Review of SLE/Antiphospholipid Syndrome - Postlethwaite/Gupta Flashcards
4 Forms of Lupus discussed?
SLE (systemic lupus erythematosus)
DLE (discoid lupus erythematosus)
DILE (Drug induced lupus erythematosus)
Neonatal Lupus erythematosus
Broad definition of SLE?
Multisystem inflammatory disorder, with autoantibodies to numerous self antigens
What is the difference between Discoid Lupus (DLE) and SLE?
- Discoid is confined to the skin; no other symptoms involved.
- However, discoid lesions can be seen in SLE (roughly 5-10% of SLE patients)
What is a less severe form of SLE that can be easily resolved? Means of resolving?
Drug Induced Lupus Erythematosus (DILE) resolves once offending drug removed
Epidemiology of Neonatal Lupus (dont miss this one)?
2 Clinical findings? Which one is permanent?
Occurs in newborns of mothers with SLE (hence the term neonatal)
Clinical findings: skin rash (transient), heart block (permanent)
Epidemiology of SLE
- Risk of SLE in general population? Was this more or less than RA (fun fact)?
- What family relation has the highest risk of having SLE?
- Gender, Race most associated with SLE? Others?
Risk of SLE in general population 0.05%. RA was 1%
Monozygotic twins have 25-50% concordance
Blacks 3-6x more likely, Hispanic and Native American 2-3x, Asians 2x. All as compared to the whites. First you take their land, then they have a 2-3x more likely risk of getting SLE. Silver lining though: they have casinos. No sun in there
Oh, females more likely then men. 9:1 in adults. 2:1 in kids
Common causes of death in SLE?
Cardivascular disease (leading cause), Malignancy, Infection, Renal disease
Several factors contribute to increased mortality. Dr. P mentioned 3 that can be greatly improved, what are they?
- poor patient adherence
- inadequate patient support system
- limited patient education
How many criteria are there for Lupus classification? How many do you need to diagnose them with Lupus? Specificity/Sensitivity associated?
List as many as you can
11 criteria, need 4 to diagnose, if have 4/11 = 95% specificity, 85% sensitivity
First Aid has the mnemonic RASH OR PAIN:
- Rash (malar or discoid)
- Arthritis
- Soft tissues/serositis
- Hematologic disorder (immune mediated hemolytic anemia, leukonpenia, lymphopenia, thrombocytopenia, smallpenisia)
- Oral/nasopharyngeal ulcers
- Renal disease (glomerulonephritis, proteinuria)
- Photosensitivity/Positive VDRL/RPR
- Antinuclear antibodies (ANA)
- Immunologic disorder (antiDNA, antiSm, Antiphospholipid)
- Nerulogic disorder (seizures and/or psychosis)
sorry for the long slide
What do most/all lupus patients have in common?
They will have +ANA (95-98% of patients)
Relate ANA and Lupus to sensitivity and specificity.
Highly sensitive, low specificty for SLE. Presents in many autoimmune disorders as well as some healthy subjects
What test is most sensitive for testing for ANA?
Immunofluoresscence
When does the low specificity of ANA in lupus still provide useful information?
If you have high suspicion based on symptoms and sign, and +ANA, most likely Lupus
When does high sensitivity of ANA in lupus provide useful information?
Patient with negative ANA is unlikely to have lupus
Hi, im Dr. Satterfield, I’m going to talk to you about sensitivity and specificity.
fuck off satterfield. Snout and Spin. we get it.
Besides Lupus, what disorder is also associated with ANA? Percentage stats?
Scleroderma (95%). Dont forget, lupus 95-98%.
Others: Hashimotos thyroiditis 50%, Idiopathic pulmonary fibrosis 50%
Normal subjects = 3-4%, know this
What antibodies are associated with High specificity for Lupus? Which one has a clinical association
Anti-dsDNA (associated with Lupus nephritis)
anti-Sm (nonspecific)
What antibodies are associated with low specificity for Lupus?
ANA, Anti-RNP, Anti-SSA, Anti-SSB
What antibody is associated with intermediate specificity for lupus? What clinical association?
Antiphospholipid, associated with clotting diathesis
Anti-SSA and Anti-SSB antibodies are associated with clinical manifestations of lupus?
Also photosensitivity and dry mouth/dry eyes