Connective Tissue Disorders Flashcards
What are the 4 questions to ask a patient you suspect of SLE (systemic lupus erythematosus)?
- Extreme fatigue?
- Mallar rash?
- Recurring ulcers - oral or nasal?
- Frontal balding/bald patches?
Who is most likely to present with SLE?
Woman aged 20-30 yo (believed to be some connection with oestrogen)
What antibody is used as a diagnostic tool in SLE?
ANA (anti nuclear antibodies)
Anti-dsDNA much more specific - preferred
What antibody can be used as a monitoring tool in SLE?
Anti-dsDNA
What is notable about the malar rash present in SLE?
It is photosensitive - worsens in sunlight
Is a patient with SLE likely to present with monoarthritis?
No - polyarthritis
What causes SLE?
A combination of genetics and environment
When cells become destroyed due to e.g. sunlight
Suspectible genetics programme for auto-cell attack meaning increased levels of nuclear antigens
Increased nuclear antigens -> increase ANA
Antigen-antibody complexes stick to endothelium and cause local inflam
SLE is commoner in Afro-Caribbean populations. True or false?
True
What drives disease in SLE?
Immune complex formation
What is subacute cutaneous/discoid lupus?
Plaques in photosensitive regions similar to psoriatic plaques
What kind of hypersensivity reaction is SLE?
Type III
What is the name of the hand condition which presents as ulnar deviation but is reversible (can still flex hand if needs be)?
It is present in many connective tissue diseases esp. SLE
Jaccoud’s arthopathy
What antibody is most specific to SLE?
Anti-dsDNA
anti- double stranded DNA
What treatment will be given to all SLE?
Hydroxychloroquine - antimalarial drug
Factor 50 suncream at all times
When would you consider upping medications for SLE?
What would you add?
If organ involvement
Minimal steroid use Add immunosuppressants (similar to RA drugs) if not responding to hydroxychloroquine
What is Sjogren’s syndrome?
Autoimmune condition characterised by lymphatic infiltrates in exocrine glands
Who most commonly presents with Sjorgen’s syndrome?
Middle aged woman
How would Sjogren’s syndrome present?
With dryness of body surfaces;
- dry eyes
- dry vagina (in pre-menopausal woman)
- dry mouth (xerostomia)
- nasal ulceration
Gland swelling and pain
Dysphagia
What 3 things are done to diagnose Sjogren’s syndrome?
- Lip biopsy
- +ve anti-Ro, +ve anti-La antibodies (swollen face you could RoLa down the hill)
- Ocular dryness test - Schirmer’s test
How is Sjogren’s syndrome managed?
Symptomatically
- eye drops
- increased saliva production
Hydroxychloroquine - can prevent disease progression
What does Sjogren’s syndrome put you at increased risk of?
Lymphoma
What 4 questions should you ask a patient with suspected Sjogren’s syndrome?
In the last 3 months…
- Waking up to drink water?
- Eyes feel gritty?
- Drink large volumes of water to swallow food?
- Vaginal dryness in pre-menopausal women?
What is the most important first test in SLE?
Why?
Urinaylsis
- often asymptomatic
- changes treatment pathway
- if kidney involvement means more severe disease
What causes systemic scleorsis?
Excessive collagen production -> increased deposits in tissues
How may a patient with systemic sclerosis present?
CREST
Calcinosis Raynauds Eosphgeal dysmotility -> GORD + dyspepsia Sclerodactyly Telangiectasia
+ organ dysfunction
- Lungs -> ILD + pul HTN
- Heart -> HTN
- kidneys -> glomerulonephritis
What antibodies are assocaited with systemic sclerosis?
Anti-Scl-70 - diffuse (including organ involvement)
Anti-centromere - limited (CREST)
How is systemic sclerosis managed?
Symptomatic management
Renal -> ACE
Lung (ILD) -> immunosuppressants
GORD -> PPIs
Raynauds -> CCB (nifedipine)
What antibody is associated with MCTD?
anti-RNP
What screening measure must patients with MCTD receive annually and why?
Echocardiogram - test for pulmonary HTN
What is MCTD?
Mixed connective tissue disease
Present with symptoms of many kinds of connective tissue diseases
What is livedo reticularis?
In what condition is it found?
net-like pattern of red/blue discolouration mainly on legs
Anti-phospholipid syndrome
What CTD is associated with foetal loss + increased risk of MI/stroke at young age?
Anti-phospholipid syndrome
What are the anti-phospholipid antibodies associated with anti-phospholipid sydrome?
Lupus anticoagulant
Anti-cardiolipin
Anti-beta2 glycoprotein
What other CTD does anti-phospholipid syndrome often occur secondary to?
SLE
What is the mainstay of treatment for anti-phospholipid syndrome?
Under what circumstances is this treatment not necessary?
lifelong warfarin (only if previous thrombotic event)
If not had previous episode of thrombosis
Does a high or low complement level indicate active disease in SLE?
Low complement level -
Complement is consumed in the response to the formation of immune antigen/antibody complexes
