SLE and CTD

Question 1

Where can SE affect?

Answer 1

Any part of the body

Question 2

Briefly, what occurs in SLE?

Answer 2

Immune syste attacks body resulting in inflam and tissue damage. Antibody-immune complexes precipitate and cause further immune response, Immune complexes get stuck in and damage the basement membrane of blood vessels.

Question 3

What aspects of genetic, enviromental and hormonal affect the pathogenesis of SLE?

Answer 3

Females > males 
urban > rural 
Increased incidence in asains, hispanics, afro-carribeans and afro-americans 
silica dust 
UV light 
EBV 
oestrogen exposure

Question 4

What are the cardinal features of SLE?

Answer 4

Fever
lethargy 
weight loss 
poor appetite 
malaise

Question 5

What mucocutaneous features are often present in SLE?

Answer 5

Photosensitivity 
malar rash with sparing of nasolabial fold 
mouth ulcers 
alopecia areata
Raynauds

Question 6

How can SLE present in the cardiovascular system?

Answer 6

Pleurisy 
Infections 
Pulmonary HTN 
Pulmonary infarct 
diffuse lung infiltration and fibrosis

Question 7

Why do patients with SLE require frequent urinalysis?

Answer 7

Renal disease from SLE presens with few symptoms very late on once the patient is in severe renal failure so do not want to miss this

Question 8

What haematological features can be present in SLE?

Answer 8

Lymphadenopathy
leucopenia
anaemia
thrombocytopenia

Question 9

How do you differentiate severe SLE from mild SLE?

Answer 9

Mild - skin rash, fatigue, mouth ulcers
Moderate - Sinusitis, joint swelling
Severe - Renal or neurological impairment

Question 10

What investigations are required for SLE?

Answer 10

FBC 
urinalysis 
ENA 
complement levels 
ANA 
Anti-dsDNA

Question 11

How useful are meauring autoantibodies in SLE?

Answer 11

Anti-nuclear antibody - gateway to CTD but not specific to SLE
Anti-dsDNA - highly specific to SLE and levels psitivelly correlate with disease activity

Question 12

In anti-phospholipid syndrome, what autoantibodies are positive?

Answer 12

Anti-cardiolipin (also + in 30% SLE and 5% of healthy population)
lupus anticoagulant activity

Question 13

What does anti-phospholipid syndrome cause?

Answer 13

Arterial/ venous thrombosis
Responsible for 15% of recurrent foetal loss
Responsible for 20% recurrent thrombosis in YP

Question 14

Does anti-phospholipid syndrome affect men more than women?

Answer 14

No

Question 15

What are other features of anti-phospholipid syndrome?

Answer 15

Superficial thrombophlebitis
mild/ moderate thrombocytopaenia
migrane
Libman Sachs endocarditis

Question 16

What treatment is required for anti-phospholipid syndrome?

Answer 16

Life-long anticoagulation
aspirin and heparin during pregnancy
attention to vascular risk factors

Question 17

Describe Sjogrens syndrome.

Answer 17

Lymphocyte infiltration of exocrine glands causing xerostomia (dry mouth) and keratoconjunctivitis sicca (dry eyes)

Question 18

Which auto-antibodies can be positive in Sjogren’s syndrome?

Answer 18

anti-Ro

anti-La

Question 19

Is Sjogren’s syndrome more common in men or women?

Answer 19

Women

Question 20

What treatment can be given for Sjogren’s syndrome?

Answer 20

eye drope 
punctal plugs 
saliva replacement 
Pilocarpine (cholinergic agonist) 
HCQ 
Steroids and immunosuppression 
Attend to CV risk factors and dental hygiene

Question 21

What auto-antibodies can be positive in systemic sclerosis?

Answer 21

anti-centromere antibodies

anti-Scl-70 antibodies

Question 22

How is systemic sclerosis classified?

Answer 22

Limited or diffuse

Question 23

What organs can systemic sclerosis involve?

Answer 23

skin 
kidneys 
gut 
muscle 
joints 
heart

Question 24

What are the management options available for systemic sclerosis?

Answer 24

CaCB 
ACE inhibitor (HTN)
Prednisolone (acute)
Immunosuppression (inflammation)
Prostacyclin (Iloprost)
Bosentan, Sildenafil (pumonary HTN)

Question 25

What auto-antibody is present in mixed connective tissue disease?

Answer 25

Anti-RNP