SLE Flashcards
what are connective tissue diseases?
conditions associated with spontaneous overactivity of the immune system, involving auto antibodies
what is SLE (systemic lupus erythematosus)?
a systemic autoimmune condition causing inflammation and tissue damage that can affect almost anywhere in the body
who is at higher risk of getting lupus
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
does smoking affect your chances of developing lupus?
yes it increases the chance of you developing lupus
describe the pathogenesis of SLE
- 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.
what is the lupus classificayion criteria called?
EULAR/ACR 2019 SLE classification criteria
what molecule is positive 95% of time in patients with lupus?
ANA positive
what are some general symptoms of lupus?
- fever
- fatigue
how may lupus present on skin?
- 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)
what are some symptoms of lupus affecting joints?
joints -arthritis, synovitis, arthralgia
what are some neurological manifestations of lupus?
- headache (common)
- seizure
- psychosis
- delirium
- cranial nerve disorder
what are some resp manifestations of lupus?
- sorositis (presents as a pleural or pericadial effusion with acute pericarditis)
- pleurisy
- effusion
- pneumonitis
- PE
- pulmonary hypertension
- ILD
what are some haemotological manifestations of lupus?
- leukopenia
- thrombocytopenia
- haemolytic anaemia
- lymphadenopathy
- lymphopenia
what are some menifestations of renal involvement of lupus?
proteinuria >0.5g in 24 hours
biopsy proven nephritis
red cell casts
what antibodies are associated with SLE?
- 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%)
can a positive ANA by itself diagnose lupus?
no- many other conditions cause positive ANA
what antibody/ markers correlate with disease activity?
dsDNA (increased disease activity, high dsDNA)
complement e.g.C3, C4 (increased disease activity , low complement)
what autoantibody is associated with lupus nephritis?
dsDNA
what are the antiphospholipid antibodies ?
lupus anticoagulant
anti cardiolipin antibodies
anti beta3glycoprotein antibodies
apart from SLE, what are antiphospholipid antibodies associated with?
venous and arterial thrombosis and recurrent miscarriage
what does it mean if a pregnant women is positive for Anti Ro antibody?
her child is at high risk of neonatal lupus and congenital heart block
what is a mild form of lupus?
-joint pain
-mouth ulcer
-skin disease
-hair loss
no organ threatening issues
how would you describe moderate lupus?
- include some organ involvement but not life thretening disease
e. g. inflammatory arthritis, recurring pleural effusions
how would you describe severe lupus?
-organ threatening or life-threatening e.g. lupus nephritis so kidney involvement, neurological involvement
in a patient with SLE what is least likely to cause chest pain: A. MI B. Pleurisy C. Pulmonary embolus D. Pericarditis E. Pneumothorax
E. Pneumothorax - all the other are associated with SLE. Many patients with anti phospholipid syndrome (which is associated to lupus) develop PEs.
what occurs to the active complement levels in patients with SLE as the disease becomes more active?
they decrease
which auto antibody is most specific for SLE?
Anti-DNA binding antibody
what antibodies are seen in SLE but are not specific?
ANA
Anti-Ro
what occurs to the level of anti-dsDNA antibody as SLE is more active?
it rises
what is the pathophysiology for lupus?
- 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
what antibodies can be seen in SLE?
ANA (most commonly seen but least specific) Anti-dsDNA (most specific) anti-smith (highly specific) Anti-RNP Anti-Ro Anti-Sm
what is the management for SLE?
- NSAIDs
- steroids
- sun cream and sun avoidance
first line= hydroxychloroquine
second line= immunosuppressants (methotrexate, azathioprine)
third line= biological therapies (Rituximab and Belimumab)
what syndrome commonly occurs in patients with SLE?
antiphospholipid syndrome
what complication commonly occurs to patients with SLE who developed antiphospholipid syndrome?
venous thromboembolism
what pulmonary complications can SLE cause?
-pleurisy and pulmonary fibrosis
is there an association between SLE and miscarrriages?
yes- SLE increases the chances of miscarriage
