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Phase 4 Question Knowledge > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

RA

A
  • Chronic autoimmune inflammation of synovial joints
  • inflammation of synovial lining, tendon sheaths, bursae
  • symmetrical polyarthritis - 5+ joints affected
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2
Q

RA Px

A
  • joint pain
  • stiffness - >30mins in morning, better with activity
  • inflammation - red, hot, swollen
  • fatigue, malaise, wt loss, myalgia
  • symmetrical distal polyarthritis - small joints of hands / feet
  • ankle, knee, hips, shoulders, c-spine
  • atlanto-axial subluxation

Hand sx

  • wrist, MCP, PIP, MTP joints affected
  • swan neck (flexed DIP)
  • Boutonniere (flexed PIP)
  • z thumb
  • ulnar deviation
  • subluxation
  • positive squeeze test - MCP/MTP joints

Extra-articular sx

  • pulm fibrosis
  • Felty’s syndrome - RA + neutropenia + splenomegaly
  • Sjogren’s
  • anaemia chronic disease
  • CV disease
  • rheumatoid nodules
  • lymphadenopathy
  • CTS
  • amyloidosis
  • bronchiolitis obliterans
  • Caplan syndrome - pulmonary nodules

Eye sx

  • Dry eye syndrome – keratoconjunctivitis sicca
  • episcleritis, scleritis, keratitis
  • cataracts (steroids)
  • retinopathy
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3
Q

RA Ix

A
  • FBC, CRP/ESR, RF, anti-CCP
  • XR - LESS - loss of joint space, erosions (bony), soft tissue swelling, soft bones
  • US / MRI - if synovitis unclear
  • HAQ / DAS28 scoring systems
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4
Q

RA Mx

A
  • physio, podiatry
  • DAS28, CRP to monitor tx
  • paracetamol, NSAIDs
  • prednisolone - at initial px / starting new tx / flares
  • DMARDs - methotrexate, sulfasalazine, leflunomide, hydroxychloroquine
  • biological therapy - infliximab / etanercept (TNF inhibitors), rituximab (anti-CD20)
  • surgery
  • pregnancy - hydroxychloroquine / sulfasalazine + folic acid
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5
Q

Psoriatic arthritis

A
  • inflammatory arthritis with psoriasis
  • HLAB27, RF-

5 patterns of disease

  • Asymmetrical oligoarthritis – 1-4 joints, one side of body
  • Symmetrical polyarthritis – like RA
  • DIP predominant pattern – primarily DIP joints affected
  • Spondylitis – back stiffness, pain – axial skeleton – spine + SI joints
  • Arthritis mutilans – phalanges – osteolysis (destruction), shortening of digits – telescoping digit
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6
Q

PsA Px

A
  • joint pain, swelling, stiffness
  • psoriatic plaques on skin - knees, elbows, behind ear, scalp
  • nail pitting
  • onycholysis
  • dactylitis
  • enthesitis
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7
Q

PsA Ix

A
  • bloods - RF-, high ESR/CRP
  • Psoriasis Epidemiological Screening Tool (PEST)
  • XR - erosions central in joint (pencil in cup deformity), periostitis, ankylosis, osteolysis, dactylitis
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8
Q

PsA Mx

A
  • physio, exercise, surgery
  • NSAIDs
  • steroids - can be intra-articular
  • DMARDs
  • anti-TNF
  • ustekinumab
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9
Q

Reactive arthritis

A
  • joint inflammation in autoimmune response to infection
  • HLAB27
  • Post-STI - SARA - sexually acquired reactive arthritis
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10
Q

ReA Px

A
  • <4wks of infection, may last 4-6mo
  • asymmetrical joint pain, warm, red, stiff, swollen
  • malaise, fatigue, fever
  • dactylitis
  • mouth ulcers
  • Reiter’s - bl conjunctivitis, anterior uveitis, urethritis, enthesitis / arthritis
  • skin changes, nail dystrophy
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11
Q

ReA Ix

A
  • bloods - ESR / CRP raised
  • STI screen, GUM referral
  • stool culture
  • XR - enthesitis
  • joint aspiration - MC+S, crystal examination
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12
Q

ReA Mx

A
  • NSAIDs
  • steroid injection
  • tx infection
  • if sx >6mo - DMARDs, anti-TNF
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13
Q

