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MRCP PACES > Clinical Consultation > Flashcards

Clinical Consultation Flashcards

1
Q

Common scenario leads which should make you think of Marfan’s?

A

Chest pain
SOB
Change in vision
Joint pains

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2
Q

Questions to think about when considering Marfan’s as a differential?

A

Chest pain
SOB
Fever, night sweats, weight loss, blood in urine, rash
Change to vision
Dislocated joints
Lumps in neck

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3
Q

Things you might find on inspection in a patient with Marfan’s?

A

Tall
Arachnodactyly
Kyphoscoliosis ± pectus excavatum/carinatum
Long arms vs torso
Thumb in fist - come out other side
Bend thumb against hand
Look quickly side to side - upwards lens dislocation - iris vibrate
Blue sclera
High arched palate
Mucosal neuroma’s
Pes planus - flat feet

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4
Q

What systems would you examine in a patient with suspected Marfan’s and what may you find?

A

CVS
- Collapsing pulse
- Prominent neck pulsations
- BP
- Feel for apex beat
- Early diastolic murmur - AR
- Ejection click and late systolic murmur - MV prolapse

Resp
- Check tracheal position
- Percuss/auscultate for pneumothorax
- Inspect axilla for chest drain scars

Abdo
- look for hernia

Legs
- Dural Ectasia - test for weakness

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5
Q

What are some differentials for Marfan’s syndrome

A

Homocysteinuria - no cardiac. cognitive impairment. downwards lens dislocation. Recurrent VTE

MEN2b - marfanoid, mucosal neuromas, medullary thyroid ca, phaeochromocytoma - no affect on heart or eyes

MASS phenotype - MV prolapse, non-progressive aortic root dilatation, skin and skeletal manifestations

Ehlers Danlos - increased hyper mobility, skin fragility,

Pseudoxanthoma elasticum - AR, plucked chicken skin, angoid streaks on fundoscopy, blue sclera, screen for HTN and accelerated atherosclerosis

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6
Q

What investigations would you do in a patient with suspected Marfan’s?

A

Bedside
- ECG
- BP - wide pulse pressure
- Temp, fundoscopy, urine dip - ?IE

Bloods depend on presentation - r/o infections/PE

Imaging
- CXR - cardiomegaly, pulmonary oedema, wide mediastinum
- Echo - annually - monitor aortic root diameter and for AR

Special
- Can do genetic tests but diagnosis usually on Ghent criteria

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7
Q

How is Marfan’s managed?

A

Counsel patient - familial component, risk of pneumothorax and heart disease

Information leaflets and support groups

Screen family members

MDT - PT/OT, Cardiologist, genetic counsellors, resp, rheum, ortho, ophthalmologist, GP

Medical
- Manage co-morbidities
- Beta blocker and ACEi to slow AR dilation

Surgical
- AV replacement
- Aortic root repair - if >5cm

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8
Q

What are some causes of a high arched palate?

A

Marfan’s
Turner’s syndrome
Homocysteinuria
Noonan’s syndrome
Freidrichs ataxia

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9
Q

What is the genetic defect in Marfan’s and how is it inherited?

A

Autosomal Dominant

FNB1 gene on chromosome 15 encoding Fibrillin-1 - complete penetrance but variable phenotype

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10
Q

What can cause a blue sclera?

A

Marfan’s
Ehlers Danlos
Pseudoxanthoma elasticum
Osteogenesis Imperfecta
Alkapturia - due to deposition. Others due to thin sclera

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11
Q

What is the cause of MEN2b

A

Spontaneous or autosomal dominant defect of RET proto-oncogene

Treated with thyroidectomy and close monitoring for phaeochromocytoma

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12
Q

What makes up the Revised Ghent Criteria?

A

Family hx of marfans
Aortic root dilatation or dissection
FNB1 mutation
Systemic score

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13
Q

What are features of the systemic score for Marfan’s?

