Term
| What are the diagnostic criteria for SLE |
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Definition
4 Skin RASH NIA
1) Malar Rash (Fixed erythema)
2) Photosensitivity
3) Oral/nasal ulcers
4) Discoid rash
5) Renal (Proteinuria and cell casts)
6) Arthritis
7)Serositis (pleuritis or pericarditis)
8) Hematologic cytopenias/hemolytic anemia.
9) Neurologic (seizures and psychosis)
10) Immunologic (anti-DNA anti-bodies)
11) ANA |
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Term
| What is the basic epidemiology of SLE? |
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Definition
15-35 years old
Female 9:1 Male
159,000 US adults |
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Term
| What does a positive ANA test mean? |
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Definition
There are circulating anti-nuclear antibodies
Almost all SLE cases have (+) ANA (sensitive)
Many people without SLE ALSO have (+) ANA (not that specific) |
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Term
| How does one diagnose SLE? |
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Definition
Need 4 criteria out of 11
Ex) Malar Rash, (+) ANA, Neurological Symptoms and Hemolytic anemia.
Ex2) Discoid Rash, Immunologi symptoms, (+) ANA, Renal protinuera) |
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Term
| What are the major subsets of SLE? |
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Definition
1) Discoid- chronic involving discrete plaques (often erythematous), covered by scale that extends into dilated hair follicles, and atrophic scaring.
2) Limited systemic (ANA, arthritis, rash, oral/nasal ulcers)
3) Severe systemic (renal, CNS)
4) Hematologic (thrombocytopenia, other)
5) Drug-induced
6) Anti-phospholipid antibody sndrome (APS)
7) Neonatal (anti SSA/Ro transfer from mother to fetus) |
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Term
| Which subset of SLE involves anti SSA/Ro transfer? |
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Definition
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Term
| Which subset of SLE involves a positive ANA, with arthritis, rash, oral/nasal ulcers? |
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Definition
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Term
| Which subset of SLE involves thrombocytopenia? |
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Definition
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Term
| Which subset of SLE involves renal failure and seizures? |
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Definition
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Term
| What different type of anti-nuclear antibody staining can you see in SLE? |
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Definition
Rim (dsDNA)
Homogenous (histone)
Speckled (Sm, RNP, SSA, SSB)
Centromeric (limited SSc) |
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Term
| What subset of SLE might a patient have with Rim antibody staining? What else might you see? |
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Definition
Rim is for dsDNA and is seen in glomerulonephritis (high specificity and low sensitivity)
The more dsDNA you see, the worse the prognosis.
Complement decreases.
You would look for "lumpy" Ab deposits in glomeruli. |
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Term
| What subset of SLE might a patient have homogenous anti-nuclear autoantibody staining? |
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Definition
| Histone staining, so this is drug-induced SLE |
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Term
| A patient presents with ANA, arthritis, rash and oral/nasal ulcers. Which Lupus subset is this? |
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Definition
| Limited systemic (NORMAL type) |
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Term
| Why might you be somewhat relieved to diagnose a case of discoid SLE? |
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Definition
| no internal organ involvement! |
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Term
| When might you see elevated SSA or Ro antibodies in the serum? |
|
Definition
1) Neonatal lupus
2) Sjogren's syndrome
3) Rash |
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Term
| When might you see elevated SSB or La antibodies in the serum? |
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Definition
| Sjogren's (you would also look for SSA) |
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Term
| When might you see elevated RNP antibodies in the serum? |
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Definition
| This would be a "speckled" immunoflourescent stain and cold indicate Systemic Sclerosis or MCTD. |
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Term
| Why might you run a "lupus band" test? |
|
Definition
Looking for subacute, cutaneous SLE.
You would see deposition of anti-Ro (SSA) antibodes with lit up nuclei of epidermal cells. |
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Term
| How can you determine if a patient has Glomerulonephritis SLE? |
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Definition
1) Complement (CH50, C3, C4, C1q) lowered in disease state (look for antibodies)
2) ESR elevated during disease
3) dsDNA elevated in glomerulonephritis
4) Urinalysis for proteinuria and microscopic hematuria & RBC clasts. |
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Term
| What are the 4 most useful laboratory measures for SLE? |
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Definition
1) Complement (CH50, C3, C4, C1q) lowered in disease state
2) ESR elevated during disease
3) dsDNA elevated in glomerulonephritis
4) Urinalysis for proteinuria and microscopic hematuria & RBC clasts. |
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Term
| What are 3 clinical markers of SLE? |
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Definition
All are scoring systems for disease activity
SLEDAI
BILAG
SLICC/ACR |
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Term
| What is the role of BLyS (BAFF) in SLE? |
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Definition
Elevated BAFF seen in SLE, and is related to increased B/T cell survival.
Released by myeloid cells, BAFF binds to either BR3 or TACI
1) If BR3, NF-kB-2 is activated, leading to BCL-2-like survival
2) If TACI, NF-kB-1 is activated, leading to B-cell class switching and T-cell-dependent responses. |
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Term
| What is the role of IFN in SLE? |
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Definition
1) Type 1 IFN genes up-regulated in blood of SLE patients
2) DCs produce IFN-a and b in response to TLR-activation mediated by apoptotic cell debris (this could result in epitope spreading of self antigens) |
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Term
| What is the relationship between blood plasmoblasts and SLE? |
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Definition
| These transient B-cells produce short-lived plasma cells that lead to fluctuations of auto-antibodies seen in SLE. |
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Term
How do the following drugs act to treat SLE:
1) Hydroxychloroquine
2) Belimumab
3) anti-IFN-a
4) Rituximab/Epratuzumab |
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Definition
1) Acts on MHC-II molecules on DCs to down-regulate antigen presentation
2) Anti-BLyS to prevent B-cell survival
3) Prevents IFN-1 signaling from macrophages to B-cells and prevents activation-induced clonal expansion.
4) Anti-CD-20 to prevent B-cell survival |
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Term
| How can you diagnose Drug-induced SLE? |
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Definition
1) Is the patient on Hydralzaine or Procainamide?
2) Is the patient suffering from fatigue, arthritis, serositis and myalgias?
3) Do you see a homogenous, histone staining on ANA? |
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Term
| How can you diagnose Anti-phospholipid antibody syndrome (APS) |
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Definition
Remember, APS can occur OUTSIDE of SLE!
1) Look for arterial and venous thromboembolism
2) Autoantibodies again phospholipids, cardiolipin, beta-2-glycoprotein
3) Venous thrombosis in UNUSUAL sites (splenic, portal, subclavian, ect.)
Look out for thrombosis or stroke (5-12%!) |
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Term
| How can you diagnose Neonatal Lupus? |
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Definition
1) Rash associated with anti-SSA (Ro) deposition in skin
2) Heart block (sometimes in the womb)
1% of children with mothers lacking anti-Ro still get this! |
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