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Year 6 > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

Differentials for an acute monoarthritis

A

Gout, pseudogout, pyogenic septic arthritis, reactive arthritis, haemarthrosis, osteomyelitis, monoarticular rheumatoid arthritis, osteonecrosis

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2
Q

Define Adult-onset Still’s disease and give its features

A

An idiopathic autoinflammatory condition which commonly presents in young adulthood - characterised by a triad of arthritis (commonly knees, wrists and fingers), spiking fevers and a transient salmon-pink maculopapular rash

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3
Q

Adult-onset Still’s disease investigations

A

Hyperferritinaemia (1500 to >10,000), marked raised inflammatory markers, leucocytosis w/ neutrophilia

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4
Q

What are the Yamaguchi classification criteria?

A

Criteria used to diagnose Adult-onset Still’s disease - need to meet 5 criteria (including 2 major):

Major:
- Fever >39 for more than 7 days
- Arthralgias or arthritis for >2 wks
- Characteristic rash
- Leucocytosis w/ >80% neutrophils

Minor:
- Sore throat
- Lymphadenopathy
- Hepato/splenomegaly
- Raised aminotransferase
- -ve RF and ANA

Exclusion:
- Malignancy
- Infections
- Other CNT disorders
- Familial autoinflammatory conditions
- Drug reactions

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5
Q

Adult-onset Still’s disease management

A

NSAIDs and aspirin for mild disease, low-dose corticosteroids added when that isn’t enough

Severe disease w/ life-threatening organ manifestations require high-dose pulsed IV corticosteroids

Biological therapies = IL-1, TNF-alpha or IL-6 blockers

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6
Q

Ankylosing spondylitis features

A
  • Inflammatory back pain - often early morning stiffness (gets better w/ activity), w/ tenderness of the sacroiliac joints and limited range of spinal motion on examination
  • Enthesitis (inflammation of where ligaments attack to bone) - esp of Achilles tendon and plantar fascia
  • Peripheral arthritis (1/3 or pts) - hips and shoulders
  • Extra-articular involvement = anterior uveitis, aortitis and aortic regurgitation, upper lobe pulmonary fibrosis, IgA nephropathy
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7
Q

Ankylosing spondylitis physical examination findings

A
  • Restricted movements in lumbar spine
  • Dorsal kyphosis of the thoracic spine may be present as the disease progresses - can lead to reduced chest expansion
  • C-spine movements can be globally reduced, with the neck forced into flexion by dorsal kyphosis
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8
Q

Ankylosing spondylitis imaging results

A

Pelvic X-ray:
- Sacroiliitis (better seen on MRI)
- Ankylosis or fusion of sacroiliac joint is seen in advanced disease

Lumbar X-ray:
- Squaring of vertebral bodies
- Syndesmophyte formation (bony bridges) between adjacent vertebrae + ossification of the spinal ligaments
- Complete fusion of the vertebral column (bamboo spine) in advanced disease

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9
Q

Ankylosing spondylitis management

A

Conservative: - critical to improve and maintain posture, flexibility and mobility
- Exercise
- Physio

Medical:
- NSAIDs + PPI 1st line
- DMARDs (sulfasalazine and methotrexate) for pts w/ peripheral joint disease - these don’t improve spinal inflammation
- Local steroid injection
- Biologics - anti-TNF 1st line, ant0IL17 2nd line

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10
Q

Antiphospholipid syndrome features

A

Mneumonic= CLOT

C = clots - usually venous thromboembolisms, but can be arterial
L = Livedo reticularis - a mottled, lace-like appearance on the skin of the lower limbs
O = obstetric loss
T = Thrombocytopenia

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11
Q

Antiphospholipid syndrome management

A

Prophylactic lose-dose aspirin

Control of thromboembolic RFs

In pts who have a venous thromboembolism = life long warfarin is used with a INR target of 2-3

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12
Q

How long should mechanical back pain take to clear up?

