SLE Flashcards
What are the 4 different types of lupus?
- Systemic Lupus Erythematosus
- Discoid Lupus Erythematosus
- Drug-induced Lupus Erythematosus
- Neonatal Lupus Erythematosus
What are the characteristics of Systemic Lupus Erythematosus?
- Mulitsystem inflammatory disorder
- Autoantibodies to numberous self-antigens
What are the characteristics of Discoid Lupus Erythematosus?
- Confined to the skin ONLY
- Discoid lesions can be seen in SLE
What are the characteristics of Drug-induced Lupus Erythematosus?
- Less severe than SLE
- Resolves once offending drug is removed
- Nephritis is very uncommon unless with anti-TNF agents
What are the characteristics of Neonatal Lupus Erythematosus?
- Newborns of mothers with lupus
- Skin rash and heart block
Do genes play in to the pathogenesis of lupus?
YES- highly!
What are the susceptibility genes in lupus?
HLA-DR2, DR-3 (weak), C4A (complement deficiency)
What ethnic groups get lupus?
African Americans (3-6X more than whites) Hispanic and Native americans (2-3X more than whites)
What are the abnormally functioning cells in SLE?
B-cells produce autoantibodies
T-cells are abnormal
pDC cells- interferon signature
What is an Interferon-signature?
Innate immune system does not turn off after containing infection. Alpha and beta-interferon causes pDC cells activates T cells to Th1/Th17 phenotype–get activation of B cells–> Lupus.
What causes tissue damage in lupus?
- Immune complexes get deposited in tissues
- Pro-inflammatory molecules are activated (complement-medicated)
What are some predictors of lupus flare?
- New evidence of complement consumption (drop in C3 and C4)
- Rising anti-dsDNA titer
- Increased ESR
- New lymphopenia
What are factors associated with higher disease severity?
- Abrupt onset of symptoms
- Increased renal, neurologic, hematologic, and serosal involvement
- Rapid accrual of damage (irreversible organ injury)
- Men
- Lower SES
- Early age of onset
What is the leading causes of death in lupus?
heart disease
malignancy
infection
How do you test for ANA?
IFA
When is ANA presence helpful in diagnosing lupus?
if patient has high pretest probability for lupus
When is ANA absence helpful in ruling out lupus?
HIGH SENSITIVITY (if patient does not have it, they will not have lupus)
What other autoantibodies are common in SLE patients?
anti-dsDNA and anti-sm and antiphospholipid
Are anti-dsDNA and anti-sm sensitive or specific?
highly specific–almost always associated with lupus ONLY
What is anti-dsDNA clinically associated with?
lupus nephritis
What is antiphospholipid antibody clinically associated with?
clotting diathesis
What can be measured to see if the classical complement pathway is being activated?
C4
usually see decreased C3 and C4
What musculoskeletal abnormalities of the hand do you see in lupus?
periarthritis (with loosening of the ligaments) –> reducible hand deformities
What are factors associated with serositis?
pleuritic chest pain
pericardial abnormality (effusion)
peritoneal cavity fluid accumulation
How does a baby get neonatal lupus?
-transplacental transfer of autoantibodies
What are some obstetrical obstacles for patients with lupus?
- fertility not affected
- recurrent fetal loss (due to antiphospholipid antibodies)
- babies small for gestational age
What is a major cardiac problem that is found in patients with lupus?
extensive coronary atherosclerosis involving MAJOR CORONARY ARTERIES that can lead to an MI
What are common autoantibodies found in Sjogren’s syndrome?
ANA (85%)
Anti-Ro (SSA)
Anti-La (SSB)
What are the complement levels like with Sjogren’s syndrome?
Low blood levels of C3 and C4
What is keratoconjunctivitis sicca?
keratitis caused by decreased lacrimal secretions
How do you diagnose Sjogren’s syndrome?
Schirmer’s test
What are oral symptoms of Sjogren’s syndrome?
- Severe dry mouth
- Multiple dental caries
- Fissuring/ulceration of lips/tongue/buccal membranes
- Parotid/submandibular salivary gland enlargement
What is primary Sjogren’s syndrome v. secondary Sjogren’s syndrome?
Primary is without another autoimmune disease; Secondary Sjogren’s syndrome (complication of another autoimmune CT disease- RA nad SLE)
If a patient has a swollen parotid gland, what should you do?
obtain a biopsy
What cancer is associated with Sjogren’s syndrome?
Non-Hodgkin’s lymphoma
What is the most common renal finding with Sjogren’s syndrome?
renal tubular acidosis
What autoantibody is associated with limited SS?
Anti-centromere antibody
What autoantibody is associated with diffuse SS?
Anti-topo1 (Scl70)
What are the clinical associations with Anti-topo1?
pulmonary fibrosis
heart involvement
What are the clinical associations with Anti-centromere antibody?
severe digital ischemia
PAH
sicca syndrome
calcinosis
If someone with lupus has SS, what antibody would likely be found?
U1-RNP
What are characteristics of diffuse scleroderma?
initial wide-spread skin involvement with rapid progression and early visceral involvement (better survival rate)
What are characteristics of limited scleroderma?
relatively mild skin involvement (only fingers and face) with visceral involvement late (CREST SYNDROME)
What is CREST syndrome?
calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia
What is the underlying pathogenesis of scleroderma?
T cell mediated disease that attacks vasculature and stimulates fibroblasts
How are B cells involved in scleroderma?
produce autoantibodies
What are the earliest pathological changes in SS?
endothelial cell abnormalities (increased apoptosis, upregulation of MHC class II, and increased ICAM-1 expression on endothehlial cells)
Why should you encourage your SS patients to stop smoking cigarettes?
digital artery occlusion occurs with SS (and is worsened by smoking)
What are the color changes that occur with Raynaud’s phenomenon?
white–> blue –> red
What are total body vasospastic attacks in response to cold called?
total body Raynaud’s
What is Raynaud’s phenomenon?
condition that affects blood vessels in extremities (can be primary or secondary related to autoimmune diseases)
What are characteristics of nail bed vasculature in Raynaud’s patients?
- Drop out of capillary number
- Dilated capillary loops
What is vitilgo-like changes in SS?
“salt and pepper” discoloration usually over places of fibrotic change
What form of SS is PAH most associated with?
late limited form (strange)
What is en coup de sabre SS?
Localized scleroderma that is usually in children–go down into muscles/bone and can affect brain development
What is the autoantibody profile found in MCTD?
Elevated Anti-U1-RNP
Positive ANA
Negative anti-sm, anti-ro, and anti-la
What is the largest cause of future morbidity and mortality in a patient with MCTD?
pulmonary hypertension
What do the hands of a patient with MCTD look like?
puffy, edematous hands that ache
What finding is seen in almost all patients with MCTD?
Raynaud’s phenomenon