A

Wrist sign
Thumb sign
Pectus Excavatum/Carinatum
Pes planus
Pneumothorax
Dural ectasia
Protrusion acetabula
Increased arm span:height ratio
reduced upper segment/lower segment ratio,
scoliosis or thoracolumbar kyphosis,
reduced elbow extension,
facial features (dolichocephaly, enophthalmos, downslanting palpebral fissures, malar hypoplasia, retrognathia),
skin striae,
myopia,
mitral valve prolapse

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14
Q

What are the indications for aortic root replacement in a patient with Marfan’s?

A
  • Dilation >50mm
  • Dilation >45mm + family hx of dissection
  • Increasing by >3mm per year
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15
Q

What are the cardinal features of systemic sclerosis?

A

CREST syndrome

Calcinosis - usually found on fingertips
Raynauds
Oesophageal dysmotility
Sclerodactyly
Telangiectasia

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16
Q

Questions to ask regarding Raynaud’s?

A

Do hands change colour in the cold?

3 stages? - white –> blue –> red

How badly is it affected
Time to normalise
Develop ulcers in hands?
Family Hx

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17
Q

What skin changes may you see in systemic sclerosis?

A

Tight shiny thick skin
Telangiectasia
Bruising
Hypo/hyperpigmentation
Swollen extremities - non pitting
Lose fat pads in hands

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18
Q

What face changes may you see in systemic sclerosis?

A

Small mouth - microstomia
Peri-oral furrows
Pinched nose

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19
Q

Features of respiratory exam in suspected systemic sclerosis?

A

Fine end inspiratory crackle at bases
Don’t change with coughing

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20
Q

Features on CVS examination in suspected systemic sclerosis?

A

Raised JVP
Left parasternal heave
Palpable P2
Loud P2
Peripheral oedema

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21
Q

What is systemic sclerosis?

A

Autoimmune, inflammatory, fibrotic connective tissue disease

Unknown aetiology

Affect skin and internal organs

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22
Q

What are the patterns of disease in systemic sclerosis?

A

Limited cutaneous (CREST)

Diffuse cutaneous
- CREST
- CVS - HTN + coronary heart disease
- Lung fibrosis and Pulm. HTN
- Renal - glomerulonephritis and scleroderma renal crisis

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23
Q

What investigations would you request for suspected systemic sclerosis?

A

Bedside:
- Observations - O2 sats + BP
- Urine dip - renal crisis
- ECG - R heart strain - ST depression and TWI V1-3

Bloods
- FBC - anaemia
- UE - renal crisis
- ANA - positive in most patients. Non specific
- anti scl-70 and topoisomerase - diffuse
- anti-centromere - limited

Imaging
- CXR
- HRCT
- Echo

Special
- Capillaroscopy - abnormal nail fold capillaries
- Thermography with cold challenge
- Spirometry
- Endoscopy - oesophagitis

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24
Q

How is systemic sclerosis managed?

A

Non-Pharmacological
- Education
- Hand warmers, avoid cold
- PT/OT, SALT
- Smoking cessation
- MDT - Rheum, renal, cardio, resp, gastro

Medical
- Prokinetics
- Antacids, PPI
- Nifedipine - Raynauds
- Sildenafil, iloprost, bosentan - Pulm HTN
- Anticoagulation
- Diuretics
- O2
- Methotrexate, mycophenolate, cyclophosphamide

Surgical
- Lung transplant
- Contracture release
- Excision of calcinosis

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25
Q

What are some features of lupus?

A

Joint pain and swelling - Jaccoud’s arthropathy - morning stiffness, polyarticular symmetrical

Systemic - Fever, weight loss, myalgia, malaise, Raynaud’s

Skin - Photosensitive molar rash, discoid rash

Neuro - headaches, cognitive impairment, seizures, psychosis/delirium, peripheral neuropathy

Pulmonary - fibrosis, vasculitis, pleural effusion

Cardio - pericarditis, pericardial effusion, myocarditis, valvular disease, HTN

Renal - lupus nephritis - 1/3 of pateints

Gastro - abdominal pain, nausea, vomiting, hepatomegaly, ulcers

Haem - anaemia, leukopaenia, antiphospholipid syndrome

Osteopaenia/Osteoporosis

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26
Q

What are some examination features indicative of lupus?

