Lupus Flashcards
What is Systemic Lupus Erythematous?
Chronic inflammatory disease of unknown cause.
What does SLE affect?
Skin, joints, kidneys, lungs, nervous system, serous membranes
What is SLE in origin?
Autoimmune
Clinical course of SLE characterized by what?
Periods of remission and chronic/acute relapses
What factors play a role in pathogenesis?
Genetics and environmental factors
Who has higher incidence?
Blacks and indian tribes
Which genetic HLA haplotypes are increased in SLE population?
HLA DR2 and DR3
Which Haplotypes on chromosome 2 are associated with increased incidence of rheumatoid and lupus?
STAT4 haplotypes
What do STAT4 haplotypes code for?
Cytokines
Deficiencies of which complements are associated with SLE and discoid lupus?
Complements C2 and C4
What do twin studies show about SLE?
Monozygotic twins both more commonly develop SLE than dizygotic twins
What is the estimated number of susceptibility genes needed for development of SLE disease?
4
Which drugs can cause an SLE like illness?
Procainamide and hydralazine
What are three other environmental factors than can start SLE?
1) Hairy dye
2) Smoking
3) UV light
Women of what age are at greatest risk?
Childbearing years
Estrogen effect on SLE?
Prolongs survival of autoimmune cells
Effects of prolonged autoimmune cells in SLE?
Increases T-helper cell cytokine production
Stimulates B-cell to produce autoantibodies
Correlation between CD40 ligand in women?
More X chromosomes express CD40 ligand in women with lupus than women without
Is a low level of auto reactivity pathologic?
No, physiologic
Activated T cells stimulate B cells to produce what?
Autoantibodies
Total T cells are what in lupus?
Decreased
T cell function is skewed toward what in lupus?
B-cell help
Direction of IL-10 and 12 in SLE?
IL-10 increased
IL-12 decreased
Problems with immune regulation in SLE?
Phagocytes defective in clearing immune complexes
T-cell CD8 suppressor cells deficient in function
What happens to lymphocytes in patients with SLE?
Apoptosis
Increased apoptosis in SLE can lead to what?
Leakage on intracellular antigens which triggers further autoimmune response
Where does tissue damage in blood vessels and kidneys come from?
Deposition of antigen-antibody complexes
Antibodies to phospholipids is known as what?
Lupus anticoagulent
Lupus anticoagulent is associated with what?
Prolonged PTT Venous/arterial thrombosis Fetal distress Thrombocytopenia Livedo reticularis
Most common systemic symptom of SLE?
Fatigue (Too nonspecific)
How does arthralgia present in SLE?
Symmetric, mimics RA
Not as erosive as RA
WBC
SLE patients have what reaction to sun?
Rashes or activation of systemic disease
Butterfly rash
Erythematous maculopapular eruption is what?
SLE pruritic eruption that can occur after sun exposure
What is Discoid lupus?
Sharply defined papule and plaques that become atrophic in the center while edges remain edematous and erythematous. Scars
Difference between subacute cutaneous lupus and discoid lupus?
Looks like discoid but no scarring
Where are oral ulcers commonly found in SLE?
Hard and soft palate
How common is alopecia in SLE?
Greater than 50%, can be patchy
Cardiac problems due to SLE?
Libman-Sacks (Valve replacement due to bacteria)
Resting tachycardia, abnormal EKG
Coronary artery disease due to immune complex
Conduction deficits
What is a big clue for SLE diagnosis?
Leukopenia
How will erythrocyte sedimentation rate present in SLE?
Elevated
Four pathologic types of Kidney damage in SLE?
1) Mesangial glomerulonephritis
2) Focal proliferative nephritis
3) Diffuse proliferative glomerulonephritis
4) Membranous glomerulonephritis
How does Cerebrospinal fluid look in SLE?
Usually normal
Abnormal EEG common or uncommon in SLE?
Common 80%
What is Raynaud’s phenomenon?
3 color change of fingers on exposure to cold. White-> blue -> red
Lupus anticoagulant is associated with what?
Frequent miscarriages
Bone most frequently damaged by avascular necrosis
Hip
Most important lab diagnostic?
ANA
Key to SLE treatment?
Patient education
Mild SLE treated with?
Antimalarial hydroxychloroquin
Major organ disease from SLE treated with?
Corticosteroids and cytotoxic agents
Greatest cause of death in SLE patients?
Infection
