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Miscellaneous Flashcards

1
Q

what is the diagnostic criteria for RA

A

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

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2
Q

what are extrarticular features of RA

A

cervical- atlanto axial subluxation
eyes- scleritis/ episcleritis/ sjogrens
skin- rheumatoid nodules
lungs- lung fibrosis/ effusions
heart- pericarditis
hepatosplenomegaly
renal- nephrotic syndrome

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3
Q

what are causes of tiredness in RA

A

anaemia of chronic disease
iron deficiency- GI loss from NSAIDs use
methotrexate- folate deficiency / bone marrow suppression (also from sulfasalazine)

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4
Q

how do you differentiate episcleritis from scleritis

A

Scleritis is painful, episcleritis is painless

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5
Q

what is RF

A

an antibody found in approx 75% of patients with RA, also present in other CTDs such as SLE

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6
Q

what are poor prognostic factors in RA

A

positive RF
presence of anti- CCP
early evidence of erosive disease
functional impairement

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7
Q

what are the radiological findings of rheumatoid arthritis

A

loss of joint space
erosions
subchondral cysts
subchondral sclerosis

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8
Q

what are the radiological findings of osteoarthritis

A

Loss of joint space
osteophytes
soft tissue swelling
see-through bones (osteopaenia)

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9
Q

what monitoring is important with the following DMARDs
sulphasalazine
methotrexate
hydroxychloroquine

A

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

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10
Q

how is systemic sclerosis classified

A

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

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11
Q

what are the diagnostic criteria for systemic sclerosis

A

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

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12
Q

what is the most common cause of mortality in systemic sclerosis

A

renal failure

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13
Q

what auto antibodies are associated with systemic sclerosis

A

limited: anti-centromere antibody, ANA, Rh factor
diffuse: anti- scl70, anti- RNA polymerase

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14
Q

what is the management of systemic sclerosis

A

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

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15
Q

complications of neurofibromatosis

A

phaeochromocytoma + RAS - HYPERTENSION
acoustic neuroma
optic glioma
epilepsy
restrictive cardiomyopathy
pulmonary fibrosis
pnuemothorax

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16
Q

what are the genetics of NF

A

autosomal dominant disease
mutation on chromosome 17 (NF1) or 22 (NF2)

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17
Q

what are the diagnostic criteria of NF1

A

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

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18
Q

what are the diagnostic criteria for NF2

A

any of:

bilateral 8th nerve involvement
neurofibroma
meningioma/ glioma/ shwanoma
first degree relative with NF

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19
Q

how would you investigate and treat a patient with a phaeochromocytoma

A

24 hour urinary catecholamines
alpha blocker then beta blocker
surgery is definitive management

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20
Q

tests for a bitemporal hemianopia

A

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

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21
Q

how to manage a pituatary tumour

A

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

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22
Q

what are some differentials for thyroid eye disease

A

orbital mass- unilateral/ tender
orbital cellulitis- unilateral, tender, systemic symptoms
orbital fracture- trauma, enopthalmos not proptosis

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23
Q

what is the management of thyroid eye disease

A

tear film lubrication
steroids for eye disease
smoking cessation
optimise thyroid function
eyes taped at night

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24
Q

what are specific features of graves thyroid disease

A

graves opthalmopathy (proptosis, chemosis, soft tissue swelling, opthalmaplegia), thyroid acropatchy, pretibial myxoedema

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25
causes of proptosis
graves disease cavernous sinus thrombosis oribital cellulitis retroorbital tumour trauma
26
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
27
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%
28
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
29
what are the systemic manifestations of ankylosing spondilitis
apical fibrosis aortic regurgitation achilles tendonitis AVN conduction defects amyloidosis anterior uveitis atlanto- axial subluxation
30
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)
31
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
32
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
33
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
34
management of endogenous cushings syndrome
management of underlying cause e.g. surgical resection medical treatment e.g. metyrapone
35
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
36
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)
37
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
37
what are the causes of primary adrenal failure
autoimmune (70-90%) TB (10-20%)
38
what other autoimmune conditions are associated with addisons disease
polyglandular autoimmune syndrome - diabetes, thyroid, vitiligo, RA, SLE, alopecia
39
what autoantibodies are present in addisons disease
antibodies to 21-hydroxylase is the most common and specific also can have antibodies to 17-hydroxylase
40
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.
41
types of meningitis
viral meningitis lyme meningitis fungal meningitis protozoa meningitis paraneoplastic/ malignant causes
42
what are the complications of bacterial meningitis
can be fatal permanent neurological sequela cognitive problems vascular complications- amputations from sepsis
43
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
44
what other organs can be affected in acromegaly
cardiac- cardiomyopathy, diabetes, hypertension visual consequences
45
possible complications of transphenoidal pituatary resection
panhypopituatarism
46
obstructive sleep apnoea scoring
epworth sleep score stop bang score
47
osteogenesis imperfecta - cardiac signs
association with bicuspid aortic valve- can lead to aortic regurgitation
48
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
49
bisphosphonates side effects
gastroc irritation osteonecrosis of the jaw (teeth check)
50
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
51
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
52
what is ehlers danlos
autosomal dominant connective tissue disorder that mostly affects type III collagen
53
antiphospholipid syndrome investigations
antibodies - anticardiolipin antibodies - anti-beta2 glycoprotein I (anti-beta2GPI) antibodies - lupus anticoagulant thrombocytopenia prolonged APTT
54
marfans syndrome cardiac features
aortic dilatation aortic regurgitation mitral valve proplapse
55
indications for aortic root surgery
dilatation ?50mm at root or 45mm if family history of aoritc dissection or rate expanding >3mm a year
56
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
57
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.
58
what are major risk factors for gastric cancer
family history increased age smoking H.pylori infection long term gastric reflux
59
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
60
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
61
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.
62
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
63
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.
64
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
65
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
66
associations with HIV and abdominal pain
more susseptible to infections HIV associated malignancy e.g. lymphoma/ Kaposi sarcoma HIV medication related pancreatitis
67
other rarer causes of abdominal pain
acute intermittent porphyria vasculitis particularly polyarteritis nodosa familial mediteranean fever- fever+ abdominal pain adrenal insufficiency
68
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
69
DVLA guidelines
70
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
71
what are the common causes of AF
cardiac- IHD, mitral valve disease, CCF non-cardiac- thyrotoxicosis, acute illness, alcohol, idiopathic
72
conditions associated with retinitis pigmentosa
Refsum syndrome- peripheral neuropathy, cerebellar ataxia, deafness, muscle wasting Alports syndrome Usher syndrome- sensorineural deafness
73
bilateral visual disturbance
night time- cataract, vitamin A deficiency (CF?) diabetes glaucoma

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