Ankylosing spondylitis

A
  • inflammatory arthritis of axial skeleton - spine + SI joints
  • HLAB27
  • enthesitis inflammation, syndesmophyte formation, fibrosis + ossification, ankylosis
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14
Q

AS Px

A
  • lover back pain, stiffness
  • pain at night, worse in morning, >30mins, improves with movt
  • peripheral arthritis 1-2 joints
  • kyphosis

Extraarticular features

  • Anterior uveitis
  • Amyloidosis
  • Apical lung fibrosis
  • Aortic regurg
  • AV node block
  • Achilles tendonitis
  • Anaemia of chronic disease

SPINEACHE

  • Sausage digit (dactylitis)
  • Psoriasis
  • Inflammatory back pain
  • NSAID good response
  • Enthesitis (heel)
  • Arthritis
  • Crohn’s/colitis/elevated CRP (but can be normal)
  • HLAB27
  • Eye (uveitis)
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15
Q

AS Ix

A
  • Schober’s - reduced spinal flexion
  • Bloods - raised ESR/CRP, anaemia, HLAB27+
  • spirometry - restrictive
  • XR spine/sacrum - sacroiliitis, fusion of joints, ossification, subchondral sclerosis, squaring of vertebrae, bamboo spine, syndesmophites
  • CXR - apical fibrosis
  • MRI - bone marrow oedema
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16
Q

AS Mx

A
  • Exercise, OT/PT, stop smoking
  • NSAIDs
  • DMARDs
  • Anti-TNF, MAbs, JAK inhibitor
  • intra-articular steroids
  • surgery - joint deformity
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17
Q

Osteomalacia

A
  • soft bones - decreased bone mineral content + low vit D
  • uncalcified osteoid + cartilage
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18
Q

Osteomalacia causes

A
  • vit D deficiency
  • CKD
  • Drugs - AEDs
  • liver cirrhosis
  • tumour induced
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19
Q

Osteomalacia Px

A
  • bone pain
    bone / muscle tenderness
  • fractures - eg femoral neck
  • proximal myopathy - waddling gait
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20
Q

Osteomalacia Ix

A
  • bloods - low 25-OH vit D (low 1,25-OH vit D in renal failure), low Ca, phos, raised ALP, high PTH
  • XR - translucent bands - Looser’s zones
  • DEXA scan - low bone mineral density
  • (biopsy)
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21
Q

Osteomalacia Mx

A
  • vit D supplementation - colecalciferol / calcitriol
  • Ca supplementation
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22
Q

Osteoporosis

A
  • low bone mass -> fragile bones, fractures
  • osteopenia - less severe, reduced bone mass
  • hip, vertebral, colles - common
  • post-menopause - lack of oestrogen -> increased bone turnover (resorption>formation)
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23
Q

Osteoporosis RFs

A
  • older
  • female
  • post-menopause
  • reduced mobility / activity
  • low BMI
  • low Ca / vit D intake
  • alcohol, smoking
  • Hx / FHx fractures
  • chronic disease - CKD, hyperthyroid, RA, Cushing’s
  • testosterone deficiency - hypogonadism
  • long-term steroids
  • Meds - SSRIs, PPIs, AEDs, anti-oestrogens, Depo-provera
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24
Q

Osteoporosis Px

A
  • asym
  • increased fractures
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25
Q

Osteoporosis Ix

A
  • FRAX
  • QFracture Tool
  • DEXA scan (femoral neck) - >-1 normal, -1 to -2.5 osteopenia, <-2.5 osteoporosis, +# is severe osteoporosis
  • XR
  • for secondary causes - FBC, U/E, CRP/ESR, bone profile, TFT, LFTs
  • > 75yo with fragility# + suspected osteoporosis - start on bisphosphonate (<75yo, will need FRAX / DEXA)
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26
Q

Osteoporosis Mx

A
  • healthy weight, exercise, stop smoking, reduce alcohol
  • adequate vit D / Ca intake
  • Tx if T<-2.5
  • bisphosphonates - alendronate
  • denosumab
  • teriparatide
  • HRT
  • raloxifene
  • strontium ranelate
  • > 75yo with fragility# + suspected osteoporosis - start on bisphosphonate (<75yo, will need FRAX / DEXA)
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27
Q

