Sjogren syndrome

Question 1

What is Sjogren syndrome?

Answer 1

It is a chronic
multisystem autoimmune disease
characterized by chronic inflammation
involving the exocrine glands

Question 2

What are the glands involved in sjogren?

Answer 2

Salivary (dry mouth)
Lacrimal glands (dry eyes)

Question 3

Primary sjogren syndrome?

Answer 3

occur alone

Question 4

Secondary sjogren syndrome

Answer 4

May coexist with other systemic connective tissue disorders

Question 5

What are the extraglandular manifestations of primary sjogren syndrome?

Answer 5

1. fatigue
2. raynaud phenomenon
3. purpura
4. arthritis
5. vasculitis
6. interstitial pulmonary disease
7. periopheral or central neuropathy
8. autonomic nervous dysfunction

Question 6

Who are at higher risk of developing lymphoma?

Answer 6

patients with systemic manifestations

Question 7

Both INNATE and ADAPTIVE immunity contribute to pSS pathogenesis and pSS is associated with upregulation of the Type 1 interferon signaling pathway

Answer 7

primary sjogren syndrome is associated with upregulation of the Type 1 interferon signaling pathway

Question 8

Sjogren syndrome is characterized by?

Answer 8

Lymphocytic infiltration 
and 
destruction of exocrine glands and epithelia leading to 
1.dry mouth
2.dry eyes
3.B-lymphocyte hyperreactivity

Question 9

What are the other autoimmune conditions associated with Sjogren syndrome? (Secondary SS)

Answer 9

rheumatoid arthritis
SLE
other collagen disease

Question 10

What percentage of patients develop systemic manifestations in patients with Sjogren syndrome?

Answer 10

more than 1/3 of patients.

Question 11

A small but definitively number of patients of the pSS develop lymphoma with higher incidence than that of the general population or patients with other autoimmune diseases

Question 12

SS is one of the most common rheumatic autoimmune diseases

Answer 12

SS is one of the most common rheumatic autoimmune diseases

Question 13

SS predominantly affects what gender?

Answer 13

WOMEN (9:1 ratio)

Question 14

patients are most commonly diagnosed in their fourth and fifth decades of life but it can affect any age, including the elderly and children

Question 15

Both innate and adaptive immunity contribute to the development of pSS; the type 1 interferon signaling pathway plays a central role in the pathogenesis of the disease

Question 16

Increasing apoptosis in epithelial cells of damaged salivary glands, plasmacytoid dendritic cells, autoreactive lymphocytes and autoantibodies are considered to play significant roles in perpetuating the chronic auto-feedback inflammatory process.

Question 17

What is the key cytokine in the innate immune system that plays a role in primary sjogren syndrome?

Answer 17

Type 1 interferon