11/11/2024 Flashcards
What is Systemic lupus erythematosus (SLE)?
A complex multisystem autoimmune disease resulting from a type III hypersensitivity reaction involving the formation of immune complexes which can deposit in any organ e.g. skin, joints, kidneys, brain
Epidemiology of SLE?
F:M 9:1
More common in Afro-Caribbean and Asian communities
Onset 20-40 usually
Indecency has risen 3 fold in the last 50 years
What factors may contribute to the aetiology of SLE?
Genetic factors - associated with HLA B8, DR2 and DR3
environmental triggers e.g. drugs and EBV
Which haplotypes are associated with an increased risk of developing SLE?
HLA-DR2 and DR3
HLA B8
Name some environmental triggers for SLE.
- Drugs (procainamide, hydralazine, minocycline, terbinafine, isoniazid, phenytoin, sulfasalazine, carbamazepine)
- Epstein-Barr virus
What are the main risk factors for SLE?
- African American ethnicity
- Female sex (9:1)
- Childbearing age
- HLA-DR2/3 carriers
- Sunlight exposure
What are the common constitutional symptoms of SLE?
- Fatigue
- Fever
- Weight loss
- mouth ulcers
- lymphadenopathy
What is the cutaneous feature of SLE?
Malar (butterfly) rash that spares the nasolabial folds
Discoid rash = scaly, erythematous well demarcated rash in sun-exposed areas
Photosensitivity
Raynaud’s phenomenon
Livedo reticularis
Non-scarring Alopecia
What are the typical musculoskeletal symptoms in SLE?
- Non-erosive arthritis of small joints
- Early morning stiffness
What are common cardiac manifestations of SLE?
- Pericarditis is the most common
- myocarditis
- Coronary artery disease
- Libman-Sacks endocarditis (rare)
What are some pulmonary manifestations of SLE?
- Pleuritis
- Pulmonary hypertension
- fibrosing alveolitis
What serious condition can lupus nephritis lead to?
End-stage renal disease = lupus nephritis
Investigtaions for SLE?
Urinalysis for haematuria and proteinuria
FBC
ESR and CRP
ANA, Anti-dsDNA, anti-smith, anti-phospholipid antibodies
Complement levels
What haematological condition is associated with and can therefore present with lupus?
Antiphospholipid syndrome
Are complement levels low or high in SLE/
Often low during active disease as formation of immune complexes leads to consumption of complement
What is lupus nephritis?
A severe manifestation of SLE that can result in ESRD
This is why all SLE pts should be monitored with urinalysis at each check up
What is the hallmark of SLE in laboratory investigations?
Anti-nuclear antibodies -99% of pts are positive
What is the typical management for mild SLE?
Sunblock
NSAIDs
* Hydroxychloroquine is the treatment of choice
If internal organ involvement then consider prednisolone or cyclophosphamide
What are the classes of lupus nephritis?
Which is the most common?
class I: normal kidney
class II: mesangial glomerulonephritis
class III: focal (and segmental) proliferative glomerulonephritis
class IV: diffuse proliferative glomerulonephritis - this is the most common and severe form!!
class V: diffuse membranous glomerulonephritis
class VI: sclerosing glomerulonephritis
What is the 10-year survival rate for SLE?
Around 90%
Management of lupus nephritis?
Treat hypertension
If class III or IV then consider glucocorticoids with either mycophenolate or cyclophosphamide
Most common causes of drug-induced lupus?
Procainamide
Hydralazine
Others:
Isoniazid
Minocycline
Phenytoin
Causes of sideroblastic anaemia?
Congenital
Myelodysplasia
Alcohol
Lead
Anti-TB meds
Outline the Keith-Wagener classification stages of hypertensive retinopathy?
1 - arteriolar narrowing and tortuosity, silver wiring
2 - AV nipping
3 - cotton wool exudates, flame & blot haemorrhages
4 - papilloedema
What is mild non-proliferative diabetic retinopathy?
1 or more microaneurysms
What is moderate non-proliferative diabetic retinopathy?
