Systemic Lupus Erythematosus (SLE) Flashcards
systemic lupus erythematosus (SLE) - defined
*a chronic autoimmune disease of connective tissue involving multiple organ systems and marked by periodic acute episodes
*severity of symptoms range from mild to severe and can wax and wane within a single patient
systemic lupus erythematosus (SLE) - epidemiology
*9:1 FEMALE to male ratio
*peak onset in women: teens to 40s
*ethnicity:
-less common in Caucasians
-African American (3-6x), Hispanic & Native American (2-3x), Asian (2x) descent
systemic lupus erythematosus (SLE) - “classic patient presentation”
*female in her 20s who has experienced several years of relapsing and remitting symptoms, such as:
-butterfly rash over the face
-fever
-pain and swelling in one or more peripheral joints (hands and wrists, knees, feet, ankles, elbows, shoulders)
-chest pain with breathing (pleuritic chest pain)
-photosensitivity
systemic lupus erythematosus (SLE) - pathogenesis
*currently unknown
*environmental triggers (infections, chemical exposures)
*genetic predisposition?
*B cells, T cells, antibodies, immune complexes, cytokines all involved
*type III hypersensitivity reaction
systemic lupus erythematosus (SLE) - classification criteria
*malar rash
*discoid rash
*photosensitivity
*oral ulcers
*non-erosive arthritis
*serositis
*neurologic disorder
*renal disease
*hematologic disorder
*ANA positivity
*other positive immunologic labs
systemic lupus erythematosus (SLE) - malar rash
*erythema across cheeks and nose, sparing the nasolabial folds; sometimes referred to as “butterfly rash” on face
*present in about 50% of SLE
*often sun-sensitive
*spares the nasolabial folds (differentiates malar rash from rosacea)
systemic lupus erythematosus (SLE) - discoid lupus
*discrete, erythematous, slightly infiltrated plaques covered by a well-formed adherent scale
*look for around ears, scalp
*scarring
*25% of lupus patients
*can be seen without SLE
systemic lupus erythematosus (SLE) - photosensitivity
*rash after sun exposure that can last days to weeks
*60-100% of SLE patients
*latency can be hours to weeks
*SPF 30 always
*protective clothing, broad-brimmed hats, and sun avoidance
systemic lupus erythematosus (SLE) - oral ulcers
*12-45% of SLE patients
*oral ulcers are usually painless
*occur on soft or hard palate
*nasal ulcers
*no specific treatment
systemic lupus erythematosus (SLE) - arthritis
*90% of SLE patients
*usually symmetric & migratory
*inflammatory arthritis (morning stiffness > 1 hour, improves with use, worsens with rest)
*small joints of the hands (MCPs and PIPs), but all joints can be involved
*Jaccoud’s arthropathy: tendon inflammation and laxity, REVERSIBLE
*no evidence of erosions or cysts on films
systemic lupus erythematosus (SLE) - serositis
*inflammation of a serous membrane: lungs, heart, abdominal cavity
*commonly:
-pleurisy
-pleural effusion
-pericarditis
-pericardial effusion
systemic lupus erythematosus (SLE) - neurologic symptoms
*seizures
*psychosis
*peripheral neuropathy
*stroke
note - always need to rule out other causes
systemic lupus erythematosus (SLE) - lupus nephritis
*50-75% of SLE patients
*often in first few years of the disease
*meets criteria with one of the following:
-persistent proteinuria > 0.5 grams per day
-cellular casts
*several classes of LN
*if untreated, 50% progress to ESRD
systemic lupus erythematosus (SLE) - hematologic abnormalities
*leukopenia on 2 occasions
*lymphopenia on 2 occasions
*thrombocytopenia
*hemolytic anemia
systemic lupus erythematosus (SLE) - antinuclear antibody (ANA)
*almost all SLE patients are positive (but recall: high percentage of positive ANA in general population)
*sensitive but not specific (if negative, it is prob not lupus; if positive, it can be many things)
*titers:
1:80 normally not clinically significant
higher the titer, the more significant the test is
systemic lupus erythematosus (SLE) - other lab tests
*anti-dsDNA
*anti-Smith (most SPECIFIC - if positive, lupus very likely)
*LOW complements
*positive finding of antiphospholipid antibodies:
-anticardiolipin
-lupus anticoagulant (cross reacts with VRDL, prolonged PTT)
-beta 2 glycoprotein
positive anti-histone antibody indicates…
DRUG-INDUCED lupus
systemic lupus erythematosus (SLE) - other findings
*subacute cutaneous lupus
*livedo reticularis
*alopecia with balding spots (regrows with treatment)
systemic lupus erythematosus (SLE) - Raynaud’s phenomenon
*cold-induced vasospasm
*50% of SLE
*3 possible phases:
1. white = vasospasm
2. blue = ischemia
3. red = re-perfusion
most SPECIFIC antibody test for lupus
*anti-Smith antibody
recall: specific = rules IN lupus if positive
systemic lupus erythematosus (SLE) - treatment overview
*complicated; many factors are important, including symptoms, organs involved, age, child bearing status, ethnicity
*goals: preserve organ function, reduce or eliminate pain, diminish drug adverse effects
systemic lupus erythematosus (SLE) - medications
*HYDROXYCHLOROQUINE
*anti-inflammatory agents
*steroids (topical or systemic)
*immunosuppression:
-methotrexate, TNF alpha antagonists, azathioprine, cyclophosphamide
*belimumab
most important medication for SLE patients to be on
*hydroxychloroquine
hydroxychloroquine - benefits in lupus
*reduces flares
*improves morbidity and mortality
*decreases organ damage
*reduces thrombosis
*improves lipids
*enhances other drugs’ therapeutic benefit
hydroxychloroquine - ADEs
*retinal toxicity
systemic lupus erythematosus (SLE) - long term prognosis
*extremely variable
*relapsing and remitting disease
*most risks come from:
-early = organ disease (esp. renal)
-throughout = infection (esp. with immunosuppression)
-late = CAD
CAD and systemic lupus erythematosus (SLE)
*early onset CAD (in 30s)
*5-8x more likely to develop CAD
*treatment options:
-low dose ASA for SLE
-hydroxychloroquine for SLE (antithrombotic and antilipidemic effects)
-statins
pregnancy and systemic lupus erythematosus (SLE)
*better if disease is quiet for 3-6 months
*stop teratogenic medications
*NEVER STOP HYDROXYCHLOROQUINE
*important labs to check:
1. SSA (anti-Ro), SSB (anti-La) due to risk of fetal heart block
2. antiphospholipid antibodies - cause recurrent early fetal loss as well as late-term loss
drug-induced lupus - symptoms
*rash
*arthritis
*pleuropericarditis
*cytopenias
*fevers
drug-induced lupus - labs
*ANA positive
*anti-histone antibody
*less commonly: dsDNA
*not seen: SSA, SSB, anti-Smith
drug-induced lupus - treatment
*stop offending drug
drug-induced lupus - common causes
*hydralazine
*TNF inhibitors: etanercept (more often have + dsDNA)
*minocycline
*procainamide
*methyldopa
*isoniazid
*phenytoin
