SLE (connective tissue disease)

Question 1

What causes inflammation and tissue damage in SLE?

Answer 1

The immune system attacking the body’s cells.
(autoimmune)
Antibody-immune complexes precipitate and cause a further immune response.

Question 2

Is SLE more common in males or females?

Answer 2

Females (9:1)

Question 3

Which races is SLE most common in IN THE UK/US?

Answer 3

Asians, Afro-Americans, Afro-Caribbeans, Hispanic Americans (more than americans of european decent)

Question 4

What hormonal factors are thought to increase incidence of SLE?

Answer 4

Incidence increased in those with higher oestrogen exposure. (those who develop early, those on oestrogen containing contraceptive, those on HRT)

Question 5

What environmental factors are thought to increase risk of SLE?

Answer 5

Viruses (e.g. epstein-barr virus)

UV light exposure (stimulating skin cells to secrete cytokines, stimulating B ells)

Silca dust (in cleaning powders, cigarette smoke and cement)

Question 6

Outline some of the main points in the pathogenesis of SLE:

Answer 6

Loss of immune regulation

Increased and defective apoptosis

Necrotic cells release nuclear material (which act as potential auto-antigens) (autoimmunity probable results from the extended exposure to nuclear and intracellular auto-antigens)

B and T cells stimulated

Autoantibodies are produced

Question 7

What constitutional symptoms can be present in SLE?

Answer 7

Fever
Malaise
Poor appetite
Weight loss
Fatigue

Question 8

What mucocutaneous features can be present in SLE?

Answer 8

Photosensitivity
Malar rash
Discoid lupus erythematosus (may scar)
Subacute cutaneous lupus
Mouth ulcers (painless)
Alopecia non-scarring)

Question 9

What musculoskeletal features can be present in SLE?

Answer 9

Non-deforming polyarthritis/polyarthralgia
Deforming arthropathy 0 Jaccoud’s arthritis
Erosive arthritis (RARE)
Myoathy - weakness, myalgia and myositis

Question 10

What type of distribution does non-deforming polyarthritis have?

Answer 10

Same distribution as Rheumatoid Arthritis but no radiological erosion

Question 11

What renal features can be present in SLE?

Answer 11

Proteinuria of .500mg in 24 hours

Red cell casts

Question 12

What is serositis?

Answer 12

Inflammation of the serous membrane

Question 13

What can serositis in SLE cause?

4

Answer 13

Pericarditis
PLeurisy
Pleural effusion
Pericardial effusion

Question 14

What neurological features can be present in SLE?

Answer 14

Depression, psychosis ( not always related to disease activity)
Migrainous headache
Seizures
Cranial or peripheral neuropathy
Mononeuritis multiplex

Question 15

What is mononeuritis multiplex (seen in SLE)?

Answer 15

A form of damage to one or more peripheral nerves (nerves outside brain and spinal cord)

Question 16

What haematological features can be present in SLE?

Answer 16

Lympadenopathy
Leucopenia (a reduction in the number of white cells in the blood)
Lymphopenia (low level of lymphocytes in blood)
Haemolytic anaemia
Thrombocytopenia (deficiency of platelets)

Question 17

How many patients with SLE will have lymphadenopathy during their course of illness?
(%)

Answer 17

Approx. 25%

Question 18

What is anti-phospholipid syndrome?

Answer 18

A disorder of the immune system that causes an increased risk of blood clot

Question 19

What other name is used for anti-phospholipid syndrome?

Answer 19

Hughes Syndrome

Question 20

What happens in anti-phospholipid syndrome?

Answer 20

Increased risk of blood clots.
Venous and arterial thrombosis.
Recurrent miscarriage.
Livido reticularis (skin appearance)
Thrombocytopenia
Prolongued APTT (activated partial thromboplastin time)

Association with other autoimmune conditions, especially SLE)

Question 21

What is livido reticularis?

Answer 21

A common skin finding consisting of a mottled reticulated vascular pattern that appears as a lace-like purplish discoloration of the skin. The discoloration is caused by swelling of the venules owing to obstruction of capillaries by small blood clots.
(Anti-phospholipid syndrome)

Question 22

Why are people with SLE particularly susceptible to infection?

