Miscellaneous Flashcards
what is the diagnostic criteria for RA
at least 4 of:
1) morning stiffness >1 hour
2) symmetrical joint involvement
3) arthritis affecting > 3 joints
4) involvement of the small joints of the hands
5) positive RF
6) rheumatoid nodules
7) radiographical evidence
what are extrarticular features of RA
cervical- atlanto axial subluxation
eyes- scleritis/ episcleritis/ sjogrens
skin- rheumatoid nodules
lungs- lung fibrosis/ effusions
heart- pericarditis
hepatosplenomegaly
renal- nephrotic syndrome
what are causes of tiredness in RA
anaemia of chronic disease
iron deficiency- GI loss from NSAIDs use
methotrexate- folate deficiency / bone marrow suppression (also from sulfasalazine)
how do you differentiate episcleritis from scleritis
Scleritis is painful, episcleritis is painless
what is RF
an antibody found in approx 75% of patients with RA, also present in other CTDs such as SLE
what are poor prognostic factors in RA
positive RF
presence of anti- CCP
early evidence of erosive disease
functional impairement
what are the radiological findings of rheumatoid arthritis
loss of joint space
erosions
subchondral cysts
subchondral sclerosis
what are the radiological findings of osteoarthritis
Loss of joint space
osteophytes
soft tissue swelling
see-through bones (osteopaenia)
what monitoring is important with the following DMARDs
sulphasalazine
methotrexate
hydroxychloroquine
1) neutropaenia/ thrombocytopaenia/ liver impairment - 3/12 LFT + FBC
2) neutropaenia/ thrombocytopaenia/ liver impairment/ pneumonitis- baseline CXR, monthly FBC/ LFT
3) hydroxychloroquine- corneal deposits and retinopathy- annual acuity testing
how is systemic sclerosis classified
diffuse scleroderma- diffuse involvement of the skin/ trunk/ extremities. Early involvement of lung, kidney, gut and heart
limited scleroderma- skin only affected in the extremities/ face. CREST syndrome- calcinosis, raynauds phenomenon, oesophageal dysmotility, sclerodactyly, telangectasia. late involvement of other organs, renal crisis is rare.
systemic sclerosis sine scleroderma- organ involvement without skin involvement
malignant scleroderma- accelerated course of disease leading to death- often found in elderly men
what are the diagnostic criteria for systemic sclerosis
American rheumatism association has clear diagnostic criteria- 1 major or 2+ minor criteria needed
minor- sclerodactyly, finger pulp atrophy, bilateral pulmonary fibrosis
major- skin sclerosis affecting arms, face, neck
what is the most common cause of mortality in systemic sclerosis
renal failure
what auto antibodies are associated with systemic sclerosis
limited: anti-centromere antibody, ANA, Rh factor
diffuse: anti- scl70, anti- RNA polymerase
what is the management of systemic sclerosis
MDT approach
physio- exercises for contractures and deformities
general- patient education and counselling, analgesia for arthralgia
raynauds- smoking cessation, hand warmers, vasodilators (CCB, ACEi, prostacyclin analogues)
GI- prokinetics for dysmotility, PPI, nutritional support
renal- strict hypertension control and ACEi for renal crisis
respiratory- vasodilators for pulmonary hypertension
complications of neurofibromatosis
phaeochromocytoma + RAS - HYPERTENSION
acoustic neuroma
optic glioma
epilepsy
restrictive cardiomyopathy
pulmonary fibrosis
pnuemothorax
what are the genetics of NF
autosomal dominant disease
mutation on chromosome 17 (NF1) or 22 (NF2)
what are the diagnostic criteria of NF1
2 of:
> 6 cafe au lait spots
axillary freckling
2 neurofibromata
2 lisch nodules
optic glioma
first degree relative with NF
bone involvement- thinning of long bones/ sphenoid dysplasia
what are the diagnostic criteria for NF2
any of:
bilateral 8th nerve involvement
neurofibroma
meningioma/ glioma/ shwanoma
first degree relative with NF
how would you investigate and treat a patient with a phaeochromocytoma
24 hour urinary catecholamines
alpha blocker then beta blocker
surgery is definitive management
tests for a bitemporal hemianopia
full hormone panel- prolactin, IGF-1, TFT, LH/FSH, cortisol, ACTH, testosterone, short synACTHen test, GTT
brain MRI to visualise the pituatary and look for a tumour
how to manage a pituatary tumour
if there were hormone deficienicies, I woul start hormone replacement.