A

6 weeks

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13
Q

Red flag symptoms for back pain

A
  • New onset when aged <20 or >55
  • Thoracic or cervical spine pain
  • Pain is progressive and not relieved by rest - suggestive of infection or cancer
  • Spinal (rather than paraspinal) tenderness
  • Early morning stiffness >30 min = ankylosing spondylitis
  • Abnormal LL neuro signs = cord compression
  • Trauma or sudden onset pain in pts w/ RFs for osteoporosis = possible vertebral fracture
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14
Q

Define Behcet’s disease

A

A rare multiorgan disease caused by systemic vasculitis of arteries and veins of all sizes, hypercoagulability and neutrophil hyperfunction - has an unknown cause, but is more common in Turks, Mediterranean’s and the Japanese

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15
Q

Behcet’s disease features

A
  • Recurrent oral/genital ulceration
  • Anterior or posterior uveitis
  • Erythema nodosum
  • Vasculitis
  • Acneiform lesions
  • Superficial thrombophlebitis at venepuncture sites
  • Pathergy = pustules at sites of puncture or minor trauma
  • Arthralgias and arthritis

Uncommon:
- Neuro involvement = cerebral vasculitis, venous sinus thrombosis
- Reso involvement = pulmonary vasculitis, PE, pulmonary haemorrhage
- GI vasculitis = abdo pain, mesenteric angina, constipation
- Cardiovascular involvement = MI, deep vein thrombosis

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16
Q

Behcet’s disease investigations

A

No specific test - more of a diagnosis of exclusion/clinical signs

Pathergy test (skin prick using a sterile needle then reassessing 48hrs later to see if papule/pustule has formed) can be used but is unreliable

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17
Q

Behcet’s disease management

A

Depends on the pt - some need immunosupression, some only need cover during flare-ups

Medication used = steroids, colchicine, azathioprine, methotrexate, biologics

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18
Q

Gout crystals - appearance and make up

A

Negatively birefringent, needle-shaped crystals - made up of monosodium urate

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19
Q

Pseudogout crystals - appearance and make up

A

Positively birefringent, rhomboid shaped crystals - made up of calcium pyrophosphate dihydrate

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20
Q

What is the double-contour sign?

A

The most sensitive US finding for gout = a hyperechoic irregular band over the superficial margin of the articular cartilage

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21
Q

Define Enteric arthritis

A

An inflammatory arthritis associated w/ IBD - can occur before, during or after the diagnosis of IBD

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22
Q

Patterns of Enteric arthritis

A

Axial spondyloarthritis:
- Gradual onset lower back pain and stiffness, worse in the mornings and improves w/ exercise
- Sacroiliac tenderness reduced ROM and reduced chest expansion on examination

Acute peripheral arthritis of IBD:
- Asymmetric, oligoarticular arthritis predominantly of the LL
- Often transient and migratory join inflammation - episodes are usually self-limiting
- Strongly associated w. flares of IBD

Chronic peripheral arthritis of IBD:
- Chronic, polyarticular inflammation w/ disease independent of IBD activity
- Usually symmetrical and most commonly affects the metacarpophalangeal joints, knees and ankles

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23
Q

Enteric arthritis investigations

A

Bloods:
- Raised inflammatory markers
- RF and ANA -ve
- p-ANCA +ve seen in 55-70%

Radiology:
- Chronic peripheral arthritis = may show erosions of affected joints
- Axial disease = similar to ankylosing spondylitis

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24
Q

Enteric arthritis management

A

DMARDs for peripheral disease

TNF-alpha/IL12/IL23 inhibitors for axial disease

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25
Q

Define Eosinophilic granulomatosis w/ polyangiitis

A

A granulomatous small and medium-vessel vasculitis

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26
Q

Eosinophilic granulomatosis w/ polyangiitis features

A

Criteria:
- Adult onset asthma
- Eosinophilia >10% on peripheral bloods
- Paranasal sinusitis
- Pulmonary infiltrates
- Histological confirmation (small granulomas w/ necrotising vasculitis + eosinophilic infiltrates)
- Mononeuritis multiplex or polyneuropathy