Many Blind Humans Can’t View Images:
Microaneurysms
Blot haemorrhages
Hard exudates
Cotton wool spots
Venous beading/looping
Intraretinal micro vascular abnormalities
What is severe non-proliferative diabetic retinopathy?
Blot haemorrhages and microaneurysms in 4 quadrants
Venous beading in at least 2 quadrants
Intraretinal microvascular abnormalities in at least 1 quadrant
Features of proliferative diabetic retinopathy?
retinal neovascularisation - may lead to vitrous haemorrhage
fibrous tissue forming anterior to retinal disc
(Note 50% will be blind in 5 years)
What is diabetic maculopathy?
hard exudates and other ‘background’ changes on macula - based on location rather than severity!
More common in T2DM
When do you treat diabetic retinopathy?
When its very severe non-proliferative you can consider
Treat when proliferative retinopathy (with panretinal laser photocoagulation and intravitreal VEGF inhibitors)
What must you do if you have a pt with a long saphenous vein superficial thrombophlebitis?
Patients with long saphenous vein superficial thrombophlebitis should have an ultrasound scan to exclude an underlying DVT
20% of al cases of thrombophlebitis have an underlying DVT and the risk is linked to the length of the vein affected
Management of orbital cellulitis?
Admission to hospital for IV antibiotics due to the risk of cavernous sinus thrombosis and intracranial spread
Features that suggest orbital cellulitis rather than periorbital cellulitis?
Reduced visual acuity
Proptosis
Ophthlamoplegia
Pain with eye movements
Brain changes in alzheimers?
widespread cerebral atrophy mainly involving the cortex and hippocampus
Poor prognostic features for RA?
Rheumatoid factor positive
anti-CCP antibodies
poor functional status at presentation X-ray: early erosions (e.g. after < 2 years) extra articular features e.g. nodules
HLA DR4
insidious onset
What is a non-haemolytic febrile transfusion reaction?
When temperature rises by less than 1.5 degrees
Thought to be caused by antibodies reacting with white cell fragments in the blood product and cytokines that have leaked from the blood cell during storage
Slow or stop the infusion, give paracetamol and monitor
What is the name of Non-cardiogenic pulmonary oedema thought to be secondary to increased vascular permeability caused by host neutrophils that become activated by substances in donated blood?
TRALI
What is allergic bronchopulmonary aspergillosis?
A hypersensitivity reaction to the mould aspergillus spores in the lungs
Typically in pts with an underlying predisposition e.g. asthma or CF
Features of allergic bronchopulmonary aspergilllosis?
Wheeze, cough, dyspnoea
Bronchiectasis
History of asthma or CF. Or other hypersensitivity disorders
Investigations to order for ?allergic bronchopulmonary aspergillosis?
Skin test for aspergillus sensitivity
Serum total IgE
Aspergillus specific IgE
FBC - looking at eosinophils in particular
CXR
High resolution CT chest
Management of allergic bronchopulmonary aspergillosis?
Oral glucocorticoids
What is the Z score adjusted for on a DEXA scan?
Age, race and gender
If a pt cannot tolerate clopidogrel, what should you give them as secondary prevention following a stroke?
Aspirin 75 mg daily with modified-release dipyridamole 200 mg twice daily may be used if clopidogrel cannot be tolerated.
When should you admit or refer a pt with a burn?
Comp;lex burn injuries
Full thickness
Deep dermal burns affecting >5% of TBSA in adults (or all deep dermal burns in children)
Circumferential deep dermal burns
Any high pressure steam injury
Any burn associated with suspected NAI
If associated sepsis
Burns affecting face, hands, feet, genitalia, perineal or any flexural surface
With associated suspected inhalation injury
If significant comorbidities that would affect wound healing or increase the risk of complications
Superficial epidermal burns >15% TBSA in adults or >10% in children OR if signs of heat exhaustion/heat stroke
Some contraindications to thrombolysis?
