Sjorgens Syndrome

Question 1

What is Sjorgens syndrom

Answer 1

autoimmune condition that affects the exocrine glands.

Sjögren’s syndrome is a chronic autoimmune disease characterised by reduced lacrimal and salivary gland function.

Question 2

Key symptoms of Sjorgens

Answer 2

dry mucous membranes, such as dry mouth, dry eyes and dry vagina.

Question 3

What is primary Sjorgens syndrome?

Answer 3

Primary Sjogren’s is where the condition occurs in isolation.

Question 4

What is secondary Sjorgens

Answer 4

here it occurs related to SLE or rheumatoid arthritis or systemic slcerosis

Question 5

Schirmer test (gold standard)

Answer 5

volves 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.

Question 6

Management

Answer 6

• Artificial tears
• Artificial saliva
• Vaginal lubricants
• Hydroxychloroquine is used to halt the progression of the disease.

Question 7

Complications of Sjorgens

Answer 7

• 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

Question 8

Disease associated with Sjorgens

Answer 8

Pneumonia and bronciectasis
Non-Hodgkins lymphoma
Peripheral neuropathy
Vasculitis
Renal impairment

Question 9

Terms in SS

Answer 9

Sicca syndrome: old term that is synonymous with SS.
Keratoconjunctivitis sicca: refers to the dry eye symptoms experienced in SS
Xerostomia: refers to dry mouth

Question 10

Epidemiology

Answer 10

SS is found worldwide but the true incidence and prevalence of the condition varies, but is estimated between 0.1-4% depending on the diagnostic criteria used. Importantly, the majority of patients with dry eyes and mouth (i.e. Sicca symptoms), especially older adults, do not have underlying SS.

Primary SS
Primary SS is most commonly seen in females (9-20:1 female to male ratio) in the 4th or 5th decade of life.

Secondary SS
Secondary SS is commonly found in association with SLE, rheumatoid arthritis and systemic sclerosis. The epidemiological data of secondary SS varies widely depending on the underlying condition.

Question 11

What other autoimmune diseases are SS found to be in assosciation with?

Answer 11

Autoimmune hepatitis
Primary biliary cholangitis
Autoimmune thyroiditis (hypothyroidism)
Graves’ disease (hyperthyroidism)
Antiphospholipid syndrome

Question 12

Which glands are exocrine and produce tears

Answer 12

Lacrimal
Sensory innervation via sensory branch of trigeminal nerve

Question 13

Parotid glands

Answer 13

• largest salivary gland
• Located inferior to the zygomatic arch in the pre-auricular region.
• Drains into the oral cavity via the parotid duct of Stensen.
• Innervated by the IX cranial nerve (glossopharyngeal nerve).

Question 14

Submandibular Glands

Answer 14

• 2nd largest salivary gland
• Submandibular triangle of neck
• Drains into oral cavity by Whartons duct
• Cranial Nerve 7 (Facial nerve)

Question 15

Sublingual glands

Answer 15

• Smallest salivary gland
• located in sublingual folds under membrane covering floor of mouth
• Innervated by cranial nerve 7

Question 16

Minor salivary glands

Answer 16

There are hundreds of minor salivary glands located throughout the oral cavity. These may have their own secretory duct or form a common duct with another gland. Any of these glands may be affected in SS.

Question 17

Aetiology

Answer 17

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.

Question 18

Pathophysiology

Answer 18

• 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.

Question 19

Autoantibodies

Answer 19

• 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

Question 20

Primary SS autoantibodies

Answer 20

60-80% of patients with primary SS with have one or both of Anti-Ro and Anti-La autoantibodies.

Question 21

Secondary SS

Answer 21

5% will have evidence of these autoantibodies. Anti-Ro may be found in 50% of patients with SLE and some healthy patients, therefore, it cannot be used alone to diagnose SS.

Question 22

Clinical features

Answer 22

keratoconjunctivitis sicca (dry eyes) and xerostomia (dry mouth). In addition, up to 50% of patients may have salivary gland enlargement.

Question 23

Lymphoma and SS

Answer 23

Lymphoma may develop in 2-5% of patients with SS. This is typically a non-Hodgkin’s lymphoma (NHL). Tumours may arise from exocrine glands, lymph nodes, or mucosa-associated lymphoid tissue (MALT). The following clinical features may suggest a lymphoproliferative disorder.

Question 24

What are B symptoms

Answer 24

fever, night sweats, weight loss

Question 25

Symptoms of lymphoma

Answer 25

• Persistent unilateral or bilateral gland swelling
• Hard, nodular gland
• New enlarged lymph node

Question 26

Extranglandular manifestations

Answer 26

Skin - abnormal dryness, prupura
MSK - myopathy, arthralgia
Pulmonary - ILD
CVD - pericarditis, heart block
Renal
Coeliac
Depression

Question 27

Tests

Answer 27

Urinalysis: assess for evidence of nephritis
Saliva gland function (see whole sialometry)
Lacrimal gland function (see Schirmer test)
Full blood count
ESR & CRP
Urea & electrolytes
Liver function tests

Question 28

Autoantibodies found

Answer 28

ANA
Extractable nuclear antigens (ENAs): this will include anti-Ro and anti-La autoantibodies
Anti-CCP (if rheumatoid arthritis suspected)
Anti-dsDNA (if SLE suspected): usually requested separately to ENAs
Rheumatoid factor
Complement
Immunoglobulins