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27
Q

Eosinophilic granulomatosis w/ polyangiitis management

A

Glucocorticoids

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28
Q

Familial Mediterranean fever features

A
  • Recurrent fevers
  • Abdo pain (peritonitis)
  • Pleuritic pain (pleuritis)
  • LL arthritis
  • Orchitis
  • Erysipeloid rash

Symptoms usually last 1-3 days and are slef-limiting

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29
Q

Familial Mediterranean fever investiagations

A

Rule out other causes + genetic testing for MEFV gene

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30
Q

Familial Mediterranean fever management

A

Symptomatic treatment during attacks

Long-term colchicine reduces risk of developing AA amyloidosis

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31
Q

Define Felty syndrome

A

A rare variant of RA, defined as the presence of 3 key features = highly active RA (often w/ extra-articular disease), splenomegaly and neutropenia

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32
Q

Define Fibromyalgia

A

A chronic, complex and widespread pain syndrome

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33
Q

Fibromyalgia features

A
  • Cardinal symptom = chronic, widespread body pain (constant dull ache)
  • Fatigue
  • Cognitive disturbance (problems w/ focus and memory AKA fibro fog)
  • Mood disorders
  • Sleep disturbances (including insomnia)
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34
Q

What USS sign is characteristic of Giant cell arteritis?

A

Halo sign on temporal artery USS

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35
Q

What is the definitive investigation for Giant cell arteritis?

A

Temporal artery biopsy - 3-5 cm to avoid missing skip lesions

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36
Q

Giant cell arteritis management

A

High dose prednisolone (40-60mg) ASAP to avoid blindness and stroke - followed by weaning program

Give bisphosphonates + PPIs due to prolonged steroid regime

Can give low-dose aspirin to decrease risk of stroke and blindness

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37
Q

Types of Gout

A
  • Acute gout
  • Tophaceous gout - usually on a background of longstanding recurrent gout, have tophi on the4 pinnae or on pressure points
  • Nephrolithiasis = uric acid renal stones
  • Uric acid nephropathy
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38
Q

Acute pseudo/gout management

A
  • NSAIDs (500mg BD of Naproxen or 120mg BD of Etoricoxib)
  • Colchicine 500 micrograms BD
  • Intra-articular corticosteroids
  • Oral corticosteroids (Prednisolone 30mg OD for 5-7 days)
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39
Q

Gout prophylaxis

A
  • Lifestyle changes
  • Avoiding triggers
  • Review meds which may cause hyperuricaemia (Thiazide and loop diuretic, low-dose salicylates, chemo)
  • If possible swap anti-HTN drugs to losartan or amlodipine which have urate-lowering effects
  • Allopurinol (start 2 wks after an attack as can cause another attack is started before then) if fit criteria - aim for urate levels <300
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40
Q

Indications for prophylactic allopurinol in gout

A
  • More than 2/3 attacks per year
  • Chronic tophaceous gout
  • X-ray changes show chronic destructive joint disease
  • Urate nephrolithiasis
  • Severe and disabling polyarticular attacks
  • Inherited syndrome s(e.g. Lesch-Nyhan)
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41
Q

Define Granulomatosis w/ polyangiitis

A

A small and medium-vessel systemic vasculitis

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42
Q

Granulomatosis w/ polyangiitis features

A

URT involvement:
- Chronic sinusitis
- Epsitaxis/nasal discharge/mucosal ulcerations
- Saddle-nose deformity - secondary to perforation of the nasal septum and disruption of the supporting cartilage of the nose

LRT involvement:
- Cough
- Haemoptysis
- Pleuritis

p-ANCA/c-ANCA glomerulonephritis:
- Haematuria
- Proteinuria

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43
Q

Granulomatosis w/ polyangiitis investigations

A

Bloods:
- ANCA +ve
- Anaemia
- Leucocytosis
- Raised CRP/ESR

Urine:
- Haematuria
- Proteinuria
- Cellular casts

  • CXR = Bilateral nodules, cavities and infiltrates

Renal biopsy = Pauci-immune, focal, segmental necrotising glomerulonephritis

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44
Q

Granulomatosis w/ polyangiitis management

A
  • High-dose glucocorticoids (1st line)
  • Cyclophosphamide
  • Rituximab
  • Plasma exchange
  • Azathioprine/methotrexate/rituximab for maintenance
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45
Q

