Systemic Lupus Erythematosus Flashcards
What is SLE?
chronic autoimmune disease with diverse clinical presentation
immune system attacks healthy tissues and organs throughout the body
Epidemiology
more ocmmon in women and in women of childbearnig age - peak incidence 15-45 yrs; more prevalent in non-whites
Etiology
exact etiology is unknown, but many pre-disposing factors
Pre-disposing factors
genetics: 1st degree relatives more likelt to develop SLE, or identical/fraternal twins
hormonal: estrogen production may modulate the incidence and severity of SLE
environment: cigaretter smoking, meds, UV light, air pollution, viruses (EBV), psychological stress, pesticides
Drug-induced lupus erythematosus
overreaction to certain meds; sx occur 3-6mo of drug initiation; resolution occurs within weeks of drug discontinuation
~10% of SLE cases are drug-induced
DILE examples
My Pretty Mala Marking Probably Has A Transient Quality
methimazole, propylthiouracil, methyldopa, minocycline, procainamide, hydralazine, anti-TNF agents (infliximab, etanercept), terbinafine, isoniazid, quinidine
Signs/symptoms
fatigue, depression, photosensitivity, joint pain, N/V, fever, weight loss, malar “butterfly” rash, discoid rash, raynaud phenomenon, lupus retinopathy, lupus nephritis
Raynaud phenomenon
exagerrated vascular response to cold temperature and emotional stress
pallor: constriction of blood flow, white; cyanosis: tissue hypoxia, blue; rubor: reperfusion, red
Diagnostic tools
SLICC
EULAR/ACR
SLICC
must meet >/=4 total features with 1 from each group OR biopsy proven lupus nephritis WITH systemic lupus (+anti-dsDNA antibodies or +ANA)
EULAR
only the highest weighted criterion score within a single domain is used
patient’s score is >/= 10 AND at least 1 clinical criterion is fulfilled
Key labs
anti-nuclear antibody (ANA), anti-double-stranded DNA (anti-dsDNA), anti-smith antibody (anti-SM), and antiphospholipid antibody
these are all negative in a healthy pt and all positive in lupus pt
ANA not specific, anti-dsDNA and anti-SM are high specificity; antiphospholipid antibody increases clotting factors
Pharmacologic treatments
hydroxychloroquine, NSAIDs, glucocorticoids, immunosuppressants, biologics
Hydroxychloroquine
MOA: antimalarial - inhibits overative immune cells
for ALL pts with SLE
reduces flares + helps manage pain
Hydroxychloroquine dosing
200-400mg PO daily
max: 400mg daily
Hydroxychloroquine SEs
retinal toxicity (bull’s eye maculopathy), neuropsychiatric effects, QT prolongation, cardiomyopathy, hypersensitivity, hypoglycemia, hemolytic anemia (if G6PD deficiency)
G6PD deficiency
X-linked disorder (present more often in males) that causes RBCs to prematurely break down
G6PD deficient, won’t convert NADP+ to NADPH, which won’t make glutathione, which won’t fight free radicals that damage blood cells
Hydroxychloroquine monitoring
CBC, LFTs, SCr, EKG
eye exam