Connective tissue disease 1 Flashcards
SLE is commercial in African Americans T/F
T
Male to female ratio for SLE in 9-1 T/F
F- commercial in women. however if it does occur in men it tends to be more severe
Examples of CTD
SLE Sjogen's syndrome Sytemic sclerosis Dermatomyosititis Polymyosititis Mixed connective tissue disease Anti-phospholipid syndrome
Connective tissue diseases are diseases of the connective tissue T/F
F- characterised by the presence of over activity of the immune system
What is SLE
Immune system attacks the body’s cells and tissue, resulting in inflammation and tissue damage
Why does kidney disease cause so many deaths?
It often does not cause any symptoms so can go unnoticed ( even though very severe- kidney can stop working entirely)
Which three factors are inportant in the aetiology of SLE and why?
Genetic- monozygotic twins, relatives, gene abnormalities
Hormonal- higher oestrogen exposure(early menarche) , on oestrogen containing contraceptives and HRT
Environmental- Viruses, UV light( may stimulate skin to release cytokines stimulating B cells) , Silica dust
What is the pathogenesis of SLE
Increased and defective apoptosis
necrotic cells release nuclear material which acts as potent auto-antigens
Autoantibody probably as a result of extended exposure to nuclear and intracellular organisms
B cells and T cells stimulated
Auto-antibodies produced
What are constitutional symptoms?
Very common - but not diagnostic ( as they are so common)
Why is there renal disease with SLE?
deposition of immune complexes in mesangium
Comlexes consist of nuclear antigens and anti-nuclear antibodies
Complexes form in circulation then are depoisited
Once present they active competent which activtes leukocytes which releases cytokines.
Cytokines release perpetuates inflammation which, over time, causes necrosis and scarring
Describe the rash associated with SLE
spares the nasallabial folds
Lasts long time
butterfly
How is Jaccoud’s arthritis different to —-. SECTIONMISSING
Reversible and if x-rays there is no change to the joints
What are the mucocutaneous features of SLE?
Photosensitivity Malar rash Discoid lupus erythematous( may scar) Subacute cutaneous lupus Mouth ulcers ( painless) Alopecia ( non-scarring)
What are you looking for in terms of SLE in a dipstick
Looking for blood or protein in urine
What are the MSK features of SLE
Non-deforming polyarthiritis/polyarthralgia (no radiological erosion)
Deforming arthropathy-Jaccoud’s arthritis
Erosive arthritis-rare
Myopathy-weakness,myalgia and myosititis
What is serosititis and what problems can it cause?
inflammation of the serous tissues of the body, the tissues lining the lungs (pleura), heart (pericardium), and the inner lining of the abdomen (peritoneum) and organs within.
Pericarditis
Pleurisy
Pleural effusion
Pericardial effusion
What are the neurological features of SLE?
Depression/psychosis migraninous headache seizures cranial or peripheral neuropathy mononeuritis multiplex
What are the haematological features of SLE
lymphadenopathy -(25% of patients) Leucopenia Lymphopenia Haemolytic anaemia Thrombocytopenia
Organ involvement that involves treatment but is not life threatening- where would this lie on the spectrum of disease
Moderate
mild- may not need treatment, severe os life threatening
What is anti-phospholipid syndrome
Venous and arterial thrombosis Recurrent miscarriage Lividio recticularis Assocaitions with autoimmune conditions especially SLE Thrombocytoenia Prolonged APTT
Why doe so many people with CTD have infections?
Intrinsic-low complement, impaired cell mediated immunity, defective phagocytosis, poor antibody response to certain antigens
Exrtinsic-Steroids, other immunosuppression drugs, nephrotic syndrome
What is ANA
Anti-nuclear antibody
High titre In 95% of SLE patients
Low titre in 20% of population
Found in rheumatoid arthritis and other autoimmune conditions, HIV, hep c
When should a positive NA be taken seriously?
if other antinuclear antibodies are positive Anti-dsDNA Anti-Sm Anti-Ro Anti-RNP
When the patient presents with CTD features
What is anti-double stranded DNA antibody?
Occurs in 60% of patients with SLE-Highly specific
Titre correlated with overall disease activity
may be associated with lupus nephritis
What antibody is most specific to SLE
Anti-double stranded DNA antibody
What is Anti-ENA
Anti-Ro (60%)
Usually associated with anti-La
Associated with cutaneous manifestations
Secondary Sjogen’s features
Congenital heart block and neonatal LE
Anti-Sm highly specific (10-20%)- probable neurological
Anti-RNP(30%)- overlap potentially with sclerodermatous skin lesions, Raynaud’s pnenonenom, low grade myosis
What would be the most appropriate first investigation in a patient with suspect SLE
Urinalysis
What are Anti-phospholiod antibodies
Anti-cardiolipin antibody
Lupus anticoagulant
Anti-beta 2 glycoprotein
Must be positive on 2 occasions 12 weeks apart
Normally as disease flares CRP increases. What disease is an exception to this?
SLE - it CRP is high indicated infection
Once diagnosis is established…
Essential to screen for organ involvement
What other investigations may be necessary?
CXR pulmonary function tests CT chest Urine protein quantification Renal biopsy Echocardiogram Nerve conduction studies MRI brain
What is the drug that everyone with SLE gets
Hydroxychloroquine- useful for arthritis,cutaneous manifestations and constitutional symptoms. may reduce sys complications
How do you monitor levels of SLE activity?
Clinical assessment inc BP Anti-dsDNA level pos correlates C3/C4 level neg correlates Urien exam inc portens, cells and casts Full blood count Blood biochemistry
What is the general management of SLE
Councelling- patients, spouse and relatives
Regular monitoring
Avoid excessive sun-exposure
Pregnancy issues
When are steroids used?
Steroids could be used for inflammatory drug problems etc. Small doses for rates,arthiritis and serotitis Moderate for resistant serosititis, haematological abnormalities and class V GN High doses for severe/resistant haematological changes, diffuse GN and major organ involvement
What is the most common immunosuppressive used?
-Cyclophosphamide (methotrexate can also be used)
What do you use to treat a mild disease?
HCQ
Topical steroids
NSAIDs
Moderate disease treatment
Oral steroids
Azathioprine
Methotexate
Severe disease treatment
IV steroids
Cyclophosphamide
Rituximab
What are the biologics that can be used in SLE
Anti-CD20 (Rituximab)
Anti-Blys (Belimumab)
