SLE Flashcards
SLE is a ___ autoimmune disorder associated with the production of ______
multi system
production of multiple antibodies
cause of SLE?
unknown
(in bold)
pts are genetically predisposed coupled with stimuli/trigger
SLE MC affects
Women
Women of Color (AA, Asian)
Young (20-30)*
SLE risk factors
- Immune system dysregulation
- Genetics (MC in daughter/mom)
- Hormonal (sex hormones)
- Environmental (geographic clusters)
characteristic manifestations of SLE
periods of active disease and periods of remission are
SLE flares caused by
illness, stress, or no identifiable cause
pathophysiologic mechanisms of SLE (2)
- trapping of immune complexes
2. direct autoimmune attack
trapping of immune complexes in SLE + symptoms
complexes in small blood vessels get trapped
causes plugging of the small capillaries that limit blood supply
direct autoimmune attack in SLE
direct attack - cell dysfunction and death
classic triad of SLE (+who gets it?)
Fevers
Rash
Joint pain
women of child bearing age
lupus mainly involves which systems
Skin joints kidney blood cells nervous system
constitutional symptoms of SLE
- fever (low grade)
- fatigue/diminished exercise tolerance
- myalgias
- weight changes
pt is classified as SLE IF
- biopsy proven lupus nephritis + ANAN or anti-dsDNA Abs
2. satisfies 4 of 11 diagnostic criteria (1 clinical and 1 immunologic)
diagnostic criteria of SLE
4/11 - SOAP BRAIN MD
Serositis
Oral Ulcers
Arthritis
Photosensitivity
Blood disorders Renal involvement Antinuclear ABs Immunologic phenomena Neurologic disorder
Malar rash
Discoid rash
derm manifestations of lupus
malar rash
discoid rash
photosensitivity rash
oral nasal ulcerations
malar rash
“butterfly” pattern of fixed erythema of cheeks (malaria eminences) and bridge of nose
SPARES nasolabial folds
discoid lesions
erythematous raised patches with thick adherent KERATOTIC scaling
how to distinguish malar rash from rosacea
scaling in malar rash
photosensitivity rash
reaction to sunlight
face and hands
occurs AFTER sun exposure
arthritis in SLE
non erosive, non derforming arthritis of two or more peripheral joints with tenderness, swelling, effusion
where is arthritis MC in SLE
MCP, PIP, wrists
migratory and fleeting
positive ANA
> 1:160
speckled centromere pattern is most specific
renal manifestations of lupus
proteinuria (0.5gm/day) cellular casts (red cell casts)
serositis
inflammation of any serial membrane
pleuritis, pleural/pericardial effusion or pericarditis
symptoms of pericardial effusion
feels better when patient leans forward
friction rub on exam
heme criteria
- hemolytic anemia
- leukopenia (<4k)
- lymphopenia (<1.5k)
- thrombocytopenia (<100k)
hemolytic anemia in SLE
elevated LDH
bilirubin
decreased haptoglobin
USUALLY Coombs +
immunologic abnormalities of SLE
anti-DNA antibodies
antibodies to Sm
presence of antiphospholipid antibodies
neuro criteria of SLE
seizures or psychosis
diagnostic imaging
Joint radiographs
CT scan of chest/abdomen/pelvis
MRI of brain
where to start with SLE lab evaluation
quantitative ANA
if > 1:160+ get more
secondary SLE labs
- ssDNA and dsDNA (HIGH specificity)
- Anti Sm
- Lupus anticoagulant
- anti-cariolipin antibodies
- anti-Ro and anti-La
- complement
- ESR, CRP
urinalysis and SLE
microscopy + spot CR to protein ratio
goals of SLE tx
- immunosuppression
- reduction of inflammation
- ID and reduce new onset of disease
tx considerations
disease and treatment causes morbidity and mortality
early rheumatology referrals and close management
behavioral tx of SLE
healthy diet,
avoid tobacco,
exercise,
annual CV risk assessment,
family planning
sunscreen and protective
clothes
vitamin D and Ca
supplementation
low dose aspirin
routine immunizations
why must SLE pts have routine immunizations
aggressive tx
pts are immunosuppressed
non live vaccines are given during stable dz
all medications used to treat lupus cause
bone marrow suppression
worsen hematologic abnormalities on CBC
what drugs don’t cause bone marrow suppression?