Offer surgery for resection of the tumour or radiotherapy if there were recurrence/ residual disease
for prolactinoma- dopamine agonists such as bromocriptine are first line
what are some differentials for thyroid eye disease
orbital mass- unilateral/ tender
orbital cellulitis- unilateral, tender, systemic symptoms
orbital fracture- trauma, enopthalmos not proptosis
what is the management of thyroid eye disease
tear film lubrication
steroids for eye disease
smoking cessation
optimise thyroid function
eyes taped at night
what are specific features of graves thyroid disease
graves opthalmopathy (proptosis, chemosis, soft tissue swelling, opthalmaplegia), thyroid acropatchy, pretibial myxoedema
causes of proptosis
graves disease
cavernous sinus thrombosis
oribital cellulitis
retroorbital tumour
trauma
management of graves disease
symptomatic- beta blocker eg propanolol for palpitations, tachycardia, anxiety
thionamides- carbimazole (preferred), PTU (for pregnancy)
radioiodine ablation- may worsen graves eye disease
thyroidectomy- rarely used unless extremely large goitre causing obstructive symptoms
advise for patients who recently received radio iodine
must avoid contact with pregnant women for 2 weeks
pregnancy contraindicated
risk of hypothyroidism is 2-3%
definition of ankylosing spondylitis
Symptomatic sacroilitis (pain and stiffness for >3 months) associated with morning stiffness and improvement on mobilising/ worsening with rest. Associated with the allele B27 which is present in up to 95% of causasian patients and implies an 80 fold risk
what are the systemic manifestations of ankylosing spondilitis
apical fibrosis
aortic regurgitation
achilles tendonitis
AVN conduction defects
amyloidosis
anterior uveitis
atlanto- axial subluxation
how do you diagnose ankylosing spondylitis
XR of the sacro-iliac joints
graded from 0 (normal) to 4 (fused)
MRI can be used to visualise further (most sensitive and specific)
what do you understand by the term seronegative arthritis
It’s a subtype of inflammatory arthritis (RA) in adults that’s diagnosed when blood tests are negative for rheumatoid factor (RF) and anti-cyclic citrullinated peptides (anti-CCPs). common subtypes:
psoriatic, enteropathy- associated, ankylosing spondylitis, reactive arthritisw
what is the management of ankylosing spondylitis
MDT approach
patient education and counselling
physiotherapy
occupational therapy
hydrotherapy
pharmacological- NSAIDs, analgesia,
DMARDs- methotrexate/ sulphasalazine for peripheral disease (not spine),
biologics e.g. adalimumab
how do you explore the differentials in a patient with excessive cortisol
low dose dexamethasone test (1mg) resulting in an abormal cortisol >50 would confirm cortisol excess
confirming cause look at ACTH:
high dose dexamethasone test
suppressed cortisol- pituatary adenoma
elevated cortisol- ectopic ACTH/ adrenal cushing
ACTH low- adrenal cushing
ACTH high- ectopic ACTH
management of endogenous cushings syndrome
management of underlying cause e.g. surgical resection
medical treatment e.g. metyrapone
what is the difference between cushings disease and cushings syndrome
cushings syndrome refers to the digns and symptoms caused by elevated cortisol (most commonly exogenous). Cushings disease is caused by the hypersecretion of ACTH from a pituatary adenoma which is a cuase of cushing’s syndrome
how would you investigate a patient with suspected addisons disease
I would perform a short synacthen test
In the case of addisons disease I would expect the adrenals to fail to produce an adequete cortisol response (170 at baseline, 550 at peak)
management of addisons disease
mainstay of treatment would be steroid replacement- glucocorticoid and mineralocorticoid
in the instance of an adrenal crisis- rapis steroid and fluid replacement with correction of any electrolyte derangements would be crucial
advise patient to wear a medic alert bracelet
educate patient e.g. sick day rules
what are the causes of primary adrenal failure
autoimmune (70-90%)
TB (10-20%)
what other autoimmune conditions are associated with addisons disease
polyglandular autoimmune syndrome
- diabetes, thyroid, vitiligo, RA, SLE, alopecia
what autoantibodies are present in addisons disease
antibodies to 21-hydroxylase is the most common and specific
also can have antibodies to 17-hydroxylase
why does pigment change in addisons disease
ACTH is synthesised from POMC which is also the precursor to MSH. These biproducts are structurally similar therefore high ACTH can activate melanocyte receptors and cause melanin deposition.
