Sjögren Syndrome

Question 1

What is Sjögren Syndrome?

Answer 1

It is a chronic autoimmune disorder of unknown etiology characterized by lymphocytic infiltration & destruction of exocrine glands

Question 2

Types of Sjögren Syndrome?

Answer 2

Primary: Occuring in the absence of other autoimmune disorders

Secondary: Occuring in the presence of other autoimmune disorders e.g SLE, HIV, RA

Question 3

What genes are associated with Sjögren Syndrome?

Answer 3

HLA DR & HLA DQ

Question 4

What viral infections might predispose to Sjögren Syndrome?

Answer 4

EBV
Retroviruses
HCV

Question 5

What are the Glandular clinical features of Sjögren Syndrome?

Answer 5

Dry mouth (xerostomia)
Dry Eyes (xerophthalmia)
Vaginal dryness
Dry skin
Dryness of upper airway

Question 6

What are the Extra-glandular clinical features of Sjögren Syndrome?

Answer 6

Rheumatoid Arthritis
Interstitial lung disease
Pericardial Effusion
Purpura
Raynaud’s phenomenom

Question 7

Triad of Sjögren Syndrome

Answer 7

Dry mouth
Dry Eyes
Rheumatoid Arthritis

Question 8

Patients with Sjögren Syndrome are at increased risk of what cancer?

Answer 8

Non-hodgkin Lymphoma

Question 9

What Serological investigations are done in Sjögren Syndrome?

Answer 9

ANA
RF
Anti-SSA/RO
Anti-SSB/LA

Question 10

What is the Schirmer’s test?

Answer 10

The Schirmer test determines whether the eye produces enough tears to keep it moist.

The eye doctor will place the end of a strip of filter paper inside the lower eyelid of each eye. Both eyes are tested at the same time. Before the test, you will be given numbing eye drops to prevent your eyes from tearing due to irritation from the paper strips.

Question 11

List the ACR/EULAR Classification Criteria for Primary Sjögren Syndrome

Answer 11

🎀Labial salivary gland biopsy with focal lymphocytic sialadenitis and focus score ≥1 (per 4 mm²)—— 3

🎀Anti-SSA/Ro antibody positivity— 3

🎀Ocular staining score ≥5 (or van Bijsterveld score ≥4) in at least one eye— 1

🎀Schirmer’s test ≤5 mm/5 min in at least one eye— 1

🎀Unstimulated whole saliva flow rate ≤0.1 mL/min— 1

Question 12

What Score in the ACR/EULAR classifies a patient who meets the inclusion criteria?

Answer 12

> / = 4

Question 13

Treatment of Sjögren Syndrome

Answer 13

Treat infections (Salivary & Vaginal)

Hydroxychloroquine for Arthralgia

Steroids for Interstitial Lung disease

Nystatin/Ketocinazole for Chronic erythematous candidiasis