Sjogren's Syndrome

Question 1

What is Sjogren’s Syndrome (SS)?

Answer 1

• chronic autoimmune disease characterised by reduced lacrimal and salivary gland function.

Question 2

What are the other terms associated with SS?

Answer 2

• 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 3

What are the two classifications for SS?

Answer 3

• Primary: Sjögren’s syndrome in the absence of an underlying rheumatic disease
• Secondary: Sjögren’s syndrome in the presence of another systemic rheumatic disease

Question 4

What rheumatic diseases are associated with secondary SS?

Answer 4

• Rheumatoid arthritis
• Systemic lupus erythematosus (SLE)
• Systemic sclerosis.

Other

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

Question 5

What patient demographic does primary SS typically affect?

Answer 5

• F
• 40-50

Question 6

Which glands are affected in SS?

Answer 6

• Lacrimal gland
  
  • produce aqueous layer of tear film
• Salivary glands
  
  • Parotid gland
  • Submandibular gland
  • Sublingual gland

Question 7

What nerves innervate each of these glands?

*lacrimal, parotid, submandibular, sublingual

Answer 7

• Lacrimal
  
  • Sensory innervation: Trigeminal Nerve (Va)
  • Autonomic innervation: Pterygopalatine ganglion, which is supplied by branches of the facial nerve (VII)
• ​Parotid gland
  
  • Glossopharyngeal nerve (IX)
  • Stensen duct
• Submandibular
  
  • Facial nerve (VII)
  • Wharton’s duct
• Sublingual
  
  • Facial nerve (VII)
  • Rivinus duct

Question 8

What autoantibodies are associated with SS?

Answer 8

• 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 9

What are the clinical features of SS?

Answer 9

• Dry eyes (keratoconjunctivitis sicca)
• Dry mouth (xerostomia)
• Lymphoma sx
• Extraglandular manifestations

Question 10

What are the sx of dry eyes?

Answer 10

• Eye irritation
• Blurry vision
• Eye itching
• Photophobia
• Corneal erosions or ulceration
• Reduced tear production

Question 11

What are the sx of dry mouth?

Answer 11

• Dry mouth & lips
• Adherence of food to buccal surface
• Difficulty eating dry food
• Difficulty speaking for long periods
• Change in taste
• Absent sublingual salivary pooling
• Increased dental caries
• Oral infections

Question 13

What are the eg of lymphoma sx?

Answer 13

• fever, night sweats, weight loss
• Persistent unilateral or bilateral gland swelling
• Hard, nodular gland
• New enlarged lymph node

Question 14

What are the eg of extraglandular manifestations in SS?

Answer 14

Skin

• Abnormal dryness
• Purpura
• Raynaud phenomenon

Musculoskeletal

• Arthralgia
• Myopathy

Pulmonary

• Interstitial lung disease (ILD): usually manifests are shortness of breath and cough
• Cystic lung disease: usually seen on imaging

Cardiovascular

• Pericarditis
• Myocarditis
• Heart block

Renal

• Interstitial nephritis
• Renal tubular acidosis
• Interstitial cystitis

Gastrointestinal

• Dysphagia
• Coeliac disease
• Primary biliary cholangitis
• Autoimmune hepatitis

Neurological

• Peripheral neuropathy: seen in 10% of patients

Psychiatric

• Depression

Question 15

What diagnostic criteria is used for SS?

Answer 15

• American-European Consensus Group’s (AECG) criteria

Question 16

What does the AECG’s criterias include?

Answer 16

• Dry eye symptoms
• Dry myth symptoms
• Objective ocular dryness: defined as Schirmer’s test ≤5mm in 5 minutes
• Objective oral dryness: salivary gland hypofunction is confirmed by sialometry (rate of saliva production) or scintigraphy (low uptake suggesting hypofunction)
• Positive anti-Ro/La autoantibodies
• Typical salivary gland biopsy findings

*must meet 4/6 of the criteria

*no. 5&6 must be included in the 4 criterias

Question 17

What other conditions can predispose patients to have dry eyes and dry mouth?

Answer 17

• Past head-and-neck irradiation
• Hepatitis C virus infection
• Acquired immunodeficiency syndrome
• Prior lymphoma
• Sarcoidosis
• Use of anticholinergic drugs

Question 18

What Ix would you order for SS?

Answer 18

Bedside

• Urinalysis
• Sialometry
• Schirmer test

Blood test

• Full blood count
• ESR & CRP
• Urea & electrolytes
• Liver function tests

Autoimmune screen

• 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

Additional test

• Salivary gland biopsy
• Salivary gland imaging

Question 19

How would you mx SS?

*breifly describe

Answer 19

• Treat dry eyes
• Treat dry mouth
• Treat systemic disease

Question 20

How would you treat dry eyes?

Answer 20

Primary tx (twice daily)

• Hypromellose eye drops
• Carmellose eye drops 0.5-1.0%
• Sodium hyaluronate eye drops 0.1–0.18%

Secondary tx

• Topical NSAIDs or Corticosteroids
• Topical cyclosporin
• Serum tear drops

Question 21

How would you treat dry mouth?

Answer 21

Non-severe dysfunction:

• sugar-free sweets, lozenges or sugar-free chewing gum
• muscarinic agonists - pilocarpine

​Severe dysfunction

• artificial saliva

Question 22

How would you tx systemic disease?

Answer 22

Corticosteroids (First line)

Immunosuppressive agents (second line)

• Leflunomide (pyrimidine synthesis inhibitor)
• Methotrexate (Dihydrofolate reductase inhibitor)
• Azathioprine and Mycophenolate (Purine synthesis inhibitors)
• Cyclophosphamide (alkylating chemotherapy agent that crosslinks DNA and RNA)

Monoclonal antibodies (third line)

• rituximab (monoclonal antibody to CD-20).

Question 23

What is the cx of SS?

Answer 23

• Lymphoma (major cx)

Occular related

• Corneal ulcer
• Corneal perforation
• Ocular infections

Oral related

• Ulcers
• Candidiasis
• Caries/dental complications

Extraglandular

• Lymphoma (NHL)
• Severe leucopaenia
• Vasculitis
• Peripheral neuropathy
• ILD
• Perimyocarditis