Answer 22

Intrinsic factors:
Low complements
Impaired cell mediated immunity
Defective phagocytosis
Poor antibody response to certain antigens

Extrinsic Factors:
Steroids
Other immunosuppressive drugs
Nephrotic syndrome (kidney disease)

Question 23

In what titre is ANA positive in in almost all SLE patients (95%)?

Answer 23

Positive in titire of 1:160 or greater

titres less than 1:160 are present in up to 20% of healthy population

Question 24

Other than SLE, what conditions is ANA found in?

Answer 24

RA, HIV, Hep C, other autoimmune conditions

Question 25

How many patients with SLE does anti-double stranded DNA antibody occur in?

Answer 25

Approx. 60% of patients with SLE | Highly specific for SLE (Titre correlates with disease activity) (May be associated with lupus nephritis)

Question 26

Is anti-double stranded DNA antibody highly specific for SLE?

Answer 26

Yes

Question 27

Is ANA highly specific for SLE?

Answer 27

No (in titres <1:160, present in about 20% of healthy population) (Can also be found in other conditions such as Hep C, HIV, Rheumatoid Arthritis and other autoimmune conditions)

Question 28

What is lupus nephritis?

Answer 28

Inflammation of the kidney caused by SLE

Question 29

When should a positive ANA test be taken seriously (as an indicator of SLE?

Answer 29

If other antinuclear antibodies are positive (Anti-dsDNA, anti-Sm, anti-Ro, Anti-RNP) When the patient presents with connective tissue disease features

Question 30

What is Anti-Ro associatd with?

Answer 30

Associated with cutaneous manifestations (usually associated with anti-La) (60%)

Question 31

Is Anti-Sm specific?

Answer 31

Yes, very. (Probable association with neurological involvement) (10-20%)

Question 32

Is Anti-Sm sensitive?

Answer 32

No | specific but low sensitivity (if positive - likely to be SLE) (if negative, doesn't rule out)

Question 33

Are Anti-Ro, Anti-La and Anti-RNP specific?

Answer 33

No, may be seen in SLE but may also be seen in other conditions

Question 34

What happens to C3/4 levels in SLE?

Answer 34

Levels are lower when SLE active, especially renal disease

Question 35

Why is urinalysis does in SLE?

Answer 35

To look for evidence of glomerulonephritis

Question 36

Which anti-phospholipid antibodies may be positive in SLE?

Answer 36

Anti-cardiolipin antibody Lupus anticoagulant Anti-beta 2 glycoprotein (Must be positive on 2 occasions, 12 weeks apart)

Question 37

What is monitored to monitor disease activity in a patient with SLE after diagnosis?

Answer 37

Anti-dsDNA (positivity correlates with activity) (C3/4 levels negatively correlate with disease activity) (clinical assessment (incl. BP), urine examination incl. protein, cells and casts, FBC, blood biochemistry)

Question 38

What is the drug management of SLE?

Answer 38

NSAIDs and simple analgesia Hydroxychloroquine (Anti-malarials) - (Useful for arthritis, cutaneous manifestations and constitutional symptoms) (May reduce systemic complications) Steroids (useful but associated wih numerous side-effects)

Question 39

When (and in what doses) would you give steroids (prednisolone) for SLE?

Answer 39

Small doses - for skin rashes, arthritis, serositis Moderate doses - for resistant serositis, haematologic abnormalities and class V glomerulonephritis High doses - for severe/resisitant haematologic changes, diffuse glomerulonephritis and major organ involvement

Question 40

Name 4 immunouppressives that are used in SLE:

Answer 40

Azathioprine Cyclophosphamide Methotrexate Mycophenolate mofetil (all cause bone marrow suppression) (all can cause increased succeptibility to infection (ptentially teratogenic)

Question 41

Which (2) Biologics may be used in SLE?

Answer 41

Anti-CD20 (Rituximab) | Anti-Blys (Belimumab)