Sjogren's syndrome Flashcards
What is Sjogren’s syndrome?
Sjögren’s syndrome is an autoimmune condition in which there is lymphocytic infiltration of exocrine glands, producing the main symptoms of xerophthalmia (dry eyes), xerostomia (dry mouth) and enlargement of the parotid glands.
What is the classification of Sjogren’s syndrome?
The disease is called primary if it develops in isolation and secondary if it occurs with other autoimmune diseases - usually rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) or scleroderma.
What are the risk factors for Sjogren’s syndrome?
Prevalence rises with age but age of onset is usually in the 30s or 40s.
Rarely, it can occur in childhood. It is up to 20 times more common in women than in men, and men who have the condition tend to be less severely affected by it.
There is a genetic predisposition
Vit. D deficiency is common in patient with Sjogren’s syndrome.
What is the presentation of Sjogren’s syndrome?
Dry mouth
Dry eyes
Recurrent parotitis (usually bilateral)
Dryness of the mucosa of trachea and bronchi may present as dry cough
Dysphagia due to dryness of the pharynx and the oesophagus.
Disease of the pancreas can lead to malabsorption
Dry skin and vaginal dryness causing dyspareunia
Purpura and annular erythema
Fatigue
Raynaud’s phenomenon (20%)
Signs:
-Dilation of conjunctival vessels and blepharitis
-Dry mouth and evidence of oral candidiasis and dental caries.
-Submandibular glands may be enlarged
How does dry mouth present?
Difficulty eating dry food, typically cracker biscuits.
Habit of having a glass of water by the bed at night.
Altered sense of taste.
Difficulty with dentures.
Complaint of the tongue sticking to the roof of the mouth.
Speaking for long periods of time causes hoarseness.
Dental and periodontal problems.
Oral candidiasis and angular cheilitis.
How can dry eyes present?
A gritty sensation
Predisposition to blepharitis
What are the associated diseases of Sjogren’s syndrome?
There may be a number of associated autoimmune conditions, such as the variant of scleroderma CREST: calcinosis, Raynaud’s phenomenon, (o)esophageal motility disorder, sclerodactyly and telangiectasia.
There may be joint pain, swelling and fatigue or recurrent miscarriage with antiphospholipid syndrome.
What are the differentials of Sjogren’s syndrome?
Amyloidosis Bulimia Chronic pancreatitis GvHD Polymyositis RA Scleroderma SLE Sarcoidosis Salivary gland tumours
What are the investigations for Sjogren’s syndrome?
FBC is usually normal, although anaemia of chronic disease may be a feature. Abnormal white cell count may suggest a lymphoma. ESR may be raised but is nonspecific.
Rheumatoid factor is more often positive in Sjögren’s syndrome than it is in rheumatoid disease.
Antinuclear antibodies are often positive - even without SLE - and there may be positive antiphospholipid antibodies.
Antibodies against alpha-fodrin and Ro/La autoantigens (antigens related to cell membranes) may be raised in non-Sjögren’s sicca syndrome.
Schirmer test to test for dry eyes.
Imaging of the salivary glands.
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.
Creatinine clearance may reduce in up to 50% of patients.
CT scan should be carried out if it is suspected that lymphoma is developing.
MRI scan of the salivary glands may help to identify changes associated with chronic sialadenitis.
How do you diagnose Sjogren’s syndrome?
The diagnosis is based on clinical suspicion then autoimmune screening tests may then reveal a positive rheumatoid factor and/or antinuclear antibodies and the patient is then appropriately referred to a rheumatologist.
What is the management of Sjogren’s syndrome?
Scoring systems are used to evaluate the severity of the condition. There are several scoring systems developed by the European League Against Rheumatism (EULAR) to assess the severity of symptoms and also of systemic involvement
Eyes:
- Artificial tears should be applied liberally
- Severe dryness may require special glasses to improve the humidification of the eyes or temporary or permanent blockage of the puncta (thus preventing tear drainage) by electrocautery or other means
Mouth:
- Drink plenty of water.
- Pilocarpine for salivary production
- Artificial saliva
Vaginal lubricants may be required and infections such as vaginal candidiasis are more likely. Dry skin may benefit from emollients. Hydroxychloroquine may be useful in suppressing arthralgia and skin symptoms.
What are the complications of Sjogren’s syndrome?
Infections of the eyes and mouth
Disease in sites other than glands such as skin vasculitis, peripheral neuropathy and glomerulonephritis.
