Autoimmune connective tissue diseases Flashcards
Read the following:
Included under this heading are SLE, systemic sclerosis. Sjogren’s syndrome, Idiopathic inflammatory myopathies etc.
They all overlap with each other, affect many oran systems and often require immunosuppressive therapies.
Consider as a differential in unwell patients with multi organ involvement, especially if no infection.
Read the following:
Included under this heading are SLE, systemic sclerosis. Sjogren’s syndrome, Idiopathic inflammatory myopathies etc.
They all overlap with each other, affect many oran systems and often require immunosuppressive therapies.
Consider as a differential in unwell patients with multi organ involvement, especially if no infection.
Systemic Sclerosis:
It is an autoimmune disease.
What is damaged?
Microvascular damage
Fibrosis of skin +/- internal organs
Systemic Sclerosis:
What is another name for skin fibrosis?
What sex is it more common in?
There are 2 types - diffuse and limited. Where is affected in both?
Scleroderma
There is always some degree of organ involvement so the term systemic sclerosis is generally preferred.
Females
Diffuse - can involve the whole body - chest, abdo
Limited - face, hands, feet
Systemic Sclerosis - Presentation:
What happens in scleroderma?
It commonly affects the fingers.
- What is this called?
- What could it lead to?
- What else do they get on their fingers?
It can cause microstomia in the face. What is it?
What sort of appearance does the skin have?
Thickened, firm tight skin
Fingers - sclerodactyly
Finger contractures
Ulcers
Fingertip pitting scars
Puffiness
Pruritis
A small mouth due to fibrosis
Shiny skin - they may also have hyper/hypopigmentation
Systemic Sclerosis - Presentation:
CREST syndrome is no longer considered a clinically distinct subtype of SSc, it is a useful mneumonic of some key features through.
C - Calcinosis - What is it?
R - affects your circulation. It causes some areas of your body, like your fingers and toes, to change colour when you’re cold or stressed. This is usually the FIRST presentation. What is it?
Esophageal dysmotility can occur. What can this lead to?
Sclerodactyly - What is it?
T - a condition in which widened venules (tiny blood vessels) cause threadlike red lines or patterns on the skin can occur. What is it called?
Calcinosis is the formation of calcium deposits in any soft tissue. It is a rare condition that has many different causes.
Raynaud’s syndrome
Reflux
Dysphagia
Oesophageal strictures or dilation
Sclerosis of the fingers
Telangiectasia
Systemic Sclerosis - Presentation:
It affects the GI tract. What does it cause?
How may it present in the lungs?
It can also affect the kidneys (CKD) and the heart!
Abdo pain Bloating Diarrhoea Vomiting Malabsorption
Interstitial lung disease (SOB, cough)
Pulmonary hypertension
Systemic Sclerosis - Investigations:
You need bilateral finger disease and other auto-antibodies to make a diagnosis!
What can be used to visualise abnormal capillaries in the nail folds which could aid diagnosis?
Tests for disease extent also needs to be done.
FURTHER INV needed for systemic disease
ANTIbody that can be tested to confirm?
Nail fold capillaroscopy
Scl-70 antibody/RNA polymerase III antibody
Systemic Sclerosis - Management:
Skin and connective tissue disease:
- What type of treatment is given?
- Why are emollients and antihistamines used?
- What drug is given for Raynaud’s?
- What can be done for calcinosis?
- What is done for ILD?
Internal organ disease is managed by their varying specialities.
Surgical removal
Immunosuppressive (e.g. methotrexate)
Itch - they get itchy fingers
Calcium channel blockers - (e.g. amlodipine) - same Rx for digital ulcers
Cyclophosphamide IV - chemo agent
Mixed connective tissue disease - what is it?
Combination of systemic sclerosis, SLE and polymyositis