Paget’s disease of bone

A
  • focal bone remodelling due to increased bone turnover
  • increased osteoclast/blast activity, new bone weaker, fibrous
  • skull, spine/pelvis, femurs
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28
Q

Paget’s Px

A
  • asym 60-80%
  • bone pain
  • bowing of tibia, bossing of skull
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29
Q

Paget’s Ix

A
  • Bloods - raised ALP
  • raised urinary hydroxyproline
  • XR - local bony enlargement, sclerotic changes, osteolytic areas, skull (thickened vault, osteolytic lesions, cotton wool appearance)
  • bone scintigraphy
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30
Q

Paget’s Mx

A
  • bisphosphonates
  • calcitonin
  • analgesia - NSAIDs
  • Ca / vit D supplements
  • surgery - fracture / deformity
  • monitor with ALP / sx
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31
Q

Gout

A
  • joint inflammation due to deposition of monosodium urate (MSU) crystals in joint
  • high serum uric acid levels (from purines)
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32
Q

Gout causes

A

Decreased excretion

  • drugs - thiazides
  • CKD
  • lead toxicity
  • alcohol
  • obesity
  • DM

Increased production

  • high purine diet - meat, seafood
  • hyperlipidaemia
  • alcohol

….

RFs - male, FHx, CVD

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33
Q

Gout Px

A
  • acute flare episodes
  • pain
  • swelling
  • erythema
  • most 1st MTPJ, also 1st MCPJ, ankle, wrist, knee
  • gouty tophi - hands, elbows, ears
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34
Q

Gout Ix

A
  • serum uric acid
  • Joint aspiration - MC+S, polarised light microscopy - needle shaped negatively birefringent crystals
  • XR - joint effusion, punched out erosions, sclerotic margins, lytic lesions in bone, joint space preserved
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35
Q

Gout Mx

A

Acute flares

  • NSAIDs (+PPI)
  • colchicine
  • oral steroids - prednisolone
  • consider intra-articular steroids

Prophylaxis

  • lose weight, hydrate, minimise purine intake, alcohol
  • allopurinol
  • febuxostat
  • don’t start until >4wks after attack, continue during future attacks
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36
Q

Pseudogout

A
  • acute inflammation of joint from deposition of calcium pyrophosphate (CPP) crystals
  • > 65yo
  • in younger pts - HHC, hyperparathyroid, low Mg, low phos, acromegaly, Wilson’s
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37
Q

Pseudogout Px

A
  • asym
  • pain, stiffness, swelling, hot joint
  • knee, shoulder, hip, wrist
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38
Q

Pseudogout Ix

A
  • joint aspiration - MC+S, polarised light microscopy - positively birefringent rhomboids
  • XR - chondrocalcinosis, loss of joint space, osteophytes, sclerosis, cysts
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39
Q

Pseudogout Mx

A
  • NSAIDs + PPI
  • colchicine
  • intra-articular steroid injection / oral steroids
40
Q

Fibromyalgia

A
  • syndrome of widespread chronic pain, specific tenderness
41
Q

Fibromyalgia Px

A
  • chronic pain - specific sites / all over
  • lethargy
  • cognitive impairment - fibro fog
  • sleep disturbance, headaches, dizziness
42
Q

Fibromyalgia Ix

A
  • clinical dx
  • America College of Rheumatology - criteria - lists 18 tender points - if tender in 11/18, dx more likely
43
Q

Fibromyalgia Mx

A
  • Educate about condition
  • Aerobic exercise
  • CBT
  • Pregabalin, duloxetine, amitriptyline
44
Q

Systemic lupus erythematosus (SLE)

A
  • inflammatory autoimmune multi-system disorder
45
Q

SLE Patho

A
  • relapsing-remitting - flares of worse sx
  • Anti-nuclear ABs (ANA) - auto-ABs against cell nucleus -> chronic inflammatory response
  • T3 hypersensitivity - immune complex deposition
  • flare triggers - sun, oestrogen, pregnancy, drugs, infection, stress

Drug-induced lupus

  • eg procainamide, hydralazine, isoniazid, phenytoin
  • arthralgia, myalgia, malar rash, pleurisy
46
Q

