5: Connective tissue diseases 2: electric boogaloo

Question 1

What is the main symptom of connective tissue disease?

Answer 1

Arthralgia / arthritis

Question 2

People with connective tissue diseases may be extremely ___.

Answer 2

tired

Question 3

People with connective tissue diseases may experience muscle ___.

Answer 3

weakness , pain

Question 4

What does sicca mean?

Answer 4

Dryness

Question 5

What sort of sicca symptoms do people with CTDs get?

Answer 5

Dry mouth

Crusty eyes

Question 6

What hand phenomenon is seen in connective tissue diseases?

Answer 6

Raynaud’s

Question 7

What is the medical name for hair loss?

Answer 7

Alopecia

Question 8

Connective tissue diseases often cause mouth ___.

Answer 8

ulcers

Question 9

Connective tissue diseases are more commonly seen in (men / women).

Answer 9

women

Question 10

What is the difference between primary and secondary symptoms?

Answer 10

Primary - no cause, appears by itself

Secondary - related to other disease

Question 11

Which hormone is associated with lupus?

Answer 11

Oestrogen

Question 12

Name six connective tissue diseases.

Answer 12

SLE

Anti-phospholipid syndrome

Sjogren’s syndrome

Polymyositis

Systemic sclerosis

Mixed CTD

Question 13

What are the characteristic events of anti-phospholipid syndrome?

Answer 13

Arterial / venous thrombosis > embolism

Miscarriage

Question 14

APS may be primary or secondary to ___.

Answer 14

lupus

Question 15

What are the three antiphospholipid antibodies tested for to diagnose APS?

Answer 15

Anti-cardiolipin

Lupus anticoagulant

Beta-2 glycoprotein

Question 16

What are the criteria for diagnosing APS?

Answer 16

Antibody elevated on 2 occasions at least 12 weeks apart

Question 17

Unexplained arterial/venous events in young patients should make you suspicious of what?

Answer 17

Anti-phospholipid syndrome

Question 18

What is levido reticularis?

What is it caused by?

Which condition should be tested for?

Answer 18

Criss-crossy redness on arms

Thrombosis in small blood vessels

Anti-phospholipid syndrome

Question 19

How long are APS patients put on anticoagulants for?

Answer 19

Forever

life

Question 20

Can warfarin be given to pregnant women?

Answer 20

No, teratogenic

Question 21

What anticoagulants are given to pregnant women?

Answer 21

Aspirin and fragmin

Question 22

Which anti-malarial drug is given to patients with APS?

Answer 22

Hydroxychloroquine (TNF alpha inhibitor)

Question 23

What is Sjogren’s syndrome?

Answer 23

Autoimmune - chronic inflammation of lacrimal and salivary glands

Question 24

What are the main symptoms of Sjogren’s syndrome?

Answer 24

Dry eyes

Dry mouth

Arthritis / arthralgia

Fatigue

Question 25

What kind of cancer can occur as a result of increased B cell activity in CTDs?

Answer 25

**Lymphoma**

Question 26

Why do people with Sjogren's syndrome tend to have poor dentition?

Answer 26

**Salivary glands are crap** **Saliva contains proteolytic enzymes which kill bacteria**

Question 27

People with Sjogren's syndrome tend to get corneal \_\_\_.

Answer 27

**ulcers**

Question 28

What is Sherman's test for Sjogren's syndrome?

Answer 28

**Filter paper put in eye, rheumatologist assesses how wet it is**

Question 29

An ANA titre higher than what is suspicious?

Answer 29

**1:160**

Question 30

Which autoantibodies are seen in Sjogren's syndrome?

Answer 30

**Anti Ro** **Anti La**

Question 31

What is the gold standard investigation for Sjogren's syndrome?

Answer 31

**Salivary gland biopsy**

Question 32

How is Sjogren's syndrome treated?

Answer 32

**Artificial tear supplements** **Saliva supplements** **Ciclosporin eye drops** (immunosuppressant) **Hydroxychloroquine** (first drug used in almost everything) **Immunosuppressants**

Question 33

Sjogren's patients are advised to keep good ___ \_\_\_.

Answer 33

**dental hygiene**

Question 34

What does sclerosis mean?

Answer 34

**Hardening**

Question 35

What is the main presenting symptom of **systemic sclerosis**?

Answer 35

**Raynaud's**

Question 36

What happens in systemic sclerosis?

Answer 36

**Autoimmune disease** - vascular endothelial changes, fibrosis

Question 37

What is diffuse cutaneous systemic sclerosis?

Answer 37

**Skin involvement PROXIMAL to forearms, torso**

Question 38

What is "limited" systemic sclerosis compared to diffuse cutaneous systemic sclerosis?

Answer 38

**Skin DISTAL to elbows involved, no torso**

Question 39

What are the CREST signs of systemic sclerosis?

Answer 39

**Calcinosis** **Raynaud's** **Oesophageal/gut dysmotility** **Sclerodactyly** **Telangectasia**

Question 40

What is thickening of the skin called?

Answer 40

**Scleroderma**

Question 41

What autoantibodies are seen in a) **diffuse systemic sclerosis** b) **limited systemic sclerosis**?

Answer 41

**a) Anti-Scl70** **b) Anti-centromere antibody**

Question 42

Which autoantibodies are associated with a) **limited systemic sclerosis** b) **diffuse systemic sclerosis**?

Answer 42

**a) Anti-centromere antibody** **b) Anti-Scl70 antibody**