Systemic Lupus Erythematosus (SLE)

Question 1

Systemic lupus erythematosus (SLE) is an i________ a______ c______ t_____ disorder

Answer 1

inflammatory autoimmune connective tissue

Question 2

“Erythematosus” refers to the typical…

Answer 2

red malar rash across the face

Question 3

Which group are most at risk of SLE?

Answer 3

Women
Asian, African, Caribbean and Hispanic
Young to middle-aged adults

Question 4

What are risk factors for SLE?

Answer 4

Genetic defect
UV radiation
Smoking
Viral and bacterial infection
Certain medication

Question 5

SLE typically takes a r_____-r____ course

Answer 5

relapsing-remitting

Question 6

The result of chronic inflammation means that SLE can shorten life expectancy with c_______ disease and i____ as significant complications.

Answer 6

cardiovascular disease
infection

Question 7

Which antibodies are seen in SLE?

Answer 7

Anti-nuclear antibodies (ANA) which act against proteins in the cell nucleus.

Question 8

How may SLE present?

Answer 8

Malar rash, butterfly shaped across nose and cheeks.

Non-specific symptoms:
Fatigue
Weight loss
Arthralgia
Non-erosive arthritis
Fever
Lymphadenopathy
Splenomegaly
SOB
Pleuritic chest pain
Mouth ulcers
Hair loss
Raynaud’s phenomenon
Oedema due to nephritis

Question 9

What triggers and worsens the malar rash in SLE?

Answer 9

Sunlight

Question 10

What may a full blood count show in an SLE patient?

Answer 10

Anaemia of chronic disease
Low WBC count
Low platelets

Question 11

CRP and ESR may by ____ with active inflammation

Answer 11

raised

Question 12

C3 and C4 (complement) levels may be ______ in active disease (seen in SLE)

Answer 12

decreased

Question 13

What may a urinalysis in SLE show?

Answer 13

Proteinuria

Question 14

Give an example of another condition that may show positive for anti-nuclear antibodies?

Answer 14

Autoimmune hepatitis
(certain other autoimmune conditions)

Question 15

Which antibodies are highly specific to SLE?

Answer 15

Anti-double strand DNA antibodies
(anti-dsDNA)

Note: only half of SLE patients have these

Question 16

A_______ antibodies and a______ syndrome can occur secondary to SLE in 40% patients

Answer 16

Antiphospholipid

(increased risk of venous thromboembolism)

Question 17

What criteria can be used for diagnosis, taking into account both the clinical features and autoantibodies present?

Answer 17

EULAR (European League Against Rheumatism)
or
ACR criteria (American College of Rheumatology criteria)

Question 18

What is the leading cause of death from SLE?

Answer 18

CVD - chronic inflammation in blood vessels leads to hypertension and coronary artery disease

Question 19

What can be the cause of anaemia in SLE?

Answer 19

Anaemia of chronic disease
Autoimmune haemolytic anaemia
Bone marrow suppression by medications
Kidney disease

Question 20

What causes anaemia of chronic disease?

Answer 20

Chronic inflammation causes release of inflammatory cytokines which interfere with normal functioning of iron-regulatory hormone (hepcidin).
Leads to decreased iron absorption from gut, reduced release of iron for macrophages and impaired iron utilisation in developing RBCs.

Question 21

What lung complications are there from SLE?

Answer 21

Pleuritis (causing sharp pain on inspiration) and Interstitial lung disease (inflammation in lung tissue leading to fibrosis)

Question 22

What complication of SLE causes sharp chest pain that gets worse on lying flat?

Answer 22

Pericarditis

Due to blood volume in central veins increasing when lying flat (gravity). Increased pericardial pressure, exacerbates chest pain.

Question 23

Neuropsychiatric SLE (inflammation in CNS) can present with optic neuritis, transverse myelitis (inflammation of spinal cord) or p____

Answer 23

psychosis

Question 24

Recurrent m____ along with pre-eclampsia, pre-term labour and intrauterine growth restriction are all increased in SLE

Answer 24

miscarriage

Question 25

V______ t_____ may be associated with antiphospholipid syndrome, secondary to SLE

Answer 25

Venous thromboembolism

Question 26

Lupus nephritis can progress to….

Answer 26

ESRF

Question 27

Treatment of SLE aims to control symptoms and reduce complications with minimum m____ and s___ e_____

Answer 27

medications and side effects

Question 28

What measure help manage the malar rash?

Answer 28

Sun cream and sun avoidance

Question 29

What are first line medications for SLE?

Answer 29

Hydroxychloroquine
NSAIDs
Steroids

Question 30

What other condition is hydroxychloroquine used to treat?

Answer 30

Malaria

Question 31

What are treatment options for more severe SLE?

Answer 31

DMARDs (eg cyclophosphamide, methotrexate)
Biologic therapies

Question 32

What does DMARD stand for?

Answer 32

Disease-Modifying Antirheumatic Drugs

Question 33

What are possible biological therapies?

Answer 33

Rituximab (monoclonal antibody targets CD20 on surface of B cells)

Belimumab (monoclonal antibody targets B-cell activating factor)

Question 34

What is lupus nephritis?

Answer 34

Anti-nuclear antibody (ANA) deposition in endothelium
Secondary to SLE

Question 35

What initiates the high prevalence of antinuclear antibodies (ANA)?

Answer 35

Damaged DNA from environmental triggers causes apoptosis. This process releases nuclear bodies and nucelar antigens into the blood stream. Clearance of the apoptotic bodies is ineffective due to some genetic defect. So increased nuclear antigens initiates immune response = production of ANA which bind to antigens and form immune complexes which deposit in tissues.

Question 36

What kind of hypersensitivity reaction is seen in SLE?

Answer 36

Type 3