types of meningitis
viral meningitis
lyme meningitis
fungal meningitis
protozoa meningitis
paraneoplastic/ malignant causes
what are the complications of bacterial meningitis
can be fatal
permanent neurological sequela
cognitive problems
vascular complications- amputations from sepsis
management of acromegaly
surgery may be considered for a pituitary tumour as the first line mangement
If it is inoperable or surgery unsuccessful then medication may be indicated:
somatostatin analogue e.g. octreotide
directly inhibits the release of growth hormone
effective in 50-70% of patients
pegvisomant. GH receptor antagonist. once daily s/c administration. very effective - decreases IGF-1 levels in 90% of patients to normal. doesn’t reduce tumour volume therefore surgery still needed if mass effect
dopamine agonists, for example bromocriptine.
the first effective medical treatment for acromegaly, however now superseded by somatostatin analogues
effective only in a minority of patients.
External irradiation is sometimes used for older patients or following failed surgical/medical treatment
what other organs can be affected in acromegaly
cardiac- cardiomyopathy, diabetes, hypertension
visual consequences
possible complications of transphenoidal pituatary resection
panhypopituatarism
obstructive sleep apnoea scoring
epworth sleep score
stop bang score
osteogenesis imperfecta - cardiac signs
association with bicuspid aortic valve- can lead to aortic regurgitation
osteogenesis imperfecta inheritence and associated features
autosomal dominant
8 different variations
abnormality in type 1 collagen due to decreased synthesis of pro-alpha 1 or pro-alpha 2 collagen polypeptides
discoloured translucent teeth
hearing impairement- middle ear bone
spinal scoliosis
bowing of long bone
short stature
bisphosphonates side effects
gastroc irritation
osteonecrosis of the jaw (teeth check)
CHADsVASc score
Age
Sex
CHF history
Hypertension history
Stroke/TIA/thromboembolism history
Vascular disease history (prior MI, peripheral artery disease, or aortic plaque)
Diabetes history
complications of ehlers danlos syndrome
elastic, fragile skin
joint hypermobility: recurrent joint dislocation
easy bruising
aortic regurgitation, mitral valve prolapse and aortic dissection
subarachnoid haemorrhage
angioid retinal streaks, glaucoma
haemorrhage- GI
what is ehlers danlos
autosomal dominant connective tissue disorder that mostly affects type III collagen
antiphospholipid syndrome investigations
antibodies
- anticardiolipin antibodies
- anti-beta2 glycoprotein I (anti-beta2GPI) antibodies
- lupus anticoagulant
thrombocytopenia
prolonged APTT
marfans syndrome cardiac features
aortic dilatation
aortic regurgitation
mitral valve proplapse
indications for aortic root surgery
dilatation ?50mm at root or 45mm if family history of aoritc dissection
or rate expanding >3mm a year
what are the secondary causes of HTN
endocrinological causes- Conn’s, Cushings, thyroid, acromegaly
phaeochromocytoma
renal causes- chronic renal disease of any cause, renovascular disease
cardio-respiratory- obstructive sleep apnoea, coarctation of aorta
how is cardiovascular risk assessed in hypertensive patients
10 year CHD risk is calculated. Scoring system based on the Faringham heart study. Looks at age, gender, systolic blood pressure, smoking status, cholesterol. Used only in patients for primary prevention.