A

Malar rash
Nail fold vasculitis
Photosensitivity
Livedo reticularis
Jaccoud’s arthropathy
Mouth ulcers
Pale palmar creases
Pericardial rub
Evidence of Pulm. HTN - raised JVP, P2
Peripheral oedema
Pleural rub
Stony dull percussion
Reduced air entry
Organomegaly
Renal transplant

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27
Q

What are some differentials for lupus?

A

Drug induced lupus

Systemic sclerosis

Dermatomyositis

Mixed connective tissue disease

Stills disease

Sjogrens

Pulmonary renal syndrome - ANCA/Goodpastures

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28
Q

How would you investigate lupus?

A

Bedside:
- Urine dip - protein, blood, pregnancy test
- BP
- ECG - pericarditis/R heart strain
- BILAG score

Bloods:
- FBC, U&E, LFT, CRP
- ANA - sensitive not specific
- Anti Ro, La, Smith
- Anti ds-DNA - specific
- Complement - low in active disease
- Anti-histone - drug induced
- Anticardiolipin/lupus anticoagulant- antiphospholipid syndrome
- Positive direct Coombs test in absence of haemolytic anaemia

Imaging
- Joint X-Rays - non erosive
- CXR - effusions, nodules, infiltrates
- Echo - pericardial effusion/sterile valve vegetations

Special
- Anti C1q - raised in renal disease
- Urine PCR
- Urine for red cell casts
- Skin biopsy
- Renal biopsy
Others - DEXA, EMG, Abdo USS/OGD, cMRI, Cranial imaging

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29
Q

What medications are associated with drug induced lupus?

A

Hydralazine
Procainamide
Isoniazid

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30
Q

How is lupus diagnosed?

A

4/17 criteria. 1 clinical + 1 immunological

Biopsy proven lupus nephritis + ANA/anti dsDNA

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31
Q

What are some clinical criteria for lupus?

A

Acute/Chronic cutaneous features
Oral/nasal ulcers
Non scarring alopecia
Synovitis
Serositis
Renal
Neuro features
Haemolytic anaemia
WCC <4 or Lymph <1
Plt <100

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32
Q

What are anti ro/la associated with?

A

Neonatal lupus
Heart block

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33
Q

What is Anti RNP associated with?

A

Mixed connective tissue disease

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34
Q

When should you suspect antiphospholipid syndrome?

A

Arterial thrombosis <50yo
Unprovoked VTE <50yo
Recurrent thrombosis
Arterial and venous events
Unusual sites - renal, liver, cerebral sinus, mesenteric, vena cava, retina
Obstetric - recurrent miscarriage, severe pre-eclampsia

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35
Q

What are features are associated with antiphospholipid syndrome?

A

Postive antibodies
Low platelets
Recurrent miscarriage
Livedo reticularis

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36
Q

What blood test findings would you see in an acute lupus flare?

A

Low complement
Low lymphocytes
Low/normal CRP
Raised ESR
Anaemia
Low Plt
Raised dsDNA

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37
Q

What are the main causes of morbidity/mortality in Lupus?

A

Infections
Atherosclerosis
Osteoporosis
Malignancy - lymphoma, lung, cervical

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38
Q

How is lupus managed?

A

Conservative - sunscreen, pregnancy advice, support groups, PT, OT, smoking cessation

Medical
- Analgesia
- Mild/Mod - Pred, hydroxychloroquine, azathioprine, MTX
- Severe - I V Methylpred, cyclophosphamide, MMF, Rituximab/Belimumab
- Optimise HTN, diabetes, hypercholesterolaemia

Surgical - renal transplant

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39
Q

How does drug induced lupus vary from SLE?

