Sjogren's Syndrome Flashcards
What is Sjogren’s Syndrome (SS)?
- chronic autoimmune disease characterised by reduced lacrimal and salivary gland function.
What are the other terms associated with SS?
- 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
What are the two classifications for SS?
- Primary: Sjögren’s syndrome in the absence of an underlying rheumatic disease
- Secondary: Sjögren’s syndrome in the presence of another systemic rheumatic disease
What rheumatic diseases are associated with secondary SS?
- Rheumatoid arthritis
- Systemic lupus erythematosus (SLE)
- Systemic sclerosis.
Other
- Autoimmune hepatitis
- Primary biliary cholangitis
- Autoimmune thyroiditis (hypothyroidism)
- Graves’ disease (hyperthyroidism)
- Antiphospholipid syndrome
What patient demographic does primary SS typically affect?
- F
- 40-50
Which glands are affected in SS?
- Lacrimal gland
- produce aqueous layer of tear film
- Salivary glands
- Parotid gland
- Submandibular gland
- Sublingual gland
What nerves innervate each of these glands?
*lacrimal, parotid, submandibular, sublingual
- 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
What autoantibodies are associated with SS?
- 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
What are the clinical features of SS?
- Dry eyes (keratoconjunctivitis sicca)
- Dry mouth (xerostomia)
- Lymphoma sx
- Extraglandular manifestations
What are the sx of dry eyes?
- Eye irritation
- Blurry vision
- Eye itching
- Photophobia
- Corneal erosions or ulceration
- Reduced tear production
What are the sx of dry mouth?
- 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
What are the eg of lymphoma sx?
- fever, night sweats, weight loss
- Persistent unilateral or bilateral gland swelling
- Hard, nodular gland
- New enlarged lymph node
What are the eg of extraglandular manifestations in SS?
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
What diagnostic criteria is used for SS?
- American-European Consensus Group’s (AECG) criteria
What does the AECG’s criterias include?
- 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
What other conditions can predispose patients to have dry eyes and dry mouth?
- Past head-and-neck irradiation
- Hepatitis C virus infection
- Acquired immunodeficiency syndrome
- Prior lymphoma
- Sarcoidosis
- Use of anticholinergic drugs
What Ix would you order for SS?
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
How would you mx SS?
*breifly describe
- Treat dry eyes
- Treat dry mouth
- Treat systemic disease
How would you treat dry eyes?
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
How would you treat dry mouth?
Non-severe dysfunction:
- sugar-free sweets, lozenges or sugar-free chewing gum
- muscarinic agonists - pilocarpine
Severe dysfunction
- artificial saliva
How would you tx systemic disease?
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).
What is the cx of SS?
- 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