Define Henoch-Schonlein Purpura

A

Small-vessel vasculitis commonly occurring in children - often following a resp tract infection

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46
Q

Henoch-Schonlein Purpura features

A
  • Arthralgia/arthritis
  • Abdo pain
  • Palpable purpura on buttocks and extensors
  • Renal disease (microscopic haematuria or an isolated proteinuria)
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47
Q

Henoch-Schonlein Purpura investigations

A

Skin biopsy of lesions - will show presence of neutrophils and monocytes

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48
Q

Henoch-Schonlein Purpura management

A
  • Analgesia
  • Corticosteroids
  • Refer to renal if renal involvement
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49
Q

Henoch-Schonlein Purpura complications

A
  • Pulmonary or GI haemorrhage
  • Renal impairment leading to ESRD
  • Neuro complications e.g. headache and seizures
  • Eye complications e.g. keratitis or uveitis
50
Q

Define Hypermobility syndrome

A

Hypermobility = the ability to move joints beyond the normal range of movement - if this causes the pt to have symptoms secondary to the flexible joints, then it’s a syndrome

51
Q

Hypermobility syndrome features

A
  • Pain or stiffness in the joins or muscles
  • Frequent sprains or dislocations
  • Poor balance
  • Thin, stretchy skin
  • GI upset
52
Q

Hypermobility syndrome investigtions

A

Use the Beighton score to assess joints for hypermobility

53
Q

Hypermobility syndrome management

A

Strengthen muscles to protect the joints, physio involvement

54
Q

Define IgG4-realted disease

A

A chronic multisystem fibroinflammatory disease characterised by a unifying histological features = tumefacient lesions w/ dense lymphocytic infiltrates and storiform fibrosis - thought to be due to the production of IgG4

55
Q

IgG4-realted disease features

A

Pts typically present w/ subacute swelling of the glandular tissue and allergic features:

  • Salivary gland swelling
  • AI Pancreatitis
  • Lymphadenopathy
  • Aortitis
  • Retroperitoneal fibrosis (lower back pain, LL oedema, urethritic obstructions)
56
Q

IgG4-realted disease management

A

Steroids

57
Q

Define Marfan syndrome

A

An A. dominant CNT disorder caused by a missense mutation of the fibrillin 1 gene

58
Q

Marfan syndrome features

A

Physical appearance:
- Tall and thin, with unusually long limbs
- High-arched palate
- Arachnodactyly (tested w/ thumb or wrist sign)

Cardiovascular:
- Thoracic/abdo aortic aneurysms/dissections
- Aortic regurgitation
- Aortic root dilatation
- Mitral valve prolapse/regurgitation
- Spontaneous pneumothoraxes

Ophthalmic:
- Lens dislocations
- Closed-angle glaucoma

MSK:
- Hypermobility
- Contractures
- Pectus deformities
- Kyphoscoliosis
- Protusio acetabuli

Neuro:
-Dural ectasia hernias
-Spontaneous CSF leaks

59
Q

Marfan syndrome diagnostic criteria

A

Use 2010 Ghent criteria

60
Q

Marfan syndrome managemnt

A

Conservative:
- Genetic testing and counselling
- Education around avoiding risky activities
- Surveillance echo’s
- Annual ophthalmological assessment

Medical:
- Strict BP control
- Avoid giving fluoroquinolones due to risk of aortic aneurysms or dissections

Surgical = treat issues that occur

61
Q

Define Microscopic polyangiitis

A

One of the more common forms of arteritis affecting smaller arteries - due to p-ANCA directed against myeloperoxidase

