Connective Tissue Disease - Systemic Lupus Erthematous Flashcards
What are connective tissue diseases?
Diseases characterized by the presence of spontaneous over activity of the immune system.
Not diseases of connective tissue
Which part of the body does SLE affect?
Any part of the body
Systemic lupus erythematous is an example of which type of hypersensitivity reaction?
Type III
(Immune complex mediated)
Which types of people are more susceptible to SLE?
- Females
- Hispanic americans
- Asians
- Afro-Carribeans
What hormone is thought to be associated with increased chances of developing SLE?
Oestrogen
Which environmental factors may trigger SLE?
- Smoking
- Silica dust or cement
- UV light
Which virus is known to induce SLE?
Epstein-Barr virus
What is the pathophysiology behind SLE?
- There is an increase in self cell death
- Defective apoptosis occurs
- Necrotic release of nuclear material occurs
- There is delayed clearance of nuclear material and autoantibodies are made against this material
- Immune complex formation occurs
Why is renal disease a major worry in SLE?
It is symptomless
Eventuallu will lead to necrosis and scarring
Why does SLE lead to necrosis and scarring in the kidneys?
- Immune complexes are deposited in the mesangium
- Complement is activated, leukocytes and other inflammatory cells are attracted
- Necrosis and scarring results
Where may ulcers be found in a patient with SLE?
Mouth (or nasal) ulcers
How is SLE diagnosed?
SLICC classification
(>= 4 criteria with at least 1 clinical and 1 labratory investigation required for diagnosis OR biopsy proven lupus nephritis and the presence of ANA or Anti dsDNA)
SLE can be associated with thrombocytopenia, what is this?
A low platelet count
How does discoid lupus erythematous differ in terms of facial rash, with SLE?
There is a scaly surface to the rash, which is also generally more well demarkated
How can the hair be affected in SLE?
Alopecia can occur
Describe the facial rash of SLE and the area(s) it avoids
- Butterfly malar rash
- Space naso-labial folds
- Can be associated with UV light exposure
Jaccoud’s arthritis is associated with SLE, what is it?
Inflammation of the joint capsule
- MCP joint subluxation (of 2nd to 5th digits) and hyperextension at the related PIP joints
- This is deforming, yet reversible
In SLE it is not uncommon for patients to present with percarditis or pleural effusion. Why is this?
SLE can cause serositis
If SLE impacts the kidneys, causing lupus nephritis, what characteristic features will be shown on urinalysis?
- Proteinuria of >500mg in 24 hours
- Red cell casts (blood in urine)
If either of these features is present then renal biopsy is required
What are some common neurological features of SLE?
- Headache/migraines (most common)
- Depression
- Seizures
- Foot drop
- Mononeuritis multiplex
- Cranial or peripheral neuropathy
What are some haematological symptoms in SLE?
- Lymphadenopathy
- Leukopenia
- Lymphopenia
- Thrombocytopenia
- Haemolytic anaemia
What does anti-phospholipid syndrome involve?
Thrombosis of arteries and/or veins
What is the term given to the mottled, net like skin appearance seen in patients with anti-phospholipid syndrome?
Livedo reticularis
Which antibody is present in almost all SLE patients, but has a relatively low level of specificity?
ANA
(anti-nuclear antibody)