SLE Px

A

General

  • Fatigue
  • Weight loss
  • Fever
  • Lymphadenopathy
  • Mouth ulcers
  • Splenomegaly

Skin

  • Photosensitive malar rash
  • Raynaud’s
  • Hair loss
  • Livedo reticularis
  • Discoid rash

MSK

  • arthralgia (polyarticular), Jacoud’s arthropathy
  • arthritis
  • myalgia

CV

  • pericarditis
  • myocarditis

Resp

  • SOB
  • pleuritic CP

Renal

  • proteinuria
  • glomerulonephritis / oedema

Neuropsych

  • anxiety, depression
  • psychosis
  • seizures
47
Q

SLE Ix

A
  • auto-AB screen - ANA, anti-dsDNA, anti-Sm
  • FBC - anaemia, low WCC/platelets
  • low C3/4
  • urinalysis, urine protein:creatinine ratio
  • renal biopsy
48
Q

SLE Mx

A
  • sunscreen / avoid sun
  • NSAIDs
  • hydroxychloroquine
  • steroids - prednisolone
  • DMARDs
  • biological - rituximab, belimumab
49
Q

SLE Cx

A
  • CV disease
  • Infection
  • Anaemia
  • Pericarditis
  • Pleuritis
  • Interstitial lung disease
  • Lupus nephritis
  • Neuropsychiatric SLE - inflammation in CNS - optic neuritis, transverse myelitis, psychosis
  • Recurrent miscarriage
  • VTE
50
Q

Discoid lupus erythematosus

A
  • autoimmune chronic skin condition
  • follicular keratin plugs
  • <5% progress to SLE

Px

  • erythematous, raised rash, +/- scaly
  • photosensitive
  • face, neck, ears, scalp
  • lesions heal with atrophy, scarring, pigmentation, alopecia

Ix

  • skin biopsy

Mx

  • topical steroid cream
  • hydroxychloroquine
  • avoid sun exposure
51
Q

Systemic sclerosis

A
  • autoimmune disorder - inflammation + fibrosis of connective tissues
  • increased fibroblast activity + collagen deposition

Scleroderma

  • hardening of skin

Limited cutaneous SSc

  • CREST

Diffuse cutaneous SSc

  • CREST and internal organs
52
Q

SSc Px

A

Scleroderma

  • hardening of skin - shiny/tight

Limited - CREST

  • calcinosis
  • Raynaud’s
  • (o)esophageal dysmotility - reflux/dysphagia
  • sclerodactyly
  • telangiectasia

Diffuse

  • CV - HTN, coronary heart disease, arrhythmias
  • Lung - pulm HTN, pulm fibrosis - dry cough, SOB
  • Kidney - glomerulonephritis, scleroderma renal crisis (HTN, renal failure)
53
Q

SSc Ix

A
  • bloods - anaemia, raised ESR, auto-ABs - anti-centromere (limited), anti-Scl-70 (diffuse), RF+, ANA+
  • urinalysis - AKI, proteinuria
  • CXR, hand XR, barium swallow, HRCT
  • nailfold capillaroscopy
54
Q

SSC Mx

A
  • Mx raynauds
  • stop smoking, emollients, OT/PT
  • PPI, analgesia, abx, ACEi,
  • bosentan, sildenafil
  • IV iloprost - digital ulcers
  • cyclophosphamide / pred
  • stem cell transplant
55
Q

Raynaud’s

A
  • episodic vasospasm in fingers (rarely toes) in response to cold/stress

Primary

  • Raynaud’s disease

Secondary

  • scleroderma, RA, SLE
  • leukaemia, cryoglobulinaemia
  • vibrating tools
  • OCP
  • cervical rib

Factors suggesting connective tissue disease

  • > 40yo, unilateral, rashes, auto-ABs, sx of RA / SLE, digital ulcers, calcinosis, abnormal nail-fold capillaries
56
Q

Raynaud’s Px

A
  • white -> blue -> red discolouration of fingers
  • ischaemia -> deoxygenation -> reactive hyperaemia
  • usually bilateral
57
Q

Raynaud’s Mx

A
  • secondary care referral if <12yo, ?secondary, poor sx control
  • keep body warm, stop smoking, exercise
  • nifedipine
  • IV prostacyclin
  • losartan, ACEi, sildenafil, fluoxetine
  • admit if severe ischaemia
58
Q

