Flashcards in Systemic Lupus Erythematosus Deck (21)
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1
What diseases come under the category of ‘connective tissue disease’?
SLE
Systemic sclerosis
Dermatomyositis/polymyositis
Sjogren’s syndrome
Mixed connective tissue disease
2
Which gender does SLE more commonly affect?
Females 9:1
3
Describe the presentation of SLE including some specific features.
Malaise, fatigue, weight loss, fever, lymphadenopathy
Specific features:
Butterfly rash
Alopecia
Arthralgia
Long history of Raynaud’s phenomenon
4
Describe the characteristics of the rash seen in SLE.
It tends to go across the nose
It may look a bit like acne
It is not painful or itchy
Some rashes become depigmented when the inflammation spreads to the dermis (depigmentation and scarring is irreversible)
5
Describe the pathogenesis of SLE.
SLE patients have a defect in apoptosis
Apoptotic cells are not cleared properly so they persist and expose their nuclear antigens and autoantibodies are generated against these nuclear antigens
The defect in apoptosis is combined with B cell hyperactivity
The overactive B cells are exposed to the nuclear antigens and the plasma cells begin to produce autoantibodies that circulate and form immune complexes
The immune complexes deposit in tissues and activate complement leading to inflammation
6
What is the first investigation performed in the diagnosis of SLE?
Check for anti-nuclear antibodies (this is not specific for SLE though)
7
The pattern with which the antinuclear antibodies bind to the nuclear antigens is important in reaching a diagnosis. List some different patterns and the antigens they are associated with.
Homogenous – DNA
Speckled – antibodies to Ro, La, Sm and RNP
Nucleolar – topoisomerase – scleroderma
Centromere – limited cutaneous scleroderma
8
What conditions are associated with the presence of anti-Ro and anti-La antibodies?
Neonatal lupus syndrome
Subacute cutaneous lupus erythematosus
9
What are some other tests that can be done for SLE?
Measuring complement levels
Anti-cardiolipin antibodies
Lupus anticoagulant
Beta 1 glycoprotein
10
Describe the haematological features of SLE.
SLE is generally associated with low blood counts
Thrombocytopenia
Lymphopenia
Normocytic anaemia
Autoimmune haemolytic anaemia
11
What renal changes might occur in SLE?
Proteinuria
Haematuria
Active urinary sediment
12
List some clinical features that could help pre-empt severe attacks in SLE.
Malaise, weight loss, alopecia, rash
13
List some laboratory markers that could help pre-empt severe attacks in SLE.
Raised ESR
Raised anti-dsDNA antibodies
Reduced complement levels
14
Describe the differences between mild, moderate and severe disease in SLE.
Mild – skin and joint involvement
Moderate – inflammation of other organs (e.g. pleuritis, pericarditis)
Severe – severe inflammation of vital organs
15
Describe the treatment of mild disease.
Paracetamol and NSAIDs
Hydroxychloroquine (good for arthropathy and cutaneous manifestations)
Topical corticosteroids
16
Describe the treatment of moderate disease.
ORAL GLUCORTICOIDS
Start with a HIGH dose and titre downwards
17
Describe the treatment of severe disease.
Azathioprine – useful steroid-sparing drug
Has a risk of neutropenia/bone marrow suppression so needs regular blood monitoring
Cyclophosphamide – one used if there is severe organ involvement
Problem – infertility
18
Name and explain the mechanism of action of two new treatments for severe disease.
Mycophenolate mofetil
Reversible inhibitor of inosine monophosphate dehydrogenase
This is the rate limiting step in de novo purine synthesis
Lymphocytes rely heavily on de novopurine synthesisRituximab
Anti-CD20 antibody
Causes depletion of B cells
Useful in lupus nephritis
19
SLE has and early peak and a late peak in mortality. What are the usual causes of the two peaks?
Early – renal failure, CNS disease, infection
Late – MI and stroke
20
What can usually be seen on the blood film of a patient with SLE?
Schistocytes (evidence of microangiopathic haemolytic anaemia)
Teardrop cells
Spherocytes
Few leukocytes
Few platelets
21
