Sjogren's syndrome

Question 1

Sjogren’s syndrome

Answer 1

systemic autoimmune disorder
lymphocyte infiltration characterised by destruction of exocrine glands
keratoconjunctivitis sicca and xerostomia
primary Sjogren’s syndrome
secondary Sjogren’s syndrome

Question 2

Epidemiology

Answer 2

Common
9F: 1M
0.5-1.56%
60% secondary SS

Question 3

Aetiology

Answer 3

oestrogen/androgen deficiency

Question 4

Presentation

Answer 4

Glandular
- Reduced lacrimal gland function
- Dry itchy, burning, gritty eye
- Redness
- Sensitivity to light
- Swelling

Extra glandular
- Fatigue
- Musculoskeletal
- Vasculitis
- Renal
- Neuropathy

Question 5

Classification system

Answer 5

2017 ACR-EULAR Classification of Primary Sjogren’s Syndrome

Question 6

Focus score >1

Answer 6

Determined by number of mononuclear cell infiltrates containing >50 inflammatory cells per 4mm2 of minor labial salivary gland obtained on biopsy

Question 7

presence of anti-SSA antibodies

Answer 7

measured in serum, only anti-Ro60 antibodies have to be considered; isolated anti-Ro52 antibodies are not specific for SS

Question 8

SICCA Ocular staining score of >5

Answer 8

determined by an opthalmologist
fluorescein and lissamine green staining

Question 9

Schirmer test of <10mm per 5 min

Answer 9

An assay for measuring tear production by inserting filter paper on conjunctiva in the lower eyelid and assessing the amount of moisture on the paper

Question 10

Unstimulated salivary flow of <0.1ml per min

Answer 10

Assay measuring the rate of salivary flow by collecting saliva in a tube for at least 5 mins after patient has swallowed