What is Raynaud's syndrome?
It is vasospasm usually in response to cold weather.
Resultant colour changes occur commonly in the fingers
When may anti-phospholipid syndrome be suspected?
- Young (<50) with occurence of 1 or more thrombosis
- Foetal death after 10 weeks due to pre-eclampsia or multiple embryonic losses
- Unexplained thrombocytopenia
What is pre-eclampsia?
High blood pressure during pregancy that can cause brain damage.
The high blood pressure may also impair kidney and liver function, cause seizures and result in pulmonary oedema.
It can result in miscarriage and in severe cases mother and foetal death
Livedo reticularis is a skin feature of which condition?
What is the main treatment for anti-phospholipid syndrome?
- Heparin, fragmin and aspirin are options during pregancy
Which medication can be given to those with anti-phospholipid syndrome that aids the endothelium?
(it reduces IFN alpha)
What is Sjogren's disorder?
A chronic autoimmune inflammatory disorder causing diminished lacrimal and salivary gland functioning.
This results in dry mucous membranes all over the body but especially dry eyes and mouth
Why do people with Sjogren's disorder often have poor dental condition?
Saliva is not produced
Proteolytic enzymes cannot enter the mouth
Bacterial growth is easier
Which antibodies are associated with Sjogren's disorder?
(Anti-Ro and anti-La are types of anti-RNP)
What is Schirmer's test?
A test used to determine the severity of dry eyes.
Filter paper is put beside the eye and if liquid makes it 10mm down the paper in 5 minutes this is normal, otherwise the patient has dry eyes
What are punctal plugs?
Plugs insterted into the tear ducts to prevent drainage of fluid
This is a treatment used in people with dry eyes e.g. Sjogren's
How can Sjogren's disorder be treated?
- Artificial tear supplements
- Ciclosporin eye drops
- Punctal plugs
- Saliva supplements
- Pilocarpine (for dry mouth)
- Hydroxychloroquine (efficacy is questioned)
- Immunosupression e.g. methotrexate
Systemic sclerosis causes which two main changes?
The autoimmunity causes:
- Vascular endothelial changes
Diffuse cutaneous systemic sclerosis affects which parts of the body?
Proximal to the forearms and includes torso
Which is more common in patients with diffuse cutaneous systemic sclerosis?
ILD or pulmonary hypertension?
(pulmonary hypertension is a common finding in systemic sclerosis)
Which antibodies are associated with diffuse cutaneous systemic sclerosis?
- Anti-RNA III polymerase
In systemic sclerosis which lung condition is common?
What is the skin distribution like in systemic sclerosis?
Distal to elbows
Does not involve torso
Systemic sclerosis is associated with which other multi-system conditions?
- Raynaud's (90% of patients have this)
- Oesophageal dysmotility
- Small intestinal bacterial overgrowth
Which antibodies are associated with systemic sclerosis?
How can Raynaud's syndrome be treated?
Calcium channel blockers
Phosphodiestase enzyme inhibitors
If lung disease is present with systemic sclerosis, what is the treatment?
Pulmonary hypertension associated with systemic sclerosis can be treated in what way?
Calcium channel blockers
Endothelin receptor antagonists
Reflux associated with systemic sclerosis can be treated with what?
Protein pump inhibitors
H2 receptor antagonists
Mixed connective tissue disease is associated with which antibody?
Polymyositis is associated with which antibody?