Connective Tissue Diseases Flashcards
How should mild cases (i.e. cutaneous manifestations and arthralgia) of SLE be managed?
Simple analgesia, topical steroids and hydroxychloroquine
Who is Sjogren’s syndrome most likely to occur in?
Women in their 50s/60s
How should moderate cases (i.e. inflammatory arthritis and minimal organ involvement) of SLE be managed?
Simple analgesia, oral steroids, hydroxychloroquine, azathioprine/methotrexate
How should severe cases (i.e. major organ involvement) of SLE be managed?
Simple analgesia, IV steroids, hydroxychloroquine, cyclophosphamide +/- rituximab
How is Sjogren’s syndrome diagnosed?
Positive Schirmer’s test (confirming dry eyes), typical features on lip gland biopsy and the presence of anti-Ro/anti-La antibodies
What type of hypersensitivity reaction is SLE and why?
Type III- it is immune complex mediated
Which connective tissue disease may present with unexplained dental caries?
Sjogren’s syndrome
How are each of the following manifestations of systemic sclerosis managed: a) Raynaud’s? b) renal involvement? c) GI involvement? d) interstitial lung disease?
a) Ca++ channel blocker b) ACE inhibitor c) PPI d) cyclophosphamide
Apart from the malar rash, name some cutaneous features of SLE?
Oral/nasal ulceration, Raynaud’s phenomenon, alopecia
What are some important organ manifestations of systemic sclerosis?
Pulmonary hypertension, pulmonary fibrosis and accelerated hypertension (leading to renal crisis)
Having Sjogren’s syndrome increases a person’s risk of developing which other disease?
Lymphoma
More than 90% of cases of SLE occur in women- when does it usually present?
At childbearing age (typically 20s and 30s)
How is Sjogren’s syndrome managed?
Generally with lubricating eye drops and saliva replacement; hydroxychloroquine may be helpful for arthralgia/fatigue
Why are a CXR and PFTs performed on any patient with suspected SLE?
To look for evidence of any interstitial lung disease
What investigations should be performed if someone is suspected to have SLE?
Routine bloods, antibody screen, C3/4 levels, urinalysis, CXR and PFTs, ECHO
Which connective tissue disease is characterised by lymphocytic infiltrates in exocrine organs?
Sjogren’s syndrome
In addition to anti-dsDNA, what is another antibody which is specific for SLE but has low sensitivity?
Anti-Sm
What is the risk of being Anti-Ro positive during pregnancy?
Can cause foetal heart block, this must be monitored with in-utero ECHOs
Name some general features of most connective tissue diseases?
Fever, weight loss, fatigue
Describe the features of limited systemic sclerosis?
Skin involvement of the face and distal arms/legs; organ involvement occurs late
Limited systemic sclerosis can sometimes be known as CREST syndrome- what are the features of this?
Calcinosis, Raynaud’s, oesophageal dysmotility, sclerodactyly, telangectasia
Describe the features of diffuse systemic sclerosis?
Skin involvement may include the proximal limbs and torso, significant organ involvement occurs early
When investigating for anti-phospholipid syndrome, as well as antibodies, what other features may be seen on blood tests?
Thrombocytopenia and prolonged APTT
What is the most important investigation in someone with suspected SLE and why?
Urinalysis- to detect evidence of glomerulonephritis as this shows no clinical features
What are the anti-phospholipid antibodies?
Anti-cardiolipin, lupus anticoagulant and anti-beta 2 glycoprotein
What are the main features of anti-phospholipid syndrome?
Recurrent venous/arterial thromboses and/or foetal loss
What antibody is specific for SLE?
Anti-dsDNA
Why is an ECHO performed on any patient with suspected SLE?
To look for evidence of any pulmonary hypertension
If a patient with SLE wanted to get pregnant, which antibody should be checked for and why?
Anti-phospholipid antibodies- if these are present then they should be given LMWH and aspirin during pregnancy until 6 weeks post-partum
Which antibody is present in > 95% of patients with SLE but is not specific?
ANA
It is unusual to have systemic sclerosis without having what feature?
Raynaud’s phenomenon
What is a common cutaneous finding of patients with anti-phospholipid syndrome?
Livedo reticularis
What happens to complement (C3/4) levels in active SLE?
They decrease
Organ screening is performed regularly in patients with systemic sclerosis- what does this involve?
PFTs, ECHO and urinalysis
Mixed connective tissue disease is mostly associated with which antibodies?
Anti-RNP
What investigations can be used to monitor patients with SLE?
Anti-dsDNA levels, C3/4 levels, urinalysis, evaluation of CV risk with BP and cholesterol levels
Do patients who have positive anti-phospholipid antibodies but no previous thrombosis require treatment?
No
For APS to be diagnosed, there must be persistent lab evidence of anti-phospholipid antibodies- what is meant by this?
These must be positive on two occasions, at least 12 weeks apart
What is the mainstay of treatment for APS?
Anticoagulation
If someone with suspected SLE has haematuria or proteinuria on urinalysis, what investigation should be done next?
Renal biopsy
What advice would you give to someone with SLE who wanted to get pregnant?
Ideally, they would be free from active disease for at least 6 months before getting pregnant
Systemic sclerosis is diagnosed when a patient has 1 major and 2 minor features- what are these?
Minor = sclerodactyly, atrophy of the fingertips, bilateral lung fibrosis / major = skin sclerosis affecting the arms, face and/or neck
What are the most common symptoms of Sjogren’s disease?
Dry eyes and mouth (parotid gland swelling and vaginal dryness may also occur)
A malar (butterfly) rash is typically seen in which condition? What are some features of this rash which differentiate it from other conditions?
SLE - it is usually photosensitive and always spares the naso-labial folds
What autoantibody is suggestive of drug-induced lupus?
Anti-histone antibodies
