sjorjens syndrome Flashcards
What is Sjorgens syndrom
autoimmune condition that affects the exocrine glands.
Sjögren’s syndrome is a chronic autoimmune disease characterised by reduced lacrimal and salivary gland function.
Key symptoms of Sjorgens
dry mucous membranes, such as dry mouth, dry eyes and dry vagina.
What is primary Sjorgens syndrome?
Primary Sjogren’s is where the condition occurs in isolation.
What is secondary Sjorgens
here it occurs related to SLE or rheumatoid arthritis or systemic slcerosis
Schirmer test (gold standard)
Involves inserting a folded piece of filter paper under the lower eyelid with a strip hanging out over the eyelid. This is left in for 5 minutes and the distance along the strip hanging out that becomes moist is measured. The tears should travel 15mm in a healthy young adult. A result of less than 10mm is significant.
Management
Artificial tears
Artificial saliva
Vaginal lubricants
Hydroxychloroquine is used to halt the progression of the disease.
Complications of Sjorgens
Eye infections such as conjunctivitis and corneal ulcers
Oral problems such as dental cavities and candida infections
Vaginal problems such as candidiasis and sexual dysfunction
Complications of ss
Pneumonia and bronciectasis
Non-Hodgkins lymphoma
Peripheral neuropathy
Vasculitis
Renal impairment
What other autoimmune diseases are SS found to be in assosciation with?
Autoimmune hepatitis
Primary biliary cholangitis
Autoimmune thyroiditis (hypothyroidism)
Graves’ disease (hyperthyroidism)
Antiphospholipid syndrome
Which glands are exocrine and produce tears
Lacrimal
Sensory innervation via sensory branch of trigeminal nerve
Major salivary glands
Parotid glands: largest salivary gland.
Submandibular glands: second largest salivary gland. Located in the submandibular triangle of the neck.
Sublingual glands: smallest salivary gland. Located within the sublingual folds that lie directly under the mucous membrane covering the floor of the mouth.).
Aetiology
Not understood
association with certain human leucocyte antigens (HLA), which are involved in antigen processing in the immune system. However, there is significant heterogeneity in risk among different ethnic groups.
gender, which is likely to relate to sex hormones, viral infectious triggers (e.g. Epstein-Barr virus) and other non-HLA genetic elements.
Pathophysiology
SS is characterised by the formation of autoantibodies anti-Ro/SSA and anti-La/SSB.
characterised by autoimmune lymphocytic infiltration (CD4 T cells and B plasma cells) of glandular tissue including lacrimal and salivary glands.
This infiltration of immune cells is accompanied by glandular and ductal atrophy.
In addition, there is suspected to be glandular dysfunction with altered release of acetylcholine and response to neural stimulation.
Autoantibodies
Anti-nuclear antibody (ANA): 90% of patients with SS.
Rheumatoid factor: autoantibody directed against Fc portion of IgG. Seen in many rheumatological conditions. In 40-60% of SS patients.
Anti-Ro/SSA
Anti-La/SSB
Primary SS autoantibodies
60-80% of patients with primary SS with have one or both of Anti-Ro and Anti-La autoantibodies.