Recent haemorrhage, trauma or surgery
Ischaemic troke in last 3 months
Known structural cerebrovascular lesions eg.. neoplasms
Any prior intracranial haemorrhage
Bleeding disorders
Recent acute cerebrovascular event
Features of cavernous sinus syndrome?n
Proptosis
Ocular and conjunctival congestion
Ophthalmoplegia - CN6 usually damaged before CN3 or CN4
Trigeminal sensory loss - may lead to hyperaesthesia of upper face and eye pain
Horners syndrome
What is most common cause of cavernous sinus syndrome?
Maligannt tumours - mostly nasopharyngeal
Imaging for intracranial venous thrombosis?
MRI venography is gold standard
Gender and age at greatest risk of cerebral venous thrombosis?
Young/middle-aged females
What is Guillain-Barré syndrome?
An acute, self-limiting autoimmune disease triggered by a preceding viral or bacterial infection, and characterised by progressive muscle weakness and respiratory paralysis associated with absent deep tendon reflexes
It develops over 3-4 weeks
What is the pathogenesis of GBS?
cross-reaction of antibodies with gangliosides in the PNS = demyelination of the nerves
Most common causes of GBS?
Campylobacter jejuni is the most common
CMV - particuarly in young women presenting with prominent involvement of sensory and cranial nerves
Mycoplasma pneumonia
EBV
VZV
HIV
Rabies vaccine
What is Miller Fisher syndrome?
A variant of GBS
Associated with ophthalmoplegia, areflexia and ataxia - eye muscles usually afefcted first!
A descending paralysis!
Features of guillain barre syndrome?
Initially: back or leg pain
Progressive symmetrical weakness of all limbs that is classically ascending - often more pronounced in proximal muscles
Reduced or absent reflexes
Mild sensory Sx
Respiratory muscle weakness
CM involvement e.g. diplopia, B/L facial nerve palsy, oropharyngeal weakness
Autonomic - urinary retention & diarrhoea
Investigations for guillain barre sundrome?
FBC, ESR, U&Es, LFTS
LP - rise in protein but normal WCC
Nerve confusion studies show decreased motor nerve conduction velocity, prolonged distal motor latency and increased F wave latency
May do serum or stool culture for campylobacter jejuni
Note spirometry must be measured every 6 hours initially at the bedside looking at vital capacity
Management of guillain barre syndrome?
IVIG or plasma exchange is first line with supportive treatment
What is chronic fatigye syndrome also known as?
Myalgic encephalomyelitis
What is transverse myelitis?
Inflammation of a transverse segment of the spinal cord causing an acute loss of sensory, motor and bladder function below the level of the lesion
It can be idiopathic, post-infectious, caused by MS, autoimmune disorders, neuromyelitis optica spectrum disorder
What is stiff person syndrome?
A rare, immune-mediated neurological movement disorder characterised by rigidity, stiffness and sudden painful spasms particularly in the axial and proximal appendicular muscles leading to severely impaired ambulation
Its incurable
What antibody is done for stiff person syndrome?
Anti-GAD
What is cataracts?
Opacification/clouding of the lens which makes it more difficulty for light to reach the retina = reduced or blurry vision
Found in 75% of people over 65
What are the 3 main types of cataracts?
Nuclear cataracts - sclerosis of lens nucleus & commo in old age
Cortical cataracts = opacifications of lens cortex
Posterior subcapsular cataracts - opacifications in posterior aspect of lens capsule. More common in young pts and indicates taking steroids
How do nuclear cataracts present compared to posterior subcapsular cataracts?
Nuclear cataracts can cause myopia so pts will often say they previously needed reading glasses but no longer require them
Posterior subcapsular cataracts cause a glare when looking at night. Often progresses rapidly
risk factors for cataracts?
Ageing - most common cause
Smoking
Systemic disease: DM, myotonic dystrophy, atopic dermatitis, neurofibromatosis type 2
Corticosteroid use long term
UV exposure
Ocular trauma
Genetics
Eye disease e.g. glaucoma, high myopia, retinitis pigmentosa, anterior uveitis etc
Clinical features of cataracts?
Gradual and painless reduction in visual clarity and sharpness
Faded colour vision
Glare
Halos around lights
Defect in red reflex and clouded lens
Investigations for cataracts?
