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45 Cards in this Set

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  • Back
What is the prototype autoimmune disease?
Systemic lupus erythematosus
What are the 5 multisystemic autoimmune diseases?
SRSSG
-SLE
-Rheumatoid arthritis
-Sjogren's syndrome
-Scleroderma
-Goodpasture's syndrome
What are 10 organ specific autoimmune diseases? (acronym)
AMPPAIPAIP
What do the first three letters of AMPPAIPAIP stand for?
-Autoimmune thyroiditis
-Myasthenia gravis
-Pernicious anemia
What does PAIPAIP stand for?
Polymyositis
Addison's
Insulin dependent diabetes
Primary biliary cirrhosis
Autoimmune hemolytic anemia
Idiopathic thrombocytopenic purpura
Penphigus vulgaris
In what patients is lupus more common?
Women
In what tissues does injury occur in SLE?
-skin
-joints
-kidneys
-serosal membranes
What test is false positive in lupus?
VDRL for syphilis
What two types of rash are very specific for SLE?
-Malar rash
-Discoid rash
What 3 ANA's are relatively diagnostic for SLE?
-Anti-dsDNA
-Anti-Sm
-Anti-histone
What are high titers of Anti-dsDNA associated with?
Active glomerulonephritis
What do rises and falls in antibody titers of SLE correlate with?
Disease activity
What type of lupus is associated with Anti-histone antibodies?
Drug-induced le
What exacerbates the classic malar rash in lupus? What type of lupus is this called?
Sunlight makes it worse;
Acute cutaneous lupus
What does a histologic exam of skin show in acute cutaneous le?
Interface dermatitis
What is the pathologic mechanism of acute cutaneous LE?
Deposition of IgG and C3 along the basement membrane
What is the characteristic finding in discoid LE?
Skin plaques with elevated erythematous border
What is the good thing about discoid LE?
Only 5-10% of patients develop systemic LE, and only after several years
What is the ANA pattern of discoid LE like?
-35% have a pos ANA
-Rarely is anti-dsDNA present
What percentage of SLE patients have hematologic involvement?
100%!!!
What are 4 antibodies present in SLE patients?
-Anti-RBCs
-Antiplatelets
-Antilymphocytes
-Antiphospholipid
What are two results of having an Anti-phospholipid antibody?
-False positive RPR test (VDRL)
-False prolonged PTT
Why is the Anti-phospholipid antibody in SLE called lupus anticoagulant?
Because it actually causes a hypercoagulable state; it's a misnomer.
What percent of SLE patients have renal involvement?
50%
What does the renal involvement in an SLE patient tell?
The prognosis or progression of disease in the patient
What is the renal involvement in SLE?
Glomerulonephritis (one of 5 types)
What does glomerulonephritis result in?
Hematuria and proteinuria
What is the mild form of glomerulonephritis?
Mesangial
What are the levels of glomerulonephritis form mildest to worst?
1. Mesangial
2. Focal proliferative
3. Diffuse proliferative
4. Membranous
5. Diffuse thickening of the BM
What abnormal findings on urinalysis result from the hematuria and proteinuria?
Cellular casts, RBC casts, and way casts.
Which is the most serious of renal lesions in SLE?
Diffuse proliferative glomerular lesions
What is drug-induced LE?
A type of "lupus" that is diagnosed by a pos ANA in patients on certain meds
What are 4 meds that cause drug-induced LE?
-Hydralazzine
-Procainamide
-d-Penicillamine
-Isoniazide
Do patients with drug-induced LE have symptoms of SLE?
no
What is the characteristic ANA finding in drug-induced LE?
Anti-histone antibodies
What is Felty's syndrome triad?
-Rheumatoid arthritis
-Splenomegaly
-Neutropenia
(with an accompanying lymphocytosis)
What is Sjogren's syndrome?
Immunologic destruction of the lacrimal and salivary glands
What is it called when Sjogren's syndrome occurs by itself?
Sicca syndrome
Who are most patients that get Sjogren's syndrome?
Females between 40-50
What are the characteristic antibodies found in Sjogren's syndrome?
Anti-SS-A (anti-ro)
Anti-SS-B (anti-la)
What is the morphologic finding of Sjogren's syndrome?
Periductal and perivascular lymphocytic infiltration in salivary glands
What is a common finding in Sjogren's syndrome patients with high titer Anti-SS-A?
Involvement of the kidneys, lungs, skin, and CNS
Where are the renal lesions (if present) in SS patients?
In the TUBULES
Are there only T-cell mediated mechanisms in SS?
No; there is also enlargement of lymph nodes due to polyclonal Bcell activation
What is the result of the B-cell activation in Sjogren's syndrome?
A 40X greater risk of developing lymphoma