General: fatigue, fever Hands / MSK - arthralgia, myalgia - Jaccoud's arthropathy - non-deforming, resolves with making fist - avascular necrosis of the hip Mucocutaneous - mouth ulcers - photosensitive rashes - malar rash - discoid lupus with scarring alopecia - vasculitic rash - livedo reticularis Cardiorespiratory - pleurisy - ILD - pericarditis Renal - hypertension - nephrotic syndrome - can progress to ESRF Neuropsych Haematological - autoimmune haemolysis - APLS

Pre-proliferative - microaneurysms - dot and blot haemorrhages - cotton wool spots Proliferative - neovascularisation of disc / macula - vitreous haemorrhage

Non specific symptoms - fevers, chills, malaise, headache, myalgia, diarrhoea Severe - sepsis - renal failure - liver failure - coagulopathy / DIC - cerebral involvement

Station 5 Flashcards by Seb Povlsen

Tuberous sclerosis features

AD inheritance (variable penetrance)

Epilepsy
Cognitive deficits
Tuberous CNS masses
Periungal fibromas
Adenoma sebaceum
Retinal phakomas
Shagreen patches (roughened)
Ash leaf marks
Respiratory
- cystic lung disease
- fibrosis
- recurrent pneumathoraces
Abdominal
- polycystic kidneys

Increased risk of renal / CNS malignancy

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Neurofibromatosis features

AD inheritance

>5 cafe au lait spots
axillary freckling 
cutaneous neurofibromas
epilepsy
increased risk of intracerebral tumours
lisch nodules (iris)
CVS: phaeo, coarctation, high blood presure
MSK: scolios, lordosis / kyphosis, fractures / dislocations
Pulmonary fibrosis

NF2 specific

less cutaneous presentation
bilateral acoustic neuromas

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Rheumatoid arthritis features

Hands

Z thumb
boutonniere deformity
swan neck deformity
MCP swelling, subluxation with ulnar deviation
disuse atrophy
carpal tunnel syndrome

Elbows
- rheumatoid nodules

Eyes

keratoconjunctivitis sicca (sjogren’s highly associated)
scleritis / iritis / episcleritis

Respiratory

pleurisy / pleural effusions
ILD

CVS

increased risk IHD
pericardial effusion / pericarditis

Vasculitis
- rash / peripheral neuropathy

Felty’s syndrome: leukopenia, splenomegaly, RA

Atlanto-axial subluxation

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XRAY rheumatoid arthritis

Soft tissue swelling
Loss of joint space
Periarticular osteopenia & erosions
Deformity

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Xray in OA

Loss of joint space
Subchondral sclerosis & cysts
Osteophytes

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Rheumatoid arthritis management

General

PT/OT
MDT for extra-articular features

1) NSAIDs 
\+ start DMARD
---> MTX
2) steroids for flares 
3) biologics - anti TNF e.g. infliximab

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SLE features

General: fatigue, fever

Hands / MSK

arthralgia, myalgia
Jaccoud’s arthropathy - non-deforming, resolves with making fist
avascular necrosis of the hip

Mucocutaneous

mouth ulcers
photosensitive rashes
malar rash
discoid lupus with scarring alopecia
vasculitic rash
livedo reticularis

Cardiorespiratory

pleurisy
ILD
pericarditis

Renal

hypertension
nephrotic syndrome
can progress to ESRF

Neuropsych

Haematological

autoimmune haemolysis
APLS

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SLE management

Acutely with usual low dose maintenance
- prednisolone
Hydroxychloroquine 
Immunosuppressants
- MMF - particularly if renal disease
- MTX
Acute renal failure -> IV cyclophosphamide 
Rituximab
Antiphospholipid -> warfarin

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Hypertensive retinopathy

1) Silver wiring
2) AV nipping
3) Flame haemorrhages, cotton wool spots, exudates
4) Papilloedema

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Diabetic retinopathy

Pre-proliferative

microaneurysms
dot and blot haemorrhages
cotton wool spots

Proliferative

neovascularisation of disc / macula
vitreous haemorrhage

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Features of malaria

Non specific symptoms
- fevers, chills, malaise, headache, myalgia, diarrhoea

Severe

sepsis
renal failure
liver failure
coagulopathy / DIC
cerebral involvement

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Dengue

Initial
- high fever, maculopapular rash, headache, GI upset

Haemorrhagic?