Ophtlamoscopy - should be normal
Slit lamp examination to see the cataract
Tx of cataracts?
Non-surgical - stronger glasses & contact lenses, encourage use of brighter lighting, control risk factors
Surgery is the only effective treatment - this decision should be dependant on the impact of QOL and pt choice
What is the most common cataract surgery?
Phacoemulsification with an intraocular lens implant
Side effects of TCAs?
Antimuscarinics SE: dry mouth, blurred vision, constipation, urinary retention, postural hypotension
Some are sedative e.g. amitryptiline, clomipramine
Cardiotoxic in OD - can lengthen QT interval
What causes Wilson’s disease?
Autosomal recessive disorder characterised by excessive copper deposition in tissues
Caused by a defect in ATP7B gene on chromosome 13
Features of Wilson’s disease?
Liver: hepatitis, cirrhosis
Neurological - speech, behavioural and psychiatric problems. Asterixis, chorea, dementia, Parkinsonism
Kayser-fleischer rings
RTA
Haemolysis
Blue nails
In Wilsons disease, where in the brain is most copper deposited?
In the basal ganglia - particuarly in the putamen and globus pallidus
Investigations for Wilson’s disease?
Slit lamp examination
Serum caeruloplasmin - reduces
Total serum copper - reduced (this is because 95% of plasma copper is carried by ceruloplasmin so free serum copper will be increased)
24 hours urinary copper excretion - increased
Genetic analysis of ATP7B gene for diagnosis confirmation
Tx of Wilson’s disease?
Penicillamine - works by chelating copper
What is acute disseminated encephalomyelitis?
An acute, rapidly progressive autoimmune process which causes demyelination in the brain & spinal cord
It causes non-specific Sx like headache, nausea, fever, fatigue. It also causes encephalopathy (mental status change) and polyfocal neurological deficits e.g. paraparesis, limb weakness, seizures, numbness/tingling, vision loss
May also affect brainstem - dysarthria or oculomotor dysfunction
Which type of autoimmune encephalitis is associated with ovarian teratomas?
Anti-NMDAR encephalitis
What antibodies are present in drug-induced lupus?
Anti-histone
Indications for LTOT in COPD?
Assess people with sats <92 breathing air/raised JVP/peripheral oedema/polycythemia/cyanosis/FEV1<30% by measuring ABGs on 2 occasions at least 3 weeks apart:
PaO2 <7.3 when stable OR
PaO2 7.3-8.0 when stable and 1 of the following: secondary polycythemia, peripheral oedema, pulmonary hypertension
List topical steroids used in psoriasis in order of potency?
Help Every Budding Dermatologist
Mild - hydrocortisone 0.05%
Moderate - Eumovate (clobetasone butyrate 0.05%)
Potent - Betnovate (betamethasone valerate 0.1%)
Very potent - Dermovate (clobetasol propionate 0.05%
What is DMSA?
Dimercaptosuccinic acid scintigraphy
a valuable nuclear medicine technique used primarily in paediatric nephrology to evaluate renal function and structure. It employs Dimercaptosuccinic acid tagged with a radioactive isotope.
DMSA binds to renal cortical tissue, allowing detailed imaging of renal parenchyma.
This procedure is particularly effective for diagnosing renal scarring, congenital abnormalities, renal tumours and differential renal function.
It has a good safety profile with minimal side effects
What is a MCUG scan?
A micturating cystourethrogram is a fluoroscopic study used primarily in paediatric urology to investigate lower urinary tract pathologies.
It involves the instillation of contrast material into the bladder through a catheter, with imaging taken during voiding. This allows detailed visualisation of the bladder, urethra, and sometimes the ureters and kidneys, helping to diagnose conditions such as vesicoureteral reflux, posterior urethral valves, or urethral strictures.
It is an invasive procedure, thus must be performed with care to minimise distress and potential infection risk.
With which heparin do you need routine monitoring with APTT?
Unfractionated heparin
Which type of heparin has a low risk of heparin-induced thrombocytopenia and osteoporiss?
LMWH
