mouth.

Smokers should make all efforts to quit - smoking irritates the mouth and increases the rate at which saliva evaporates.

Complications such as tooth decay should be managed by a general dental practitioner who can refer on the the dental hospital if necessary.

Systemic or Joint symptoms

Referral to a rheumatologist via eRS should be considered for patients with symptoms or signs of systemic disease such as inflammatory arthritis or for consideration of systemic treatments such as pilocarpine or hydroxychloroquine.

Persistent Severe Symptoms

If patients continue to struggle with persistent severe symptoms despite appropriate mouth care/eye care in the community, then consider referral via eRS as appropriate (see Referral section below)

Red Flag

Non- Hodgkins Lymphoma

Patients with Sjorgren's syndrome are at in increased risk of non-Hodgkin's lymphoma. Consider Haematology 2WW referral if this is suspected

Pregnancy

In rare cases Sjorgren's can be associated with heart defects in babies so referral for pre-pregnancy antibody testing should be considered.

Referral

If diagnostic uncertainty or interpretation of investigations is required then consider requesting Rheumatology Advice and Guidance via eRS.

If associated systemic or rheumatological symptoms are present then refer via eRS to Rheumatology.

For severe localised manifestations then consider appropriate specialist referral via eRS. For example:

• Opthalmology (cornea) for complications of dry eye.
• Max Fax for complications of dry mouth or consideration of biopsy.

Resources

(1) Sjögren's syndrome - Illnesses & conditions | NHS inform

(2) Sjögren's Syndrome (Causes, Symptoms, and Treatment) | Patient

(3) British Society for Rheumatology guideline for the management of adults with primary Sjögren’s Syndrome | Rheumatology | Oxford Academic (oup.com)

(4) Sjögren's Syndrome Symptoms | Sjogrens Treatment & Information | Dry Eyes, Dry Mouth, Sore Joints, Autoimmune (bssa.uk.net)

Efforts are made to ensure the accuracy and agreement of these guidelines, including any content uploaded, referred to or linked to from the system. However, BNSSG ICB cannot guarantee this. This guidance does not override the individual responsibility of healthcare professionals to make decisions appropriate to the circumstances of the individual patient, in consultation with the patient and/or guardian or carer, in accordance with the mental capacity act, and informed by the summary of product characteristics of any drugs they are considering. Practitioners are required to perform their duties in accordance with the law and their regulators and nothing in this guidance should be interpreted in a way that would be inconsistent with compliance with those duties.

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