A

Equal sex distribution - SLE = F>M
No renal involvement
Antihistone
Normal complement

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40
Q

What are some complications of hydroxychloroquine

A

Retinopathy - rate but seen in those at high doses >5 years

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41
Q

Causes of anaemia in SLE?

A

Chronic disease
NSAID associated gatritis
Myelosuppression
Folate deficiency - MTX
B12 deficiency
Autoimmune haemolytic anaemia
Hypersplenism

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42
Q

What is mixed connective tissue disease?

A

Overlapping features of SLE, scleroderma, myositis, Raynauds

ANA positive
Positive Anti U1 RNP
Elevated inflammatory markers and CK

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43
Q

What are the types of neurofibromatosis?

A

Autosomal dominant condition

Type 1 - affect chromosome 17 - neurofibromin
- Cafe au lait spots
- axillary/groin freckles
- peripheral neurofibromas
- Lisch nodules
- Scoliosis

Type 2 - chromosome 22
- Bilateral acoustic neuromas.
- Meningiomas
- Schwannomas
Much more rare

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44
Q

Scenarios you should consider neurofibromatosis?

A

Seizures
Headaches
Rashes
Visual disturbances

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45
Q

Questions to ask in a neurofibromatosis history?

A

Headache, visual disturbance or personality changes - optic glioma/meningioma

Seizures - epilepsy

BP - HTN secondary to RAS, phaeochromocytoma or co-arctation

Palpitations, anxiety, sweating, tremor - phaeo

Hearing - acoustic neuromas

School/college/uni - reduced IQ

Family history

Skin-skin discolouration, lumps etc.

Abdo pain, bloating, dyspepsia - GI carcinoid, obstruction, bleeding

Breathing - lung cysts/fibrosis

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46
Q

Examination features in neurofibromatosis?

A

Skin and armpits - assess for neurofibromas, cafe au last spots, axillary freckles

Back - kyphosis/scoliosis

Neuro
- CN 5-8
- Cerebellar
- Pronator drift

Eyes - linch nodules - visual acuity, fundoscopy (if time), visual fields, pupils, eye movements

CVS/Resp/Abdo
- murmur of co-arctation - ESM, back L shoulder blade
- Lungs - creps
- Abdo - freckles, renal bruits

Offer to check BP
- renal artery stenosis, Phaeo, co-arctation

Order:
Hands –> pronator drift/rebound, intention tremor, past pointing –> eyes –> CN 5-8 –> look at armpits –> CVS/resp/abdo –> check shape of spine/look for neurofibromas

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47
Q

Differentials for neurofibromatosis?

A

Decrums disease - multiple lipomas - skin can be mobilised seperately from lipoma

Tuberous Sclerosus
- Adenoma sebacaeum
- Subungal fibroma
- Shagreen patch
- Ash leaf macules

48
Q

How would you investigate neurofibromatosis?

A

Bedside
- Blood pressure

Imaging
- MRI head - optic nerve glioma
- CXR/CT chest - fibrosis, cysts
- Abdo US + doppler - renal artery stenosis

Special
- Slit lamp - lisch nodeules
- Urinary metanephrines
- Skin biopsy - exclude sarcoma
- Genetic testing

49
Q

How is neurofibromatosis managed?

A

MDT - geneticist, neurologist, plastics, orthographic, ophthalmology

Non-pharmacological
- Education
- Family screening
- Genetic counselling
- Annual BP, eye, bone and skin check

Medical
- Anti-epileptics
- Antihypertensives - alpha then beta for phaeo

Surgical
- Remove lesions - bothersome, compressive, neoplastic

50
Q

What is a plexiform neurofibroma?

A

Larger neurofibroma which can grow to considerable size and cause significant distortion

Risk of neoplastic transformation with these.

51
Q

Which syndromes have neurocutaneous features?

A

Neurofibromatosis
Tuberous sclerosis
Von Hippel Lindau
Ataxic telangiectasia
Sturge Weber Syndrome

52
Q

What hereditary conditions are associated with phaeochromocytoma?

