Seminar G - SLE

Question 1

Definition

Answer 1

A multi-system inflammatory disease with a wide range of clinical manifestations.

Question 2

Pathophysiology

Answer 2

Excess production of pathogenic autoantibodies

→Anti-nuclear anti-body (ANA)

Question 3

Epi

Answer 3

1 in 4000

Female: Male 9/13:1

Question 4

Genetic Link

Answer 4

First degree relative family members being 50 times more likely to develop the disease than the background population.
HLA-DR2 and DR3 (increased susceptibility)

Question 5

Environmental Link

Answer 5

Sex hormones are known to influence disease activity:

1. Patients can experiences an exacerbation of symptoms pre-menstrually
2. OCP can cause a flare
3. Pregnancy can cause a flare (can be a threat to baby and mother)
4. UV light → causes increased rashes and systemic flare
5. Hydralazine – can causes a lupus-like illness.

Question 6

Autoantibodies

Answer 6

Anti-dsDNA
Anti-Ro
Anti-LA

Question 7

Antibodies cause

Answer 7

Complement mediated lysis
Enhanced phagocytosis (by mononuclear cells)
Vasculitis → via binding of immunoglobulins to intracellular proteins and nucleic acids
Glomerulonephritis

Question 8

Rare presentation

Answer 8

SLE may cause a symmetrical small-joint polyarthropathy similar to that seen in RA
Note: it is non-deforming (i.e. correctible) Jaccoud’s arthropathy

Question 9

5 year survival

Answer 9

90%

Question 10

15 year survival

Answer 10

85%

Question 11

Mortality from

Answer 11

Renal disease
Cardiovascular disease
Accelerated Atherosclerosis

Question 12

General Features

Answer 12

Fever
Malaise
Weight loss

Question 13

MSK

Answer 13

Arthralgia in 90%
Jaccoud’s arthropathy
Myalgia/myositis

Question 14

Skin

Answer 14

Butterfly rash in 30%
Other rashes
Photosensitivity
Alopecia
Mucosal ulceration
Raynaud’s phenomenon

Question 15

Cardiovascular

Answer 15

Pericarditis
Myocarditis
Endocarditis (libman-Sachs)
Thomboembolism (arterial and venous)
Accelerated atherosclerosis

Question 16

Pulmonary

Answer 16

Pleurisy (in up to 60%)
Pleural effusions
Interstial fibrosis

Question 17

Renal

Answer 17

Proteinuria/nephrotic Syndrome
Haematuria
Glomerulonephritis (any subtype)

Question 18

Haematological

Answer 18

Anaemia
Leukopenia (particularly lymphopenia)
Thrombocytopenia

Question 19

Neurological

Answer 19

Headaches
Seizures
Psychosis
Mononeuritis multiplex

Question 20

GI

Answer 20

Mesenteric vasculitis

Autoimmune hepatitis

Question 21

Immunological

Answer 21

Immune paresis

Question 22

Reproductive

Answer 22

Recurrent miscarriages
Premenstrual flares
Flares during pregnancy
Neonatal lupus with congenital heart block

Question 23

Treatment → Avoid

Answer 23

Sun exposure

High factor sun protection

Question 24

Treatment → NSAIDs for

Answer 24

Joint pains

Caution in pains with renal disease

Question 25

Treatment → Hydroxychloroquine

Answer 25

Alleviate symptoms in the medium term, but does nothing to treat the more serious manifestations of the disease such as neurological

Question 26

Treatment → Glucocorticoids

Answer 26

Under specialist supervision

Question 27

Treatment → Immunosuppressants

Answer 27

Azathioprine
Cyclophoshamide
Methotrexate