Sjogren’s syndrome

A
  • autoimmune destruction of exocrine glands
  • primary / secondary (RA, SLE, SSc)
  • lymphocytic infiltration / fibrosis of glands
59
Q

Sjogren’s Px

A
  • dry eyes - blurry, itchy, red, burning
  • dry mouth, caries - cracks/fissures, difficulty swallowing, dry cough
  • parotid swelling
  • nasal septum crusting/bleeding
  • vaginal dryness, dyspareunia
  • systemic signs - polyarthritis, Raynaud’s, lymphadenopathy, fatigue, vasculitis
60
Q

Sjogren’s Ix

A
  • Bloods - anti-Ro, anti-La ABs, RF+ 50%, ANA+ 70%
  • Schirmer’s test
61
Q

Sjogren’s Mx

A
  • artificial tears
  • artificial saliva
  • vaginal lubricants
  • oral pilocarpine
  • hydroxychloroquine (consider)
62
Q

Myositis

A
  • autoimmune inflammation / necrosis of muscles
  • polymyositis - muscle proteins attacked
  • dermatomyositis - muscle, skin, other organs attacked
  • malignancy association, also Coxsackie, HIV
63
Q

Myositis Px

A

Polymyositis

  • symmetrical progressive proximal muscle weakness - shoulders, hips
  • dysphagia, dysphonia, resp failure
  • fatigue, myalgia, muscle cramps
  • hand fine motor movts affected

Dermatomyositis

  • as above
  • heliotrope
  • periorbital oedema
  • Gottron’s sign
  • photosensitive erythematous rash on back, shoulders, neck
  • GI ulcers, infections

Extra-muscular sx

  • fever, arthralgia, Raynaud’s, lung fibrosis
64
Q

Myositis Ix

A
  • bloods - elevated CK, LDH, aldolase, AST, ALT
  • auto-ABs - anti-Jo-1, ANA+ 80%
  • EMG
  • MRI, CXR, PFTs, HRCT
  • muscle biopsy
65
Q

Myositis Mx

A
  • OT/PT
  • oral prednisolone
  • immunosuppressants - methotrexate, azathioprine
  • IV Ig
  • biologics - infliximab, etanercept
66
Q

Behcet’s disease

A
  • complex autoimmune inflammatory condition affecting vessels / tissues
67
Q

Behcet’s Px

A
  • oral ulcers + genital ulcers + anterior uveitis
  • thrombophlebitis, DVT, aneurysms
  • arthritis
  • Neuro - aseptic meningitis, cerebral venous sinus thrombosis
  • GI - abdo pain, diarrhoea, colitis
  • Skin - erythema nodosum, papules / pustules like acne
  • Sx vary, van be relapsing-remitting
68
Q

Behcet’s Ix

A
  • clinical dx
  • positive pathergy test
69
Q

Behcet’s Mx

A
  • soluble betamethasone tablets - mouth ulcers
  • oral prednisolone
  • colchicine
  • lidocaine ointment - genital ulcers
  • immunosuppressants - azathioprine
  • biologic - infliximab
70
Q

Chronic fatigue syndrome (CFS)

A
  • Disabling fatigue affecting physical / mental function >50% of the time, in the absence of other disease explaining sx
71
Q

CFS Px

A
  • Fatigue (suspect after >6wks), >50% of the time
  • Sleep problems
  • Muscle / joint pains
  • Headaches
  • Painful lymph nodes, without enlargement
  • Sore throat
  • Cognitive dysfunction - difficulty thinking, memory impairment
  • Sx worse with physical / mental exertion
  • Malaise, flu-like sx
  • Dizziness
  • Nausea
  • Palpitations
72
Q

CFS Ix

A
  • Bloods to r/o other causes - FBC, U/E, LFTs, glucose, HbA1c, TFT, ESR, CRP, Ca, phosph, CK, ferritin, coeliac screen, urinalysis
  • clinical dx
  • sx >3mo
73
Q

CFS Mx

A
  • refer to CFS service
  • energy management
  • physical exercise / activity
  • CBT
74
Q

Vasculitis

A
  • inflammation of blood vessels
  • also necrosis, vessel wall destruction, thrombosis, ischaemia
  • anaemia, raised ESR