A

Neurofibromatosis
Von Hippel Lindau
MEN 2a and 2b

53
Q

What are the features of von hippel lindau?

A

Cysts everywhere

Retinal and Cerebellar haemangiomas
Renal cysts
Phaeochromocytoma
Epididymal, pancreatic and hepatic cysts

54
Q

Types of MEN

A

MEN1 - 3P’s - present with hypercalcaemia
- Parathyroid - hyperparathyroidism
- Pituitary
- Pancreas - gastrinoma

MEN2a - 2P’s
- Phaeochromocytoma
- Parathyroid
(also medullary thyroid Ca)

MEN2b 1P
- Phaeochromocytoma
- Medullary thyroid Ca
- Neuromas
- Intestinal polyps
- Marfanoid body habitus

55
Q

Things in history for acromegaly?

A

Change to appearance
Rings, shoes, gloves, dentures not fitting anymore
Snoring at night - ?new or worsening
Headache
Change in vision
Increased sweating
Bumping into things at sides
Numbness or tingling in hands
Change to bowel habit
Change in libido, maintaining erections/milk production
Other medical conditions - diabetes, heart disease, bowel cancer
Meds currently taking
Family hx

56
Q

Examination findings in acromegaly?

A

Skin - tags, acanthosis nigricans, oily skin, finger prick test

MSK - large spade like hands. Carpal Tunnel

Face - Prominent supra-orbital ridge, wide spaced teeth, macroglossia, protruding lower jaw

Ocular - bitemporal hemianopia

Cardiac - AF, displaced apex beat, 3rd HS, bibasal lung creps, pitting peripheral oedema

Thyroid - multi nodular goitre

57
Q

Examination routine for Acromegaly?

A

Hands –> check peripheral muscle wasting –> Tinnels + Phalens –> check pulse, ask for BP –> Face –> visual fields –> Mouth –> Neck –> Cardiac –> Assess skin

58
Q

Complications of acromegaly?

A

Cardiomyopathy
HTN
IHD
Bitemporal hemianopia
T2DM
Carpal Tunnel syndrome
GI malignancy
Goitre

59
Q

Investigations for acromegaly?

A

Bedside
- Urine dip - glycosuria
- BP
- ECG - LVH, arrhythmia
- Finger prick glucose
- Fundoscopy
- Review old photos

Bloods
- IGF1 - raised
- OGTT with growth hormone suppression - fail to suppress GH
- Prolactin
- TFT’s
- FSH/LH
- Bone profile - hypercalcaemia in MEN1
- HbA1C

Imaging
- MRI pituitary fossa
- Echo
- CXR

Special
- Goldmans perimetry - formally assess visual fields
- Sleep oximetry

60
Q

How is acromegaly managed?

A

Non pharmacological:
- Education
- Support groups and information leaflets
- PT/OT

Medical
- Somatostatin analogues - octreotide - 2nd line
- Dopamine agonist - carbergoline - 3rd line
- GH recepter antagonist - Pegvisomant
- Radiotherapy
- Optimise complications - IHD, diabetes, OSA

Surgery
- Transphenoidal resection - 1st line

Use IGF1 to monitor response to treatment

61
Q

What are the main complications of surgical management of acromegaly?

A

Hypopituitarism
Meningitis
Diabetes

62
Q

How is acromegaly followed up?

A

Regular GH and pituitary tests
ECG
Visual fields assessment

Consider MRI

63
Q

Causes of acromegaly?

A

Most common - benign growth hormone secreting tumour in anterior pituitary

Rare:
- pituitary carcinoma
- GHRH secreting carcinoid tumour
- Hypothalamic tumour

64
Q

What features on history and examination indicate active disease in acromegaly?

A

Headaches
Sweating
Bitemporal hemianopia
HTN
Hyperglycaemia

65
Q

Hereditary conditions which pre-dispose to acromegaly?