hydroxychloroquine (Plaquenil)
categories of drugs used to treat SLE
- anti-malarials
- NSAIDs
- Glucocorticoids
- DMARDs
antimalarials
mainstay of tx
decrease flares and prolong life
anti-malarials are most useful for
fevers
arthritis
muco-cutaneous symptoms
anti-malarials example
hydroxychloroquinalone (Plaquenil)
NSAID use in therapy
anti-inflammatory
control of arthritis, HA, serositis systems
monitor for renal and liver toxicities
glucocorticoids use in therapy
main agent for flares (high dose)
ADRs in glucocorticoids
toxicities
obesity, DM, osteoporosis, AVN, ocular dx, increased infection risk
non biologic DMARDs when?
symptoms of dz are severe
OR they are not well controlled to allow for discontinuing steroids
non biologic DMARDs list
Azathioprine (imuran)
Mycophenolate mofetil (cellcept, myfortic)
methotrexate (arthritis)
biologic DMARDs list
Belimumab (Benlysta)
Rituximab (Rituxan)
when do you use biologic DMARDs
SLE is refractory to other tx
family planning SLE
Intact fertility
pregnancy may worsen SLE and meds used to tx SLE are teratogens
appropriate contraception is essential in these patients
safe family planning methods SLE
IUDS
Non hormonal barrier methods
unsafe family planning SLE
estrogen products, Depo Shot
risk of osteoporosis
pregnancy and SLE
causes SEVERE flares in patients
higher rates of spontaneous abortion and prematurity (Esp. at time of pregnancy)
CVD in SLE
substantially increased risk of coronary artheriosclerotic disease
ESP. accelerated sclerotic disease
leading cause of death in SLE
CV mortality
when is CVD risk highest in pts?
prior to or within a few years of diagnosis
cause of renal disease
occurs insidiously from dz or result of medications
when should we preform a renal biopsy in SLE?
- increasing Cr w/o cause
- greater than 1 g proteinuria/24 hrs
- greater than 0.5 g proteinuria/24 hrs on hematuria or cellular casts on UA
what symptoms/signs might occur in a flare?
new AKI or psychosis/seizure
elevated fevers
worsen joint
treatment of an SLE flare
corticosteroid
heme disease in SLE
have some at baseline + medications worsen it
must determine if infection or flare
SLE infection heme abnormalities
- high fever
- leukocytosis
- thrombocytosis
- both ESR and CRP elevated
- Stable complement and dsDNA levels
SLE disease flare or meds heme abnormalities
- afebrile/low grade
- lymphopenia
- thrombocytopenia
- ESR elevation, CRP normal
- hypocomplementemia, increased dsDNA
life threatening SLE (4 main manifestations)
- lupus nephritis
- lupus cerebritis
- pulmonary hemorrhage
- vasculitis of small vessels
drugs to avoid in SLE
sulfa drugs and estrogens
pts with SLE are also at an increased risk for
CAD
dx of lupus requires
CAD screening
early causes of death in pts with SLE (5-10 yrs from diagnosis)
opportunistic infection
severe disease
later cause of death in pts with SLE
MI or CVA 2/2 accelerated arteriosclerosis
pts who survive long term must be monitored for
CAD
Cancer
AVN
what must be excluded in pts with SLE diagnosis
drug induced lupus
how does drug induced lupus present
mild arthritis, serositis, skin and constitutional symptoms
rare CNS or renal involvement
drug induced lupus epi
individuals 50-70s
M=F
caucasians MC
tx of drug induced lupus
withdrawal of drug