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Typhoid

Fever, rigors, relative bradycardia -> GI upset, rose spots -> GI perforation

Ix: stool mcs

Mx: depends on local resistance, but ceftriaxone

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Causes of traveller’s diarrhoea

E Coli - bloody
Shigella - bloody
Salmonella - bloody
Campylobacter - bloody
Malaria
Typhoid
Dengue
Entomoeba histolytica - bloody
Giardiasis - parasite

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HLA-B27 associations

Psoriasis
Ankylosing spondylitis
IBD
Reactive arthritis

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Associations with SAH

ADPKD
Marfan’s
Ehlers Danlos

Carbimazole counselling

marrow suppression -> must attend if feel feverish / unwell for blood count
explore family plans!
–> start after thyroid disease controlled
–> if planning for family soon, propylthiouracil

Radioiodine cautions

makes Grave’s eye disease worse
cannot be used in pregnancy / breastfeeding
avoid if iodine allergy
avoid close contact with others 12 days, pregnant & children 30 days

Thyroid eye disease grading

0-6 scale

No symptoms -> soft tissue swelling -> proptosis -> extraocular involvement -> corneal involvement -> sight loss

Long-term risks hyperthyroidism

Heart failure, arrhythmias, hypertension, osteoporosis

Complications of OSA

Pulmonary hypertension -> breathlessness, peripheral oedema with progressive right heart failure

Pyrexia unknown origin causes

Malignancy e.g. haematological (B symptoms)
Infection
- HIV
- TB 
- EBV
- subacute infective endocarditis 
- malaria
Rheumatological conditions!

Arterial insufficiency signs

Punched out ulcers
Loss of hair
Shiny skin
Loss of pulses 
Cool peripheries

Causes of peripheral visual field loss

Pituitary pathology
Diabetic retinopathy
Retinitis pigmentosa
Glaucoma
Retinal artery occlusion
Ischaemic optic neuropathy

Retinitis pigmentosa features and associated syndromes

sporadic, AD, AR, X-linked Progressive visual loss from childhood, peripheral, night vision, colour vision first Bone spicule appearance Associated syndromes - refsum's disease (phytanic acid metabolism) - --> deafness - --> cerebellar ataxia - --> peripheral neuropathy - --> icthyosis - --> pes cavus - usher syndrome - + congenital SN hearing loss (commonest cause deaf/blindness UK)

Dermatomyositis signs

``` Mechanic hands Gottron papules knuckles Heliotrope rash V shape sign chest Hyperpigmentation ILD? Proximal myopathy Myositis ```

Anti synthetase syndrome

Anti jo / SRP (synthetase abs) + ILD + polymyositis or dermatomyositis

Polyglandular autoimmune syndromes

Type 1 AIRE AR mutation: chronic mucocutaneous candidiasis, Addison's, hypoparathyroidism, other autoimmune disease Type 2: addison's, type 1 diabetes, hypo/hyperthyroidism

MEN

MEN1 - AD - pituitary adenoma - pancreatic tumour - parathyroid hyperplasia MEN2a - AD - medullary thyroid - phaeochromocytoma - parathyroid hyperplasia MEN2b - AD - medullary thyroid - phaeo - marfanoid - neurofibromas

Methotrexate counselling

- immunosuppressant -> seek medical advice if feel unwell - common SEs: nausea, diarrhoea, mouth ulcers, hair loss -> all improved by taking folic acid - baseline bloods, CXR, LFTs, renal function - avoid etoh - avoid pregnancy

Rheumatoid arthritis diagnostic criteria

American college of Rheumatology criteria - number of joints - size of joints - duration of symptoms - positive serology - elevated inflammatory markers

Causes of recurrent VTE

RFs VTE - immobilisation - long haul travel - surgery - malignancy - drugs: OCP, HRT Thrombophilias - APLS - Factor V leiden - Protein C / S deficiency - Antithrombin deficiency - Active malignancy - Nephrotic syndrome Other conditions - nephrotic syndrome - IBD

Well's score DVT & VTE

PE >4 - PE likely -> CTPA 4 or less -> D dimer first DVT 3 or higher - DVT likely -> duplex <3 -> D-dimer first 1. 5x kg weight enoxaparin - > NOACs e.g. rivaroxaban 3 months if known trigger 6 months if none Consider longer if cancer / other active disease ongoing

MTX toxicity mx

Stop methotrexate and statin Repeat LFTs in 2 weeks Monitor +/- consider restarting (altered dose) or switch

PE severity PESI score in PE

Massive - hypotensive <90mmHg, bradycardia, cardiac arrest Submassive - RV strain/dysfunction, raised troponin PESI 0 - can be managed as OP Otherwise manage as IP

Systemic sclerosis management

``` Telangiectasia: camouflage creams Scleroderma: immunosuppression HTN: ACEi Raynaud's: hand warmers, calcium channel blockers Reflux: PPI / antacids Oesophageal dysmotility: prokinetics ILD: immunosuppression, transplant PHT: sildenafil / bosentan Inflammatory arthritis: low dose steroid taper (beware scleroderma renal crisis) ```

Risk factors / associations IIH

``` Female Obesity Cushing's Hypothyroidism PCOS Systemic disease: CKD Drugs: GH, tetracyclines, (OCP) ```

Migraine

Aura Unilateral Dark room, photophobia, phonophobia RFs: etoh, caffeine, poor sleep, stress, diet, menstrual cycle Mx - Acute - --> simple analgesia - --> triptans - --> metoclopramide - Chronic - --> propranalol - --> topiramate - --> lifestyle

Cluster headache management

``` Acute - oxygen - SC / nasal triptan Chronic - verapamil ```