62
Q

Microscopic polyangiitis features

A
  • Constitutional symptoms = WL, fevers, malaise, arthralgia, myalgia
  • Necrotising glomerulonephritis
  • Skin lesions =palpable purpura of the LL
  • Mononeuritis multiplex
  • Lung involvement = pulmonary capillaritis leading to alveolar haemorrhage
63
Q

Microscopic polyangiitis management

A

High dose glucocorticoids + cyclophosphamide or rituximab

64
Q

Define Polyarteritis nodosa

A

A segmental transmural necrotising vasculitis of medium-sized muscular arteries - associated w/ Hep B

65
Q

Polyarteritis nodosa features

A
  • Mononeuritis multiplex
  • Renal infarcts and failure
  • Skin lesions = livedo reticularis, arthritis, myalgias
  • MSK = arthralgias, arthritis, myalgias
  • GI = abdo pain, abnormal LFTs
  • MI/HF
  • Stroke
  • Seizures

Pulmonary arteries are NOT affected

66
Q

Polyarteritis nodosa management

A

Oral corticosteroids for moderate, IV pulsed high-dose corticosteroids for severe, immunotherapy (azathioprine, methotrexate, cyclophosphamide, rituximab)

67
Q

Polyarteritis nodosa complications

A

Can destroy the arterial media and internal elastic lamina - leads to aneurysm formation

68
Q

Polymyalgia Rheumatica features

A
  • Shoulder/hip girdle stiff (in the mornings) for > 1 hr, w/ associated inflammatory pain
  • Low-grade fever
  • Anorexia
  • WL
  • Malaise
  • Associated w/ GCA
  • No muscle weakness - if there is weakness then more likely to be dermato/myositis
69
Q

Polymyalgia Rheumatica management

A

Low-dose corticosteroids

70
Q

Subtypes of inflammatory myopathies

A
  • Polymyositis
  • Dermatomyositis
  • Amyotrophic dermatomyositis
  • Antisynethetase syndrome (polymyositis w/ antisynthetase abs)
  • Inclusion-body myositis
71
Q

Polymyositis features

A
  • Main feature = bilateral, proximal muscle (hip and shoulder) weakness developing over weeks to months
  • Myalgia and tenderness in 1/3 of pts
  • Muscle bulk reflexes are preserved until very late
  • As disease progresses pt get: pharyngeal or oesophageal involvement, resp muscle involvement
72
Q

Dermatomyositis features

A

Symptoms of polymyositis + skin rashes:
- Heliotrophic rash = lilac discolouration of the eyelids w/ periorbital oedema
- Gottron’s papules = scaly, erythematous papules over knuckles and extensors
- Macular erythematous rash on anterior chest and neck (V-sign) or upper arms and shoulders (shawl sign)
- Nailfold abnormalities
- Cutaneos vasculitis
- Calcinosis

73
Q

Amyotrophic dermatomyositis features

A

Characteristic cutaneous symptoms of dermatomyositis (e.g. Heliotrophic rash, Gottron’s papule) w/out any muscle involvement

74
Q

Antisynthetase syndrome features

A
  • Cracking and fissuring of the skin on the finger pads
  • Interstitial lung disease
  • Raynaud’s
  • Arthralgia/arthritis
  • Systemic features
  • Dermatomyositis-like rash
  • Polymyositis
75
Q

Inclusion-body myositis features

A

Bilateral, asymmetrical painless weakness of the distal muscles +/- dysphagia

Slowly progressive and poorly responsive to immunosuppression

76
Q

Dermato/myositis investigations

A
  • Raised creatine kinase + other enzymes
  • Muscle biopsy is definitive test - polymyositis shows endomysial inflammatory infiltrates, dermatomyositis shows perivascular, perimysial inflammatory infiltrates

Elevated enzymes can be remebered with:
‘Dermato/mysotis turn your muscles into CLAAA’
- Creatine kinase
- Lactate dehydrogenase
- Aldolase
- ALT
- AST

77
Q

Dermato/myositis management

A

Corticosteroids, immunosuppression (methotrexate, mycophenolate), hydroxychloroquine can help w/ skin disease, rehabilitation