A

Carney complex - autosomal dominant. Myxomas of heart and skin. Hyperpigmentation. Endocrine tumours

Familial isolated pituitary adenoma

MEN type1

McCune Albright - new mutation, bony abnormalities, cafe au lait patches, overactive endocrine glands

66
Q

Causes of macroglossia

A

Acromegaly
Amyloidosis
Hypothyroidism
Down’s syndrome

67
Q

Causes of acanthosis nigricans

A

Obesity
T2DM
Acromegaly
Cushing’s
GI ca
Indian individuals

68
Q

Skin conditions associated with diabetes?

A

Diabetic Ulcer
Granuloma Annulare
Diabetic dermopathy
Necrobiosis lipoidica diabeticorum
Diabetic cheiroarthropathy
Acanthosis Nigerians
Eruptive xanthoma
Vitiligo

69
Q

What is a diabetic ulcer?

A

Painless deep ulcer over pressure areas
Associated with peripheral neuropathy and PVD

Managed with weight relieving aids, dressings, ABx and good glycemic control

70
Q

What is granuloma annulare?

A

Round annular, non-tender, non pruritic skin coloured lesion on dorsum of hands, feet and elbows

71
Q

What is diabetic dermopathy?

A

Round atrophic lesions on pre-tibial area
Pigmented due to haemosiderin deposition
Usually multiple and bilateral
Heal with scarring

72
Q

What is necrobiosis lipoidica

A

Well demarcated tender orange-brown patched with central epidermal atrophy
Waxy appearance with overlying telangiectasia
Grow slowly on pre-tibial area
Tendency to ulcerate if injured + risk of secondary infection
Granulomatous reaction to collagen on biopsy

Treat with topical or intralesional steroids

73
Q

What is diabetic cheiroarthropathy

A

Seen in long standing T1DM
Sclerodactyl with limited joint mobility
Thickened, waxy skin

74
Q

What are diabetic bullae?

A

Spontaneous non-scarring, sterile bullae
Can become secondarily infected
Usually affect hands and feet

75
Q

What is acanthosis nigricans?

A

Insulin resistant states

Flexoral surfaces, neck and axillae

Velvety thickening and hyperpigmentation of skin
Associated papillomatosis

Advise weight loss and r/o underlying malignancy

76
Q

What are eruptive xanthomas?

A

Crops of small yellow papules with erythematous rim
Pruritic
Resolve over weeks

Seen in hyperglycaemia and hypertriglyceridaemia

77
Q

What is vitiligo?

A

Hypopigmented patches
Usually sun-exposed areas

Typically with T1DM as co-existing autoimmune disease

78
Q

Systems to examine in patient with diabetic skin disease?

A

Skin - finger prick marks, injection sites (lipoatrophy/lipodystrophy), rashes above, skin folds - candida/staph, hair loss, xanthelasma

CV - pulses

Abdomen - insulin pump/injection sites

Neuro - glove and stocking sensory loss

79
Q

Investigations for patient with diabetic skin disease?

A

Bedside:
- Finger prick glucose
- Fundoscopy - cotton wool spots. Dots, Blot haemorrhages, hard exudates, neovascularisation
- ABPI - PVD

Bloods:
- FBC - anaemia, WCC (infection/inflammation)
- U&E - nephropathy
- Fasting glucose/HbA1C
- Lipids

Imaging
- US - look for stenosis

Special
- Skin biopsy if unclear aetiology

80
Q

How is diabetic skin disease managed?

A

Non pharmacological
- Education
- DSN support

Medical
- Good glycemic control - doesn’t help necrobiosis lipoidica
- Optimise CVS factors
- ?Steroids - necrobiosis
- Abx/Dressings

Surgery may be required

81
Q

What is osteogenesis imperfecta?

A

Autosomal dominant condition predisposing a patient to fractures. Multiple different types

82
Q

Key presentation features of osteogenesis imperfecta

A

Hypermobility
Blue/grey sclera
Triangular face
Short stature
Early deafness
Dental problems
Bone and joint issues
Associated with aortic regurgitation and BAV

83
Q

How would you investigate osteogenesis imperfecta?

