2.4 Autoimmune Disorders Part 2 Flashcards
What are the components of the RASH OR PAIN mnemonic for SLE?
- Rash (malar)
- Arthritis
- Soft tissue/ serositis
- Heme DO
- Oral/ nasopharyngeal ulcers
- Renal dz
- Photosensitivity
- ANA
- Immunosuppressants
- Neurological DO
Who is the classic SLE patient?
Middle aged AA /hispanic female
Is the female:male ratio with SLE more or less pronounced at extremes of age?
Less
What is the general pathophysiology of SLE?
Antibodies directed against host nuclear material, leading to the activation of C3, C4, and CH50 complement
Which three components of complement are classically depleted with SLE?
CH50
C3
C4
What type of hypersensitivity rxn is SLE?
III
What is CH50?
Test that measures complement C1-C9 levels and ability for complement to lyse RBCs
What are the three classic antibodies that are seen in SLE?
ANA
Anti-smith (snRNP)
Anti-dsDNA
What is the classic antibody that is elevated with drug induced SLE?
Anti-histone
What is the pathophysiology behind ANA?
As cells are damaged, nuclear material is released, exposing B cells to nuclear material, and activating the adaptive immune system.
-Ag/ab complexes are then phagocytosed by APCs, and further activate the immune system
Patients with what deficiency have an increased risk of developing SLE?
C1q, C2, C4 deficiency
What are the components of C3 convertase?
C1, C4, C2
What is the major function of C3b?
Removal of ag/ab complex
What is the function of Cr1 on RBCs?
Binds C3b, and takes the bound ag/ab complex to the spleen for removal
A deficiency in what complement components predisposes patients to SLE?
C1q
C2
C4
When in particular are malar butterfly rashes present?
Upon exposure to sunlight
What are the characteristics of the discoid rash with SLE?
Circular rash with erythema and plaques
What are the different forms of serositis that can occur with SLE patients?
Pericarditis
Pleuritis
What are the neurological s/sx of SLE?
Psychosis or Seizures
What is the most severe form of kidney damage that can occur with SLE?
Diffuse proliferative glomerulonephritis
What are some of the hematological disorders that can occur with SLE? (3) What type of hypersensitivity reaction does this represent?
Anemia
Thrombocytopenia
Leukopenia
Type II
What is the specific heart abnormality that can occur with SLE? What is this?
Libman-Sacks endocarditis
-Wart-like vegetations on both side of a valve
What is the role of anti-dsDNA in the prognosis of SLE?
Follows with the course of disease, and is used to predict renal involvement
What, generally, are antiphospholipid antibodies?
Autoantibodies against proteins bound to phospholipids
What are the three major antiphospholipid antibodies?
- Anticardiolipin
- Lupus anticoagulant
- Anti-beta2-glycoprotein I
Which antibody commonly found in SLE will produce a false positive VDRL and RPR test? Why?
Anticardiolipin
-lyses of cells 2/2 treponema pallidum infx releases cardiolipin