78
Q

Psoriatic arthritis management

A

Conventional synthetic DMARDs (csDMARDs):
- Methotrexate
- Leflunomide

Biological DMARDs (bDMARDs):
- If pt fails to respond to csDMARDs
- Anti-TNF agents
- Anti-IL12/23
- Anti-IL17
- CTLA4 inhibitors

Tofacitinib (Janus kinase inhibitor) if those don’t work

79
Q

Causes of Raynaud’s phenomenon

A

Primary = idiopathic

Secondary:
-SLE
- Scleroderma
- Buergers disease
- Sjogrens syndrome
- RA
- Oclusive vascular disease e.g. atheroscerlosis
- Polymyositis
- Coagulopathies
- Thyroid disorders
- Pulmonary HTN

80
Q

Raynaud’s phenomenon management

A

Conservative:
- Smoking cessation
- Warm gloves
- Avoiding cold exposure

Medical:
- Dihydropyridine CCB (e.g. nifidepine) = 1st line
- Phsophodiesterase inhibitors and IV prostacyclins = 2nd line

Surgical (in severe cases):
- Nerve blocks
- Digitil amputation

81
Q

Define Reactive arthritis

A

A sterile inflammatory arthritis occuring w/in 4 weeks of an infection - most commonly chlamydia, shigella, yersinia or salmonella

82
Q

Reactive arthritis features

A
  • Asymmetrical oligoarthritis most commonly affecting he large joints of the lower limbs
  • Constiutional symptoms
  • Keratoderma blenorrhagica = dark maculopapular rash on palmes and soles
  • Circinate balantitis = erosion of the glands penis
  • Conjuctivitis/anterior uevitis
  • Dactylitis
  • Urethritis and sterile dysuria

Cant see, cant pee, cant climb tree

83
Q

Reactive arthritis management

A

NSAIDs, DMARDs

84
Q

Rheumatoid arthritis wider systemic features

A
  • Haem = anaemia of chronic disease, AA amyloidosis
  • Derm = rheumatoid nodules (firm, dark nodules around inflammation), small vessel vasculitis, pyoderma gangrenosum
  • Ophthalmic = keratoconjunctivitis, Epi/scleritis
  • Resp = pleural disease, rheumatoid nodules, ILD, methotrexate induced pneumonitis
  • Cardiac = pericarditis, pericardial effusion, CVD
  • Neuro = peripheral neuropathy (secondary to small-vessel vasculitis), mononeuritis multiplex (secondary to medium-vessel vasculitis)
85
Q

Antibodies in Rheumatoid arthritis

A

Anti-CCP, RF

86
Q

Rheumatoid arthritis management

A

Conservative:
- Physio
- Occupational therapy

Medical:
- Simple analgesia
- Oral or intra-articular corticosteroids
- DMARDs (1st line) = methotrexate, hydroxychloroquine, leflunomide, sulfasalazine
- Biologics (in addition if DMARD monotherapy doesn’t work) = Anti-TNF (infliximab), CTLA4 inhibitors, andi-CD20 (rituximab), anti-Il6 (tocilizumab)

87
Q

Causative agents of Septic arthritis

A
  • Staph aureus = 50% of cases
  • Streptococci = 2nd most common (haematogenous spread)
  • N. gonorrhoeae
  • Pseudomonas = healthcare associated or IVDU
  • Salmonella = if SCA
  • Gram-ve’s

Special cases = TB, Lyme disease, fungi

88
Q

Septic arthritis investigations

A
  • Synovial fluid aspiration = gram stain, culture and microscopy
  • 2x blood cultures

All done before giving Abx

89
Q

Septic arthritis management

A

Empirical abx and then change based on culture

Pt should be treated for 2 wks w/ IV abx, and then orally for 4 more wks

If the joint is prosthetic = aggressive debridement and prolonged abx

90
Q

Side effects of corticosteroids

A

C = Cushing’s syndrome
O = osteoporosis
R = retardation of growth
T = thin skin, easy bruising
I = immunosupression
C = cataracts and glaucoma
O = oedema
S = suppression of HPA axis
T = teratogenic
E = emotional disturbance (including psychosis)
R = rise in BP
O = obesity (truncal)
I = increased hair growth
D = DM
S = striae

