Systemic Lupus Erythematosus Flashcards
What is SLE?
- A collagen vascular disease
- Autoimmune disease
What are some common histological features of SLE?
- Inflammatory damage to connective tissue and blood vessels (due to the fibrosis that collects)
- Fibrinoid material deposition
What cells are involved with the immune response that SLE causes?
- Lymphoid stem cell
- B-cell
- T-cell
- Macrophage, monocyte
- Natural killer cells
What is the prevalence and demographic of SLE?
- 9 to 1 female to male (85% female)
- More common in black women
- Peak age is 15-25
When do most causes of SLE develop?
- Most develop between menarche and menopause
What are some of the genetics factors that play into SLE?
- HLA DRB 2/3 classes
What are some bodily responses that SLE causes?
- Widespread inflammation in multiple organs, blood vessels and other connective tissues
What is the inflammation caused by?
- Profound immune alteration that leads to the development of T cell and antibody responses
- The tissue damage occurs as a result of cell mediated immune response and direct damage caused by auto-antibodies or accumulation of immune complexes
What causes the symptoms of SLE?
- The symptoms are a direct result of the formation of the immune complexes that deposit within various tissues
- The immune complexes then recruit complement and inflammatory cells to cause disease
What type of hypersensitive is SLE?
Type III
* due to the formation of and collection of the immune complexes
What are the different types of hypersensitivity reactions?
Type I: Reaction mediated by IgE
Type II: cytotoxic reaction mediated by IgG or Ig antibodies
Type III: reaction mediated by immune complexes
Type IV: delayed reaction mediated by cellular response
What is the etiology of SLE?
The cause is unknown
*Thought to be a multi-faceted etiology
*possibly due to genetics or environmental
What is the clear gender and cultural bias for SLE?
- Women are 10 times more likely than men to get SLE
- Black women and Hispanic women are more likely to get SLE than whites women
What are the environmental factors that can trigger SLE?
- UV light (someone in remission that was out in the sun can get a SLE flare up)
- Infectious agents
- Certain drugs
*Drug induced lupus DOES NOT mean SLE
*Drug induced lupus is a flare up
What are the SLE clinical manifestations due to?
- Trapping of antigen-antibody complexes in capillaries of visceral structures (causing damage to organs)
- Autoantibody-mediated destruction of host cells
What leads to complement-mediated lysis?
- The auto antibodies that are specific for RBC and platelets
What is the criteria for diagnosing SLE?
- If 4 or more of the 11 criteria are present, serial, or simultaneously during any interval of observation
*The patient may have SLE and not have 4 of the criteria
What is the pneumonic for the SLE criteria?
M D S O A P
B R A I N
What is M for?
Malar rash
*Butterfly rash
*bridge of nose, checks, forehead, chin
What is D for?
Discoid rash
*raised circular rash
*on sun exposed areas
What is S for?
- Serositis
* pleuritis, pleural effusion
*Pericarditis, pericardial effusion
*Ascites (fluid in the abdomen, liver involvement)
What is O for
Oral ulcers
What is A for?
- Arthritis (With swelling)
*Not just joint pain, there will also be swelling - Arthritis (jaccouds)
*happens during a flare
*will go away during remission
What does P stand for?
Photosensitivity
* in sun exposed areas
*looks like a sunburn
What does B stand for?
Blood abnormalities *bc the body is attacking itself and the blood cells
1. Leukocytopenia (<4,000 on 2+ occasions)
2. Lymphopenia (<1500 on 2+ occasions)
3. Hemolytic anemia
4. Thrombocytopenia (<100,000)
What does R stand for?
Renal
1. Proteinuria (>500mg/24hrs)
2. Cellular casts
What does A stand for?
Anti-nuclear antibodies (ANA)
1. Rim
2. Diffuse
3. Nuclear
4. Speckled (less conclusive)
What does I stand for?
Immune abnormalities
*Get an ANA first and if that is really high order these test
1.Smith antibody
2. Anti-ds (double stranded) DNA antibody
3. Anti-phospholipid antibody
*Anti-cardiolipin antibody (IgG or IgM)
*Biologic false positive VDRL
*Lupus anticoagulant
What does N stand for?
Neurologic *change in a persons baseline behavior
1. Seizure
2. Psychosis
What are the head and neck manifestations of SLE?
- Malar rash is usually the first sign
- Erythematous maculopapular eruption after sun exposure
- Oral ulceration
- 3-5% perforated nasal septum
- Acute parotid enlargement 10%
*if there is parotid enlargement and joint pain (90%) then SLE
*ddx sjogren syndrome
What is drug induced lupus?
- Nephritis and CNS features not present
- Hypocomplementemia and antibodies to double-stranded DNA are absent
- When drugs are discontinued person will return back to normal
- ANA is positive for anti-histone (does not happen with SLE)
What is a common feature of SLE?
Joint pain
* 90% of patient have joint pain
*will not be erosive
*reversible swan neck occurs during flares goes away once in remission
What labs should you order if you’re suspecting someone with SLE?
- CBC with diff
- Serum creatinine
- UA with urine sediment
- Serum protein electrophoresis (not common, will not be the first lab you order)
- ESR, CRP
- ANA
- Antibodies to double-stranded DNA (correlates with severity) and Smith
- Depressed serum complement (C3 and C4 or CH50)
What is definite, probable, and possible SLE?
D: meets 4/11, 4/17 of the criteria
Probable: 2-3
Possible: 1 or 2
What are the current therapies for SLE?
- Immunosuppressants
*cyclophosphamide
*Azathioprine
Do therapies cure SLE?
No they only alleviate symptoms
What is the first line treatment if SLE involves joint symptoms and skin symptoms?
- Antimalarials *hydroxychloroquine-plaquenil
*have annual monitoring for retinal changes
When would corticosteroids be used for SLE treatment?
- If there is a life-threatening manifestations due to major organ involvement
- Start with lowest dose possible
What is the first line treatment for SLE if there is issues with the kidney or systemic issues?
- Low dose methotrexate
- Azathioprine, cyclophosphamide *improves renal survival but not patient survival
*use if resistant to corticosteroids
What are the biologics treatment for SLE?
- Belimumab
- Rituximb
*only if the patient has tired everything, a last resort
What is the prognosis for SLE?
- Usually a bimodal mortality pattern
*infection in early yers=death
*Atherosclerosis in later years=death - Risk of AVN (Avascular necrosis)
*Bone is dying to the lack of blood supply
*Prednisone increases risk of AVN