what are major risk factors for gastric cancer
family history
increased age
smoking
H.pylori infection
long term gastric reflux
how to manage malnutrition
1) exclude sepsis- nutritional support is often less effective when patients have an infection + may not present typically with infection
2) rehydration
3) micronutrient deficiency replacement
4) enteral feeding with dietician support
5) Paraenteral feeding if needed
6) refeeding bloods
causes of weight loss in elderly
cognition
mood
systemic causes- malignancy, malabsortion, thyroid disease
reduced intake- oral soreness, ill fitting dentures, dysphagia
medication side effects
social problems- isolation, shopping
what do you know about hereditary colorectal cancer syndromes
Lynch syndrome (HNPCC)- autosomal dominanat. most common. Increased risk of CRC, gynae esp endometrial. Amsterdam criteria
FAP- also autosomal dominant. 100s of polyps. mutation in APC gene.
Peutz-Jehger sundrome- autosomal dominant. pigmented lesions and GI haemartomous polyps.
pathophysiology of jaundice
jaundice can be pre-hepatic, hepatic or post-hepatic. In pre-hepatic jaundice, the breakdown of red cells results in the formation of unconjugated bilirubin. In the liver, this is then conjugated and released into the GI tract via the bile. In the colon it is metabolised to urobilinogen to stercobilinogen and then stercobilin which is what gives the faeces its brown colour. Some urobilinogen is released in the urine.
In prehepatic jaundice, you will see largely a unconjugated bilirubin.
In hepatic disease it will be likely a mixed picture due to impaired conjugation but also cholestasis.
In post hepatic jaundice you will see high levels of conjugated bilirubin
how to diagnose and manage alcohol hepatitis
Blood tests characteristic- AST: ALT ratio >2
other classic liver derangement- increased clotting, decreased albumin.
severity is calculated using the Glasglow alcoholic hepatitis score/ Maddreys score. Both will indicate severity of disease. Mainstay of treatment is alcohol cessation, steroids/ pentoxyfylline may be trialed.
liver abnormalities in pregnancy
HELLP syndrome- haemolytic anaemia, elevated liver enzymes and low platelets
obstetric cholestasis- usually in 2nd/ 3rd trimester characterised by pruritis and raised bile acids
acute farrty liver of pregnancy- is associated with pre-eclampsia
how does one diagnose IBS
may use the Rome III criteria to aid a diagnosis. It is important to look out for any red flag features in the history which would need colonic investigations. These include: bleeding rectally, weight loss, IDA, age over 65, family history.
Rome criteria- patients should have pain 3 days per month for 3 days which is : relieved on defecation, change in stool frequency, change in stool form. Associations- mucous PR, bloating, straining/ urgency
associations with HIV and abdominal pain
more susseptible to infections
HIV associated malignancy e.g. lymphoma/ Kaposi sarcoma
HIV medication related pancreatitis
other rarer causes of abdominal pain
acute intermittent porphyria
vasculitis particularly polyarteritis nodosa
familial mediteranean fever- fever+ abdominal pain
adrenal insufficiency
management of acute coronary syndrome
morphine and metoclopramide
oxygen- if hypoxic, is normal o2 can increase rate of ischaemia
nitrates- GTN spray
aspirine 300mg
clopidogrel 300mg
STEMI- PCI/ thrombolysis
DVLA guidelines
what is the management of CCF
diuretics are useful in controlling congestive symptoms
all patients should be on an
ACEi unless contraindications. Can see a rise in serum creatinine up to 20% which is expected- if more, then rule out renovascular disease.
BB
spironolactone
digoxin- if heart failure and AF
what are the common causes of AF
cardiac- IHD, mitral valve disease, CCF
non-cardiac- thyrotoxicosis, acute illness, alcohol, idiopathic
conditions associated with retinitis pigmentosa
Refsum syndrome- peripheral neuropathy, cerebellar ataxia, deafness, muscle wasting
Alports syndrome
Usher syndrome- sensorineural deafness
bilateral visual disturbance
night time- cataract, vitamin A deficiency (CF?)
diabetes
glaucoma
