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22 Cards in this Set

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  • Back
What are some drugs that induce lupus?

What is Neonatal Lupus? What can you say about the mom's status in terms of whether or not she has lupus?
Drugs: hydralazine, procainamide, quinidine, INH, minocycline

Neonatal Lupus- transient (from Anti-Ro antibodies being transferred transplacentally from mom into fetus). Complication= heart block, from anti bodies attacking conduction system. Mom does NOT have to have lupus for this to occur.
What are the diagnostic criteria for lupus?
4 out of 11:

1. malar rash (spares nasolabial folds)
2. discoid rash
3. photosensitivity
4. oral ulcers
5. arthritis
6. serositis (pleuritis/pericarditis)
7. Renal disorder
8. Neurologic disorder (seizure/psychosis)
9. Hematologic (anemia, thrombocytopenia)
10. Immunologic (dsDNA, Sm, etc.)
11. ANA+ (anti-nuclear antibody)

*basically, it is a systemic disease
Describe the pathogenesis of Lupus?
1. Genetic predisposition (certain HLA type) + 2. environmental trigger (UV light, infection?, etc.) -->
3. Abnormal Immune response (T cells activate complement and autoantibodies are produced 
4. Inflammation (systemic)
5. End organ damage
1. Genetic predisposition (certain HLA type) + 2. environmental trigger (UV light, infection?, etc.) -->
3. Abnormal Immune response (T cells activate complement and autoantibodies are produced
4. Inflammation (systemic)
5. End organ damage
Is there a genetic component to Lupus? If so what?
Yes- there is a strong familial aggregation (no known specific genes, but most likely polygenic).

MCH II polymorphisms in patients --> HLA-DR2, HLA-DR3 (mnemonic: 2,3, S.L.E)
What happens to complement in patients with SLE? Describe the mechanism of how this results in SLE?
Complement deficiencies seen in SLE (C1q, C2, C4a, etc.)

Complement usually clears the apoptotic cells. If deficient, apoptotic cell debris can persist and stimulate antibody response.
What is ANA? What other antibodies are targeted in SLE?

What is an autoantibody to a surface antigen that is seen in lupus?

Which one is often correlated with renal diseasE? Which one is more frequently seen in drug induced lupus?
ANA- antibodies to nuclear components (and other specific ones anti-dsdna, anti-sm, antiSSA, antiSSB, etc.)

anti-platelet antibodies- antibody to surface antigen
DsDNA- correlated to renal disease
anti-histone: seen in drug induced lupus
What is shown here?
What is shown here?
LE cell

Neutrophil that has engulfed chromatin/nuclear material + ANA. Presents as these reddish globs. *note: test is not done anymore
This is an immunofluorescence image from a biopsy of unaffected skin in a person with SLE. What do you see?
This is an immunofluorescence image from a biopsy of unaffected skin in a person with SLE. What do you see?
Ig deposition in the DEJ. Immune complexes will deposit in the dermal epidermal junction.
This is an image of the glomerular capillary interface in the kidney of a person with SLE. What is seen here?

What do you expect to see on immunofluorescence?
This is an image of the glomerular capillary interface in the kidney of a person with SLE. What is seen here?

What do you expect to see on immunofluorescence?
Immune complex deposition--> Mainly subendothelial deposits (and some subepithelial and intramembranous deposits).

Lumpy bumpy pattern on Immunofluorescence.
Immune complex deposition--> Mainly subendothelial deposits (and some subepithelial and intramembranous deposits).

Lumpy bumpy pattern on Immunofluorescence.
Is there a gender predominance in lupus?

What about race?
Female >> Male (9:1). Tends to occur more significantly during reproductive years.

No increased incidence in race but Increased mortality rates in African Americans.
What are the two most commonly involved systems of the body in Lupus?
Skin and Musculoskeletal system
Describe Acute, Subacute, and Chronic skin changes that occur in patients with SLE?
Acute= malar rash, erythema, bullae
Subacute= papulosquamous and annular polycyclic rash
Chronic: localized discoid rash

*note: lupus non-specific rashes may occur like urticarial lesions, oral lesions, etc.
What type of rash is seen here (chronic/acute/subacute)? How can you tell the difference between this and rosacea
What type of rash is seen here (chronic/acute/subacute)? How can you tell the difference between this and rosacea
Malar Rash (butterfly rash over the cheeks and bridge of nose)

*different from Rosacea- not pustular, spares the nasolabial folds
Malar Rash (butterfly rash over the cheeks and bridge of nose)

*different from Rosacea- not pustular, spares the nasolabial folds
What type of rash is seen here (chronic/acute/subacute)? What condition can occur if this rash is found on the surface of the head?
What type of rash is seen here (chronic/acute/subacute)? What condition can occur if this rash is found on the surface of the head?
Chronic- this is a discoid rash (there is also post-inflammatory hypo and hyperpigmentation)

Scarring discoid rash can cause alopecia (loss of hair that generally doesn't grow back).
Chronic- this is a discoid rash (there is also post-inflammatory hypo and hyperpigmentation)

Scarring discoid rash can cause alopecia (loss of hair that generally doesn't grow back).
What is shown in this person with lupus?
What is shown in this person with lupus?
Sun exposed skin --> photosensitivity

(remember, sun exposure = environmental factor that can trigger auto-inflammatory process)
What is shown in this image? Can you tell Lupus related arthropathy or Rheumatoid Arthritis and how?
What is shown in this image? Can you tell Lupus related arthropathy or Rheumatoid Arthritis and how?
Specific arthritic change *ulnar deviation and swans neck deformity i.e. Jaccoud's Arthropathy*

This is LUPUS and you can tell because there is no joint erosion or damage (unlike RA in which you see that).
Besides arthritis, what other musculoskeletal system manifestations occur in Lupus?
- Avascular necrosis (especially of femoral head)
- Septic arthritis
- Myositis (inflammation of muscles)
What is the predominant renal problem in patients with Lupus?

If you strongly suspect someone has renal involvement in lupus, what should you do?
Lupus Nephritis--> a glomerularnephritis (proteinuria, elevated creatinine, etc.). There are different types (mesangial, focal proliferative, diffuse proliferative, etc.)

Renal involvement suspected? GET A BIOPSY!!
What are the diagnostic criteria for NeuroPsychiatric Lupus?

What are the key hematologic manifestations of Lupus?
CNS criteria = unexplained seizure or psychosis

Heme= leukopenia and thrombocytopenia
A person comes in with pleuritic chest pain. You do a CXR and see no evidence of effusion. Why is Lupus on your differential?

What cardiac manifestation of lupus are we most worried about?
Pleuritic chest pain on inhalation without effusion suggest Lupus due to inflammation of pleura.

Cardiac manifestation = Accelerated Atherosclerosis!
What characteristic valvular lesion  (shown here) is seen in SLE? Why is it worrisome?
What characteristic valvular lesion (shown here) is seen in SLE? Why is it worrisome?
Libman-Sacks endocarditis- verrucous vegetation on the valve. From fibrin thrombi and necrotic cell debris.  

Can embolize and cause problems.
Libman-Sacks endocarditis- verrucous vegetation on the valve. From fibrin thrombi and necrotic cell debris.

Can embolize and cause problems.
What treatment is used in lupus frequently for cutaneous and MS manifestations?

Otherwise, how would you manage the mild constitutional sxs of lupus?
Anti-Malarial, specifically Hydroxychloroquine used for CUTANEOUS and MS symptoms. Also helps with fatigue.

NSAIDS used for mild constitutional sxs.