SLE Flashcards
Systemic Lupus Erythematosus (SLE)
Etiology
Etiology
- a combination of factors from genetics & environmental influences which alter immune cell function and trigger an exacerbated inflammatory reaction by autoantibodies
- this targets virtualy every organ within the body
- type III antigen-antibody complex maker that widespread attacks teh body
environmental triggers include
- UV light (sunlight)
- tobacco smoke
- viral exposure
- silica expsoure
- pesticids
- gur micrbome influence
- demethylating meds
Typically
- younger female (15-45)
- high prevelence in non-white females
SLE
Diagnosis Criteria
Immunologic Criteria and Clinical Critera
EULAR and ACR say
- pt. must have a postive serum titer ANA of 1:80 or equlivent test
- pt. must have at leas 10 points from the below criteria boxes
Domains : Clinical
- Constitutional (Fever)
- Cutaneous (ulcers, alopecia, cutane. lupus)
- Arthrtis
- Neurologic
- Serositis (pleural/pericardial effusion)
- Hematologic (leukopenia, thrombocytopenia,etc.)
- Renal Domain (classes of SLE kidneys)
Immunologic domains
- Antiphosphoplipid antibody
- complement protiens (LOW c3 or C4)
- specifc Antibdoies (anti-dsDNA or anti-Smith)
SLE: commont additional symptoms but ones not associated with making the diagnosis
Additional Symptoms
- fatigue
- fever
- weight loss
- arthritis/arthragia
- Raynauds phenomen
- lymphadenopahty
- sicca symptosm (dry eyes and mouth– secondary sjogrens)
can have those following ab. + but not specific for SLE
- SS-A
- SS-B
- RNP
SLE: Common symptoms
SOAP BRAIN MD
Symptoms
S: serositis: pleuritis or pericarditis
O: oral or nasal ulcers
A: arthrtis
P: Photosensitivity (skin)
B: blood disorders: hemolytic anemia, leukopenia, lymphopenia, thrombocytopenia
R: Renal; proteinuria or cellular casts
A: ANA antibodies
I: Immunologica markers: anti-dsDNA, anti-smith, anti-phospholipid ab.
N: Neurologi disroders: seizures, psychosis
M: Malar rash (Butterfly)
D: Discoid rash
Actue Cutaneous Lupus
- what is it
- assocaited with SLE or no?
Acute Cutaneous Lupus: Malar “Butterfly” Rash
- characteristisc skin manifestation of SLE!
- a bright red, pathcy rash over the cheesk and nasal bridge which spares the nasolabial folds
- exacerbated by the sun!!!!
- lasts weeks to months
Assocaition
- 100% of those with this rash will develop SLE
- can occur prior to other symptoms or with them, but assocaited with systemic lupus, not just cutaneous lupus
Subacute Cutaneous Lupus
- what is it
- assocaited with SLE?
Subacute Lupus
- dry, red, scaley raises patches of a non-scarring rash which exisit in sun-exposed areas
- photosensitivtiy rash due to lupuse
- annular and slightly scaley with central clearing and red boarder
Assocaited with SLE?
- it is its own skin condition of lupus but
- 50% of those with this will go on to get SLE: systemic
Chronic Discoid Lupuse
- what is it
- associated with lupus?
Chronic Discoid
- hypo/hyper-pigmented, possible to be red, patches or thin plaques that are atrophic or hyperkatatotic
- typically on the face, ears and scalp
- can cuase hari loss but it can come back: nonscarring
assocaited?
- not a hallmark feature, but a small % of those with this will develop SLE
- otherwise, it s a cutaneous lupus by biopsy
Arthritis in SLE
Arthritis
- a hallmark domain for the diagnosis
- synovitis in at least 2 joints or tenderness in at least 2 joints
- characterisitcs of inflamm. = stiffness in the first 30 mins. of waking
can have ulnar deviation in severe cases (like RA)
- but in RA: the ulnar deviation is not redicable
- in SLE: the ulnar deviation is redicable
Lupuse Nephritis
- labs to indicate
- what is it
- classes of lupus nephritis
Lupus Nephritis
- renal development resultingin proteinuria, hematuria and can lead to renal failure
Labs
- look at ds-DNA and C3 C4 levels
- look at the spot protein to creatitine level OR a 24/hr creatitine urine level
Abnormal = ratio > 0.5g/24hours (indicates kidney involvement)
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classes do not dicated CKD/kidney trasnfers and do not act as a progression of disease state
Class I and Class II: not clinically seen as they dont provide lots of clinical symptoms
Class III: Focal LN: endo/extracapillary glomerulonephritis involving < 50% of the glomeruli
- can have active and chronic lesion
Class IV: Diffuse LN, impacting >50% of the glomeruli with subendothelial diffuse immune desposts
Class V: Membranous LN
- global or segmental subepithelial immune depostion
Class VI: advanced sclerosis
- terminal prognosis, 90% sclerosis of glomeruli
Labs for Lupus
- which stay constant
- which flux. with disease progress
Labs
- ANA
- anti-smith
- anti-dsDNA
- antiphosphlipid (cardiolipin)
- low C3 and C4 complement
- other Rhum: RNP, SS-A, SS-B can be postive
to monitor the progression of disease, anti-dsDNA and C3 and C4 levels are watched
low c3 and c4 + high ds-DNA = worsening
higher c3 and c4 with low ds-DNA = better
Lupus Nephritis
- how do you diagnose
- treatment
Diagnosis
Immunoflorecnces stain will show : Full House
- indicating all the antibodies tests shown up
Kideny Biopsy is the real Dx. in order to determine which class
Treatment
Treatment depends on the Class: ans the higher activitiy of the lupus in the kideny = the worse response to treatment it will have
Nonproliverative LN + Class II or Class V (subnephrotic proteinuria: not enough protiens) = immunosup. for extra renal disease
Nonproliverative LN + Class V (nephrotic proteinuira) = immunosup. to treat the LN
Proliverative LN + Class III/IV chronic = treat extrarenal only
Porliverative LN + Class II/Iv or mixed = treat with immunosup. to treat the LN
(LN with proliverative Class action)
first high dose steroids are used: prednisone methylpred. & tapered down
then
MMF: mycofant fenxiel is mainstay of thearoy for lupus nephritis
kidney protective: ACE, etc.
SLE: Cardiac Involvement
Cardiac Involvement
Libman Sacks endocarditis: nonbacterical thrombotic endocarditis
- due to immune deposition in the heart which leads to inflamation (antiphospholipid syndrome)
Pericarditis: the most common
- can happen with or without pericardial effusion
- EKG changes
- Myocardtis
- increased risk of CAD!
Pulmonary Involvement: SLE
Pulmonary
- diffuse alveolar hemorrhage : hemoptysis
- penumonitis
- pleuritis: with or without effusion
- interstitial lung disase (rare)
- pulmonary HTN
- shrinking lung syndrome
Neurologica Involvement: SLE
Neuropsychatric Lupus
often times the CNS is involved»_space;> than PNS
white matter invovled: on LP: see increased WBC in the CSF
- acute confusion
- cognitis dysfunction
- psychiatric disease
- pseudotumor cerebri
Hematologic Involvement: SLE
Hematologic SLE
- leukopenia: can WBC decreased
- Thrombocytopenia: < 100 k
- autoimmune hemolyisis (not anemia) : just ab. against cells drstorying them
