Systemic Lupus Erythematosus (SLE) Flashcards

1
Q

systemic lupus erythematosus (SLE) - defined

A

*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

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2
Q

systemic lupus erythematosus (SLE) - epidemiology

A

*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

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3
Q

systemic lupus erythematosus (SLE) - “classic patient presentation”

A

*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

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4
Q

systemic lupus erythematosus (SLE) - pathogenesis

A

*currently unknown
*environmental triggers (infections, chemical exposures)
*genetic predisposition?
*B cells, T cells, antibodies, immune complexes, cytokines all involved
*type III hypersensitivity reaction

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5
Q

systemic lupus erythematosus (SLE) - classification criteria

A

*malar rash
*discoid rash
*photosensitivity
*oral ulcers
*non-erosive arthritis
*serositis
*neurologic disorder
*renal disease
*hematologic disorder
*ANA positivity
*other positive immunologic labs

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6
Q

systemic lupus erythematosus (SLE) - malar rash

A

*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)

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7
Q

systemic lupus erythematosus (SLE) - discoid lupus

A

*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

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8
Q

systemic lupus erythematosus (SLE) - photosensitivity

A

*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

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9
Q

systemic lupus erythematosus (SLE) - oral ulcers

A

*12-45% of SLE patients
*oral ulcers are usually painless
*occur on soft or hard palate
*nasal ulcers
*no specific treatment

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10
Q

systemic lupus erythematosus (SLE) - arthritis

A

*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

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11
Q

systemic lupus erythematosus (SLE) - serositis

A

*inflammation of a serous membrane: lungs, heart, abdominal cavity
*commonly:
-pleurisy
-pleural effusion
-pericarditis
-pericardial effusion

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12
Q

systemic lupus erythematosus (SLE) - neurologic symptoms

A

*seizures
*psychosis
*peripheral neuropathy
*stroke

note - always need to rule out other causes

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13
Q

systemic lupus erythematosus (SLE) - lupus nephritis

A

*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

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14
Q

systemic lupus erythematosus (SLE) - hematologic abnormalities

A

*leukopenia on 2 occasions
*lymphopenia on 2 occasions
*thrombocytopenia
*hemolytic anemia

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15
Q

systemic lupus erythematosus (SLE) - antinuclear antibody (ANA)

A

*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

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16
Q

systemic lupus erythematosus (SLE) - other lab tests

A

*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

17
Q

positive anti-histone antibody indicates…

A

DRUG-INDUCED lupus

18
Q

systemic lupus erythematosus (SLE) - other findings

A

*subacute cutaneous lupus
*livedo reticularis
*alopecia with balding spots (regrows with treatment)

19
Q

systemic lupus erythematosus (SLE) - Raynaud’s phenomenon

A

*cold-induced vasospasm
*50% of SLE
*3 possible phases:
1. white = vasospasm
2. blue = ischemia
3. red = re-perfusion

20
Q

most SPECIFIC antibody test for lupus

A

*anti-Smith antibody

recall: specific = rules IN lupus if positive

21
Q

systemic lupus erythematosus (SLE) - treatment overview

A

*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

22
Q

systemic lupus erythematosus (SLE) - medications

A

*HYDROXYCHLOROQUINE
*anti-inflammatory agents
*steroids (topical or systemic)
*immunosuppression:
-methotrexate, TNF alpha antagonists, azathioprine, cyclophosphamide
*belimumab

23
Q

most important medication for SLE patients to be on

A

*hydroxychloroquine

24
Q

hydroxychloroquine - benefits in lupus

A

*reduces flares
*improves morbidity and mortality
*decreases organ damage
*reduces thrombosis
*improves lipids
*enhances other drugs’ therapeutic benefit

25
Q

hydroxychloroquine - ADEs

A

*retinal toxicity

26
Q

systemic lupus erythematosus (SLE) - long term prognosis

A

*extremely variable
*relapsing and remitting disease
*most risks come from:
-early = organ disease (esp. renal)
-throughout = infection (esp. with immunosuppression)
-late = CAD

27
Q

CAD and systemic lupus erythematosus (SLE)

A

*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

28
Q

pregnancy and systemic lupus erythematosus (SLE)

A

*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

29
Q

drug-induced lupus - symptoms

A

*rash
*arthritis
*pleuropericarditis
*cytopenias
*fevers

30
Q

drug-induced lupus - labs

A

*ANA positive
*anti-histone antibody
*less commonly: dsDNA
*not seen: SSA, SSB, anti-Smith

31
Q

drug-induced lupus - treatment

A

*stop offending drug

32
Q

drug-induced lupus - common causes

A

*hydralazine
*TNF inhibitors: etanercept
(more often have + dsDNA)
*minocycline
*procainamide
*methyldopa
*isoniazid
*phenytoin