91
Q

Side effects of methotrexate

A
  • GI disturbance
  • Folate deficient anaemia
  • Immunosuppression
  • Pulmonary fibrosis
  • Liver toxicity
  • Interstitial pneumonitis
  • Rash
  • Teratogenic
92
Q

Side effects of sulfasalazine

A
  • Myelosuppression
  • Nausea
  • Rash
  • Oral ulcers
  • Decreased sperm count
93
Q

Side effects of hydroxychloroquine

A
  • Retinopathy
  • Rash
94
Q

Sjogren’s syndrome features

A

Ocular:
- Keratoconjunctivitis sicca = reduced tear secretion
- Causes dry, gritty eyes

Oral:
- Dry mouth
- Intermittent parotid gland swelling

Other exocrine glands:
- Vaginal dryness
- Reduces GI secretions = dysphagia, oesophagitis, gastritis

Extra glandular features:
- Systemic symptoms
- Arthritis = episodic, non-erosive
- Raynaud’s
- Cutaneous vasculitis

95
Q

Causes of secondary Sjogren’s syndrome

A
  • SLE
  • RA
  • Systemic sclerosis
  • Poly/dermatomyositis
  • AI thyroiditis
  • PBC
  • AI hepatitis
96
Q

Sjogren’s syndrome investigations

A

Bloods:
- raised ESR/CRP
- RF +ve in 90%
- Anti-Ro and anti-La in 40-90%

Special tests:
- Schirmer’s test = filter paper on lower eyelid, with wetting of <5mm being +ve
- Rose Bengal staining of the cornea = demonstrates keratitis when using a split lamp
- Salivary flow rate monitoring w/ radiolabelled dye
- Salivary gland biopsy to confirm diagnosis

97
Q

Sjogren’s syndrome management

A

Symptomatic control

No effective disease-modify therapies

98
Q

SLE subtypes

A

SLE = multiple organ systems - usually +ve for ANA, anti-dsDNA

Subacute cutaneous lupus = skin only 0 ANA +ve or -ve, anti-SS-A and anti-SS-B +ve

Drug-induced lupus = systemic, usually sparing renal and neuro systems - anti-histones

Discoid lupus = skin only - usually ANA -ve

99
Q

Dermatological manifestations of SLE

A
  • Photosensitivity - usually a malar rash
  • Discoid rash
  • Recurrent mouth ulcers
  • Raynaud’s phenomenon
  • Non-scaring alopecia
  • Cutaneous vasculitis - manifesting as splinter haemorrhages/purpura
100
Q

MSK manifestations of SLE

A
  • Non-reosive arthritis
  • Flitting arthralgia w/ early morning stiffness
  • Jaccoud’s arthropathy = tendon involvement leading to an RA-like deformity
101
Q

Renal manifestations of SLE

A

Lupus nephritis (most common cause of SLE-related mortality) - starts as asymptomatic before presenting w/ either nephrotic or nephritic syndrome

102
Q

Classification of Lupus nephritis

A

Minimal mesangial (class I) = mesangial immune deposits - no clinical manifestations

Mesangial proliferative (class II) = mesangial hypercellularity or matrix expansion, few subepithelial immune complexes - microscopic haematuria +/- proteinuria

Focal (class III) = <50% of glomeruli affected w/ hypercellularity and subendothelial/epithelial immune complex deposition - haematuria, proteinuria, reduced eGFR, HTN and nephrotic syndrome

Diffuse (class IV) = >50% of glomeruli affected, same histo as above - haematuria, proteinuria, urine cellular casts, HTN. reduced eGFR, reduced C£/r, increased anti-dsDNA

Membranous (class V) = Global or segmental subepithelial immune deposits - nephrotic syndrome, expansive proteinuria w. minimal haematuria