Classification

Large vessel

  • GCA/temporal arteritis/PMR
  • Takayasu’s arteritis

Medium vessel

  • polyarteritis nodosa
  • Kawasaki’s

Small vessel

ANCA associated

  • microscopic polyangiitis (p-ANCA - perinuclear)
  • granulomatosis with polyangiitis (c-ANCA - cytoplasmic)
  • eosinophilic granulomatosis with polyangiitis (p-ANCA)

Immune complex

  • essential cryoglobulinaemia
  • HSP
  • Goodpasture’s
  • cutaneous leucocytic vasculitis
75
Q

Vasculitis general Px

A
  • Joint / muscle pain
  • Peripheral neuropathy
  • Renal impairment
  • Purpura
  • Necrotic skin ulcers
  • GI sx - diarrhoea, abdo pain, bleeding
  • Fatigue
  • Fever
  • Night sweats
  • Wt loss
  • Anorexia
  • Anaemia
76
Q

Vasculitis general Ix

A
  • CRP / ESR raised
  • check ANCA
77
Q

Vasculitis general Mx

A
  • steroids
  • cyclophosphamide / rituximab
78
Q

Polymyalgia rheumatica (PMR)

A
  • inflammatory condition causing pain/stiffness in shoulders, pelvic girdle, neck
  • GCA associated
79
Q

PMR Px

A
  • rapid onset days/wks
  • sx present >2wks for PMR dx
  • pain / stiffness - shoulders, pelvic girdle, neck
  • worse in morning/after rest
  • sleep interference
  • > 45mins morning stiffness
  • systemic - wt loss, fatigue, fever
  • muscle tenderness
  • CTS
  • peripheral oedema
80
Q

PMR Ix

A
  • raised ESR/CRP
  • bloods to r/o ddx - FBC, U/E, LFTs, Ca, serum protein electrophoresis, TFTs, CK, RF, urine dip
  • consider - ANA, anti-CCP, urine bence-jones, CXR
81
Q

PMR Mx

A
  • oral prednisolone 15mg - reducing regime (may last 1-2yrs)

Long term steroid mx - dont STOP

Don’t - stop abruptly

S - sick day rules - increase dose when unwell

T - Tx card

O - osteoporosis - bisphosphonates, Ca, vit D

P - PPI - omeprazole

82
Q

Temporal arteritis / GCA

A
  • vasculitis of medium/large arteries
  • 50% of GCA pts will have PMR
  • ocular cx - from anterior ischaemic optic neuropathy - occlusion of posterior ciliary artery (branch of ophthalmic)
83
Q

GCA Px

A
  • onset <1mo
  • unilateral headache
  • jaw claudication
  • amaurosis fugax
  • scalp tenderness
  • tender / thickened artery
  • PMR sx
  • lethargy, depression, fever, anorexia, night sweats
  • muscle tenderness, CTS, peripheral oedema
84
Q

GCA Ix

A
  • raised ESR, maybe CRP
  • fundoscopy
  • temporal artery biopsy - skip lesions, multinucleated cells
  • Duplex USS
85
Q

GCA Mx

A
  • steroids - prednisolone / IV methylpred - wean over 1-2yrs
  • ophthal / rheum referrals, maybe vascular
  • bisphosphonates, vit D, Ca, PPI
  • low dose aspirin
86
Q

Takayasu’s arteritis

A
  • large vessel vasculitis - aorta, branches, pulmonary arteries
  • vessels swell - aneurysms / narrow + block

Px

  • <40yo
  • fever, malaise, muscle aches
  • claudication sx - upper/lower limbs
  • unequal BP in limbs
  • carotid bruit / tenderness
  • absent / weak peripheral pulses
  • aortic regurg 20%

Ix

  • CT / MRI angiography

Mx

  • steroids
87
Q

Polyarteritis nodosa (PAN)

A
  • medium-vessel vasculitis, necrotising inflammation, aneurysms
  • associated with hep B

Px

  • fever, malaise, arthralgia, wt loss, HTN
  • mononeuritis multiplex
  • testicular pain, livedo reticularis, haematuria, renal failure
  • MI, stroke, mesenteric arteritis (GI pain), tender, erythematous skin nodules

