SLE

Question 1

what are connective tissue diseases?

Answer 1

conditions associated with spontaneous overactivity of the immune system, involving auto antibodies

Question 2

what is SLE (systemic lupus erythematosus)?

Answer 2

a systemic autoimmune condition causing inflammation and tissue damage that can affect almost anywhere in the body

Question 3

who is at higher risk of getting lupus

Answer 3

females, especially in child bearing years (9:1 ratio of female to male)

more common and severe in afro-caribbean, hispanic american, asian and chinese ethnicity

Question 4

does smoking affect your chances of developing lupus?

Answer 4

yes it increases the chance of you developing lupus

Question 5

describe the pathogenesis of SLE

Answer 5

• defect in apoptosis that causes increased cell death
• a problem with clearing the apoptotic cell debris, creating lots of nuclear auto antigens
• B cells begin to produce auto antigens that form auto antigen-antibody complexes
• immune complexes circulate the body and deposit themselves in different tissues/organs
• The deposit of these complexes in the tissue triggers the activation of the complement system which attracts leucocytes which release cytokines. The cytokines cause inflammation. These lead to inflammation, necrosis and scarring.

Question 6

what is the lupus classificayion criteria called?

Answer 6

EULAR/ACR 2019 SLE classification criteria

Question 7

what molecule is positive 95% of time in patients with lupus?

Answer 7

ANA positive

Question 8

what are some general symptoms of lupus?

Answer 8

• fever

- fatigue

Question 9

how may lupus present on skin?

Answer 9

• non-scarring alopecia (bottom right)
• oral ulceration
• half patients have a facial rash (top right)
• subacute cutaneous may present with erythematous rashes on chest/arms (top left)
• discoid lupus presents with scaly rash on cheeks/nose (bottom left)

Question 10

what are some symptoms of lupus affecting joints?

Answer 10

joints -arthritis, synovitis, arthralgia

Question 11

what are some neurological manifestations of lupus?

Answer 11

• headache (common)
• seizure
• psychosis
• delirium
• cranial nerve disorder

Question 12

what are some resp manifestations of lupus?

Answer 12

• sorositis (presents as a pleural or pericadial effusion with acute pericarditis)
• pleurisy
• effusion
• pneumonitis
• PE
• pulmonary hypertension
• ILD

Question 13

what are some haemotological manifestations of lupus?

Answer 13

• leukopenia
• thrombocytopenia
• haemolytic anaemia
• lymphadenopathy
• lymphopenia

Question 14

what are some menifestations of renal involvement of lupus?

Answer 14

proteinuria >0.5g in 24 hours
biopsy proven nephritis
red cell casts

Question 15

what antibodies are associated with SLE?

Answer 15

• ANA (not very specific)
• dsDNA (highly specific affects 60%)
• lupus anticoagulant
• anti cardiolipin antibodies
• anti beta2glycoprotein antibodies
• Anti Ro antibody (not specific)
• Anti Sm (highly specific but only present in 30%)

Question 16

can a positive ANA by itself diagnose lupus?

Answer 16

no- many other conditions cause positive ANA

Question 17

what antibody/ markers correlate with disease activity?

Answer 17

dsDNA (increased disease activity, high dsDNA)

complement e.g.C3, C4 (increased disease activity , low complement)

Question 18

what autoantibody is associated with lupus nephritis?

Answer 18

dsDNA

Question 19

what are the antiphospholipid antibodies ?

Answer 19

lupus anticoagulant
anti cardiolipin antibodies
anti beta3glycoprotein antibodies

Question 20

apart from SLE, what are antiphospholipid antibodies associated with?

Answer 20

venous and arterial thrombosis and recurrent miscarriage

Question 21

what does it mean if a pregnant women is positive for Anti Ro antibody?

Answer 21

her child is at high risk of neonatal lupus and congenital heart block

Question 22

what is a mild form of lupus?

Answer 22

-joint pain
-mouth ulcer
-skin disease
-hair loss
no organ threatening issues

Question 23

how would you describe moderate lupus?

Answer 23

• include some organ involvement but not life thretening disease
  e. g. inflammatory arthritis, recurring pleural effusions

Question 24

how would you describe severe lupus?

Answer 24

-organ threatening or life-threatening e.g. lupus nephritis so kidney involvement, neurological involvement

Question 25

``` in a patient with SLE what is least likely to cause chest pain: A. MI B. Pleurisy C. Pulmonary embolus D. Pericarditis E. Pneumothorax ```

Answer 25

E. Pneumothorax - all the other are associated with SLE. Many patients with anti phospholipid syndrome (which is associated to lupus) develop PEs.

Question 26

what occurs to the active complement levels in patients with SLE as the disease becomes more active?

Answer 26

they decrease

Question 27

which auto antibody is most specific for SLE?

Answer 27

Anti-DNA binding antibody

Question 28

what antibodies are seen in SLE but are not specific?

Answer 28

ANA | Anti-Ro

Question 29

what occurs to the level of anti-dsDNA antibody as SLE is more active?

Answer 29

it rises

Question 30

what is the pathophysiology for lupus?

Answer 30

- SLE causes the body to produce anti-nuclear antibodies - ANA attack cells within the own persons cell nucleus - this triggers the immune system to activate which activates the inflammatory response - this causes inflammation of the organs

Question 31

what antibodies can be seen in SLE?

Answer 31

``` ANA (most commonly seen but least specific) Anti-dsDNA (most specific) anti-smith (highly specific) Anti-RNP Anti-Ro Anti-Sm ```

Question 32

what is the management for SLE?

Answer 32

- NSAIDs - steroids - sun cream and sun avoidance first line= hydroxychloroquine second line= immunosuppressants (methotrexate, azathioprine) third line= biological therapies (Rituximab and Belimumab)

Question 33

what syndrome commonly occurs in patients with SLE?

Answer 33

antiphospholipid syndrome

Question 34

what complication commonly occurs to patients with SLE who developed antiphospholipid syndrome?

Answer 34

venous thromboembolism

Question 35

what pulmonary complications can SLE cause?

Answer 35

-pleurisy and pulmonary fibrosis

Question 36

is there an association between SLE and miscarrriages?

Answer 36

yes- SLE increases the chances of miscarriage