A

Bloods:
- Bone profile - usually normal
- Vit D - replace if low

Special
- Genetic screening
- X-Rays
- DEXA - bone density
- Echo - AR risk

84
Q

How is osteogenesis imperfecta managed?

A

Replace any deficiency

If low bone mineral density - bisphosphonates

MDT - orthotics, OT, PT, Specialist nurses, Social workers, orthopaedics, Cardio if AR

85
Q

Things to consider before commencing bisphosphonate therapy

A

Sit upright when taking
Take after meal

Risk of osteonecrosis of jaw - have XR before + regular dental review

86
Q

How does Ehlers Danlos syndrome present?

A

Most common - hyper flexible joints and increased skin elasticity

Other features
- easy bruising
- Widespread joint pain
- Blue sclera
- Tinnitus
- Hernias
- Prolapse - uterocervical/rectal
- CV - MR, MVP, AR
- Aneurysm

87
Q

What are known complications of Ehler’s Danlos?

A

Hypermobile joints - requiring corrective surgery
Cardiovascular - MV prolapse, MR, AR
Vascular - increased risk of haemorrhage, aneurysms
Ophthalm - Glaucoma

88
Q

What is Ehlers Danlos syndrome?

A

Mostly autosomal dominant connective tissue disorder. Some autosomal recessive subtypes + de novo mutations

Abnormal collagen production

6 types
Grouped subtypes - hypermobile, classical, vascular

89
Q

What are some differentials for Ehlers Danlos syndrome?

A

Osteogenesis Imperfecta
Marfans
Loeys-Deitz syndrome

Fibromyalgia and Chronic Fatigue Syndrome

90
Q

How is Ehlers Danlos syndrome investigated?

A

Usually clinical diagnosis
May need echo - valve lesions
CT/MRI - aneurysm
Genetic testing

91
Q

How is Ehlers Danlos syndrome managed?

A

MDT
- PT/OT
- Ortho - fractures
- Cardiology - CV screening
- Genetic counselling
- Vascular - aneurysm repair

Needs pain management

92
Q

What can cause symmetrical deforming polyarthropathies?

A

OA
RA
SLE - Jaccoud’s
Psoriatic
Juvenile idiopathic arthritis

93
Q

Key questions to always ask with joint problems

A

When did it start?
Ever had a rash?
Medications they take - including infusions
Pain, early morning stiffness

94
Q

How would you go about examining someone’s hands?

A

INSPECT!! (use pentorch)
- Scars
- Nail changes
- Rheumatoid nodules
- Deformities
- Rashes
- Muscle bulk
- Palmar erythema
- Look at elbows, neck and back

Palpate - feel for bogginess
Movement - flex, extend, wrist flex and extend, O sign with thumb
Function - pick up coin, write

95
Q

Nail changes associated with psoriasis?

A

Pitting
Thickening
Nail ridging
Onychylosis

96
Q

What other systems can RA commonly affect?

A

RA affects the synovium.
Similar tissue seen in eyes, pleural and pericardial linings –> inflammatory manifestations.

Doesn’t normally affect the spine, however, C1/2 odontoid peg has synovial cover so this can be affected

97
Q

How should you approach a history for cognitive decline?

A
  • Vascular risk factors
  • Ask r.e. hallucinations
  • Changes to movement - slowing etc.
  • Visual disturbances
  • Postural hypotension
  • Falls
  • Risk behaviours - leaving hob on, driving, losing keys
  • Support at home
  • Mood
98
Q

How would you examine a patient with progressive cognitive decline?

A

Check for focal neurology
Pulse - ?AF
CVS review

99
Q

Important tips for all consultation stations

A

Ask about skin changes - if there, ask if photosensitive
If multi systemic + unclear - consider endocrine/rheum cause
Always ask for observations - key part of station is deciding whether the patient needs admission

100
Q

Why may a patient have a liver and kidney transplant?