Ix

  • pANCA (20%), hep B serology, anaemia, raised WCC/ESR
  • Biopsy - eg kidney
  • angiography

Mx

  • ACEi
  • prednisolone
  • immunosuppression - azathioprine / cyclophosphamide
  • tx HBV - antivirals
88
Q

ANCA-associated vasculitis

A

Anti-neutrophil cytoplasmic ABs (ANCA), associated with

  • Microscopic polyangiitis – pANCA – perinuclear
  • Granulomatosis with polyangiitis – cANCA – cytoplasmic
  • Eosinophilic granulomatosis with polyangiitis – pANCA

Common features

  • Renal – immune complex glomerulonephritis -> raised creatinine, haematuria, proteinuria
  • Resp – SOB, haemoptysis
  • Systemic – fatigue, fever, wt loss
  • Vasculitic rash – in minority
  • ENT – sinusitis

General Ix

  • urinalysis
  • bloods - FBC, CRP, ANCA
  • CXR

General Mx

  • renal / rheum / resp referral
  • kidney / lung biopsies
  • immunosuppression
89
Q

Microscopic polyangiitis

A
  • small vessel vasculitis
  • glomerulonephritis -> renal failure - raised creat, haematuria, proteinuria
  • lungs - diffuse alveolar haemorrhage, haemoptysis, cough, SOB
  • fever, lethargy, myalgia, wt loss
  • mononeuritis multiplex
  • pANCA in 50-75%, cANCA 40%
90
Q

Granulomatosis with polyangiitis

A
  • Wegener’s granulomatosis - small vessel vasculitis affecting lungs + kidneys

Px

  • epistaxis, nose crusting
  • saddle-shaped nose
  • sinusitis
  • hearing loss
  • cough, wheeze, haemoptysis
  • glomerulonephritis
  • vasculitic rash, proptosis, CN lesions

Ix

  • cANCA+
  • CXR
  • renal biopsy

Mx

  • steroids
  • cyclophosphamide
  • plasma exchange
91
Q

Eosinophilic granulomatosis with polyangiitis

A
  • Churg-Strauss syndrome
  • small vessel vasculitis affecting lungs, skin, kidneys…

Px

  • severe asthma
  • sinusitis, allergic rhinitis
  • mononeuritis multiplex

Ix

  • FBC - raised eosinophils
92
Q

Antisynthetase syndrome

A

Syndrome caused by anti-Jo1 autoABs
Characterised by
- Myositis
- ILD
- Thickened / cracked skin of hands
- Raynaud’s

93
Q

Hypersensitivity

A

Type 1 - anaphylactic

  • Ag reacts with IgE bound to mast cells
  • anaphylaxis, atopy

Type 2 - cell bound

  • IgM / IgG binds to Ag on cell surface
  • AIHA, ITP, Goodpasture’s, pernicious anaemia, acute haemolytic transfusion reaction, rheumatic fever

Type 3 - immune complex

  • free Ag and Ab (IgG/IgA) combine
  • SLE, post-strep glomerulonephritis, EAA

Type 4 - delayed hypersensitivity

  • T cell mediated
  • TB, graft vs host disease, scabies…

(Type 5 - ABs bind to cell receptors)

  • Graves, MG
94
Q

Langerhans cells histiocytes

A

Abnormal proliferation of histiocytes – childhood, bony lesions

Px

  • Bone pain, typically in the skull or proximal femur
  • Cutaneous nodules
  • Recurrent otitis media/mastoiditis
  • Tennis racket-shaped Birbeck granules on electromicroscopy
95
Q

McArdle’s disease

A
  • autosomal recessive type V glycogen storage disease
  • myophosphorylase deficiency -> decreased muscle glycogenolysis

Px

  • Muscle pain, stiffness after exercise
  • Muscle cramps
  • Myoglobulinuria
  • Low lactate during exercise
96
Q

Still’s disease in adults

A
  • Bimodal age distribution – 15-25yo, 35-46yo

Px

  • arthralgia
  • salmon pink, maculopapular rash
  • fever
  • lymphadenopathy

Ix

  • raised serum ferritin
  • RF / ANA -
  • Yamaguchi criteria to dx

Mx

  • NSAIDs
  • Steroids
  • consider methotrexate, biologics

Phase 4 Question Knowledge (34 decks)

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