Lecture 101 - SLE Flashcards
what is SLE
Prototypical Systemic Auto-immune Disease
Multiorgan involvement
flares and remissions
auto antibodies – ANA, anti cytoplasmic and cell surface compoentns
who is susceptible
Female Dominant (9:1) – at middle age onset.
Child Bearing age is most susceptible
Predominance of females is less in younger and older ages
estrogen is involved in pathogensis
AA, hispanics > Whites
Theories of etiology –
_____complement compoennts may be deficienct; these function to ______
(C1q, C2, C4) – “Deficiency of Early Complement Components” – necessary for generation opsins which are necessary to clear Antigen-Antibody Complexes
Envirnomental Triggers
drugs
Viral
other exposure
Drugs – Hydralazine, procainamide, isonizide, penacillamine
viral –EBV
Other: UV Light
drug induced lupus –
what auto antibody is seen?
what is the treatment?
Anti-histone
Treat - remove the drug
What is the sensitive but not specific auto antibody test for lupus?
if positive how should you proceed?
Anti-nuclear antibodies
(sensitivity – 98%, Specificity – 86%; PPV -11%)
if positive – proceed to more specific antibody test (Anti-SMith and Anti-DsDNA)
what are the highly specific anti-body test for SLE?
which can be used as a prognostic factor (marker of disease activity)
anti-dsDNA – used to follow disease activity/nephritis (prognostic factor); marker of inflammation
anti-Smith (ribonuclear)
what autoantibodies (associated with anothre disease) should SLE patients (esp pregnant SLE patients) be screened and why?
Anti- SSB (la) - associated with Sjogren’s syndrome
Anti - SSA (ro) – associated with Sjogren’s syndrome; known to cause AV conduction block
SSA can cross the placenta and lead to Neonatal Lupus + Congential Heart Block – usually irreversible
Outcomes: Death vs pace maker implanted for life
what extracellular auto antibodies may also be present in SLE:
Anti-Phospholipid Antibodies –
what are the three types of Anti-Phospholipid Antibodies –
Anti Cardiolipid –
Lupus Anticoagulant
Anti B2 Glycoprotein
Anti Cardiolipid is known to cause false positive for what assay?
Lupus Anticoagulant – associated with what disease state that features a false______ test, even though there is _______
Anti Cardiolipid: VDRL and PRP – false positives for syphilis
Lupus Anticoagulant: Antiphospholipid Syndrome
falsely prolonged PTT, even thought it actually causes a hypercaogulable state
11 critaer assocaited with lupus
Malar Rash Discoid Rash Photosensitivity Oral Ulcers Arthritis SErositis Renal Disease -- Nephritic --> nephrotic syndromes Neuro -- psychosis/sz Hematologic -- Anemia, Luekopenia,Thrombocytopenia Immunologic d/o -- Abn ANA titers
FIRST AID mneumonic for lupus manifestations/findigs
RASH OR PAIN Rash -- Discoid or Malar Arthritis Serositis Hematologic -- anemia, TTP Oral Ulcers R -- Renal: nephritic/nephrotic P -- Photosensitivity; positive VDRL/RPR Antinuclear antibodies Immunologic (immunsuppresants) Neurologic -- Psychosis/Sz
causes of mortality for persons with lupus
early
late
early – active disease – nephritis, vasculits, organ failure
Infections from immunsuppreion
Late: Accerlated athersclerosis – Infarct, stroke due to chronic inflamatory states
Treatment of mild lupus
what is the mainstay of treatment
low dose corticoid steroids –
NSAIDs
Anti-malarial drugs
