Connective Tissue Disease

Question 1

What are connective tissue diseases?

Answer 1

diseases with spontaneous over activity of the immune system

Question 2

Which condition is often known as the great imitator?

Answer 2

SLE

Question 3

Who does SLE affect more, M or F?

Answer 3

F

Question 4

What causes SLE?

Answer 4

• Immune complexes deposited in wall of blood vessel
• presence of immune complexes activates complement and attracts inflammatory cells eg neutrophils
• enzymes released from neutrophils cause damage to endothelial cells of basement membrane

Question 5

What cutaneous features occur in SLE?

Answer 5

photosensitivity
malar rash
discoid lupus erythematosus

Question 6

What Jaccoud’s arthritis?

Answer 6

deforming arthropathy present in SLE

Question 7

What endocarditis may be present in SLE?

Answer 7

Libman Sach

Question 8

What haematological features are there of SLE?

Answer 8

lymphadenopathy
leucopenia
anaemia
thrombocytopenia

Question 9

What tests should be done for suspected SLE?

Answer 9

FBC
ANA
dsDNA
ENA
complement levels

Question 10

In how many SLE patients is ANA positive?

Answer 10

95%

Question 11

When should an ANA test be taken seriously?

Answer 11

if other antinuclear antibodies are positive

Question 12

How often is anti double stranded DNA antibody positive for patients with SLE?

Answer 12

60%

Question 13

Which antibody is highly specific for SLE?

Answer 13

anti double stranded DNA antibody

Question 14

What else may anti double stranded DNA antibody be associated with?

Answer 14

lupus nephritis

Question 15

Drug treatment for moderate SLE?

Answer 15

Moderate disease: oral steroids, azatrioprine, methotrexate

Question 16

Drug treatment for mild SLE?

Answer 16

NSAIDs + hydroxychloroquine for maintenance

Topiccal steroids for skin

Question 17

Drug treatment for severe/acute SLE?

Answer 17

Acute flare up: IV Cyclophosphamide + High dose prednisolone

Rituximab

Question 18

What is arterial/venous thrombosis, pregnancy loss associated with?

Answer 18

Anti-phospholipid syndrome

Question 19

What is responsible for 15% of cases of recurrent foetal loss and 20% of recurrent thrombosis in young people?

Answer 19

anti phospholipid syndome

Question 20

Treatment of Anti-phospholipid syndrome?

Answer 20

thrombosis (life long anticoagulation)
pregnancy loss - aspirin and heparin during pregnancy
attention to vascular risk factors

Question 21

What does Sjogren’s syndrome involve?

Answer 21

lymph infiltration of exocrine glands, causing xerostomia and keratoconjunctivitis sicca

Question 22

What are some manifestations of Sjogren’s?

Answer 22

fatigue, arthralgia, dry mouth/skin/vagina, neonatal complete heart block, Raynauds, salivary swelling

Question 23

Treatment for Sjogren’s?

Answer 23

eye drops, punctual plugs
saliva replacement
pilocarpine
hydroxychloroquine
Steroids and immunosupression

Question 24

What is systemic sclerosis?

Answer 24

vasculopathy (raynauds)

fibrosis, can be localized or systemic

Question 25

What does the acronym CREST stad for in systemic sclerosis?

Answer 25

calcinosis, Raynauds, esophageal dysmotility, sclerodactylyl, telangiectasia

Question 26

Treatments for sclerosis?

Answer 26

calcium channel blockers
Prostacyclin
ACE Is
Prednisolone
Immunosuppression
Bosenta, Sildenafil

Question 27

What is MCTD?

Answer 27

mixed connective tissue disease

Question 28

What antibody is used for MCTD?

Answer 28

anti-RNP