Connective Tissue Disease Overview Flashcards
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?
Anti-phospholipid syndrome
What is the main treatment for anti-phospholipid syndrome?
Lifelong anticoagulation
- Warfarin
- Heparin, fragmin and aspirin are options during pregancy
Which medication can be given to those with anti-phospholipid syndrome that aids the endothelium?
Hydroxychloroquine
(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-nuclear antibody
Anti-Ro
Anti-La
(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
- Fibrosis
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?
ILD
(pulmonary hypertension is a common finding in systemic sclerosis)