A

Due to immunosuppressive medications from liver transplant leading to renal failure

Hepatorenal syndrome

101
Q

Scenarios which make you consider tuberous sclerosis?

A

Rash
Headaches
Seizures
SOB
HTN - difficult to control
Poor progress at school

102
Q

Features of tuberous sclerosis?

A

Hamartomas of the skin, brain, kidneys, retina, heart, lungs, liver and bone:

  • Epilepsy
  • Retinal phakomas
  • Adenosebaceous nodules
  • Cutaneous - ash leaf/shagreen patches
  • Renal angiomyolipomas/cysts - renal transplant
  • Rhabdomyomas in heart - murmur
103
Q

Differentials for tuberous sclerosis?

A

Neurofibromatosis
Von Hippel Lindau

104
Q

What does Von Hippel Lindau cause?

A

Cysts:
- Renal
- Pancreas
- Hepatic
- Epididymis

Cerebellar haemangioblastomas
Retinal angiomas
Phaeochromocytoma
Renal Cell Ca

105
Q

Investigations to consider for Tuberous Sclerosis?

A

Bedside:
- BP
- ECG - WPW, LVH, conduction blocks
- Urine dip - haematuria
- Woods lamp - highlight ash leaf
- O2 sats - cystic lung disease

Bloods
- FBC - anaemia
- U&E - renal impairment

Imaging
- Skull X-Ray - railroad track calcification
- MRI head - tubers and subependymal tumours which calcify
- US/MRI Renal - renal cysts/angiomyolipomas
- CT chest - lymphangioleiomyomatosis, pneumothorax, chylus pleural effusion

Special
- Spirometry

106
Q

How is tuberous sclerosis managed?

A

Education - discuss inheritance (AD) and risk of epilepsy
MDT involvement
If epilepsy - DVLA

Medically treat co-morbidities - HTN, renal disease, epilepsy

Surgery - renal transplant, excision of large lesions, arterial embolisation

107
Q

History to consider with cold hands?

A

1 - Confirm whether raynauds - colour change, worse in cold, pain, bilateral

2 consider secondary causes:
- Systemic Sclerosis
- SLE
- Sjogrens

Other differentials:
- Hypothyroid
- Carpal tunnel
- Thoracic outlet syndrome
- Poor peripheral circulation

108
Q

How can Raynaud’s be treated?

A

Calcium channel blockers
Iloprost

109
Q

How do you differentiate between diffuse and limited systemic sclerosis and associated bloods?

A

Diffuse - above elbows - scl70

Limited - below elbows only - centromere

110
Q

Types of psoriatic arthropathy?

A

1 Rheumatoid
2 Mutaloid
3 Oligoid
4 Spondyloarthropathy
5 Distal

111
Q

What are your key differentials if you see DIP arthritis

A

Psoriatic
OA

112
Q

Key causes of spondyloarthropathies?

A

Psoriatic
Ankylosing spondylitis
Reactive arthritis
IBD

HLAB27 associations

113
Q

How is psoriastic arthritis managed?

A

Methotrexate first
Leflonamide or Sulfasalazine

After 2 DMARDS - consider biologics:
- AntiTNF
- Anti IL17
- JAK inhibitors

If spinal involvement - straight to biologics

114
Q

How is Lupus treated?

A

Hydroxychloroquine
Mycophenolate/azathioprine

Rituxamab - reduce B cell activity
Cyclophosphamide - renal/neuropsych

115
Q

6S’s of Dermatomyositis, sarcoidosis, SLE and psoriasis

A

Sun sensitivity - lupus and dermatomyositis
Scalp involvement - SLE and psoriasis
Scarring - discoid lupus and sarcoidosis
Scaly - psoriasis
Spare nasolabial fold - SLE
Systemic
- weakness/muscle pain
- Renal, neuro, CVS affected

MRCP PACES (6 decks)

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