Advanced sclerosing (class VI) = >90% glomerulosclerosis - CKD

103
Q

Cardiovascular manifestations of SLE

A
  • Pericarditis (most common)
  • Myocarditis (rare)
  • Increased CV risk factor
  • Libman-Sacks (non-infective) endocarditis (very rare)
104
Q

Respiratory manifestations of SLE

A
  • Pleurisy (most common)
  • Pleural effusions
  • Pulmonary HTN (rare)
105
Q

Neurological manifestations of SLE

A
  • Cognitive impairment
  • Seizures
  • Migraines
  • Peripheral neuropathy
  • Cranial neuropathy
  • Psychiatric symptoms
  • Movement disorders
  • Headaches
106
Q

Haematological manifestations of SLE

A
  • Anaemia of chronic disease (common)
  • Lymphopenia (common)
  • AI haemolytic anaemia
  • Thrombocytopenia
  • Leukopenia
  • Reactive lymphadenopathy and splenomegaly (esp in active disease)
  • Antiphospholipid syndrome
107
Q

GI manifestations of SLE

A
  • Aseptic peritonitis
  • Hepatosplenomegaly
108
Q

What ANA titre is considered significant for SLE?

A

1:160

109
Q

SLE management

A
  • 1st line = NSAIDs and hydroxychloroquine
  • Add long-term corticosteroids + DMARD if not working
  • Belimumab (inhibits B cell stimulation ) = biologic

DMARDs used = hydroxychloroquine, methotrexate, leflunomide, azathioprine, mycophenolate

110
Q

Which drugs can induce Lupus?

A
  • Sulfadiazine
  • Hydralazine
  • Procainamide
  • Isoniazid
  • Methyldopa
  • Quinidine
  • Minocycline
  • Chlorpromazine
111
Q

Limited cutaneous systemic sclerosis features

A

CREST syndrome
- Calcinosis
- Raynaud’s (typically occurs for years before onset of symptoms)
- Oesophageal dysmotility (dysphagia, GORD)
- Sclerodactyly
- Telangiectasia

Skin fibrosis is limited to the hands and forearms, feet and legs, and head a neck

112
Q

Diffuse cutaneous systemic sclerosis features

A
  • CREST features not limited across whole body
  • Abnormalities w/ blood vessels
  • Fibrosis of internal organs (pulmonary fibrosis, renal crisis, myocardial disease, bowel hypomotility, pericarditis w/ effusion)
  • Digital ulcers
  • Tendon friction rubs and contractures

Pts may present with acute onset hands/feet swelling + Raynaud’s

113
Q

Cutaneous systemic sclerosis (both types) management

A

Symptomatic management - DMARDs (mycophenolate, methotrexate, cyclophosphamide) can be used to treat skin thickening, but with limited evidence

114
Q

Define Takayasu’s arteritis

A

A chronic granulomatous vasculitis affecting large arteries, particularly the aorta and its main branches, this can cause stenosis, occlusions and aneurysms - most common in Asian women aged 10-40

115
Q

Takayasu’s arteritis features

A

Depends on the vessel involved:
- Subclavian artery = upper limb claudication’s
- Carotid and vertebral artery = headaches and presyncope
- Pulmonary artery = pulmonary HTN
- Cardiac = angina, HF and valvular pathologies

Systemic symptoms

Vascular exam shows:
- Abnormalities in peripheral pulses
- Bruits over the central pulses
- Asymmetric BP
- Aortic regurgitation (due to root dilatation)

116
Q

Takayasu’s arteritis investigations

A

Imaging is requred for diagnosis:
- Angiography
MR/CT angiography and FDG-PET are the modalities of choice

117
Q

Takayasu’s arteritis management

A

High dose corticosteroids + bone/GI protection

118
Q

Define Trochanteric bursitis

A

Inflammation of the bursa situated over the greater trochanter

119
Q

Trochanteric bursitis features

A
  • Lateral hip pain, aggravated by physical activity and more severe at night
  • Swelling + tenderness in affected area
  • +ve Trendelenburg test
120
Q

Trochanteric bursitis management

A
  • Physio
  • Analgesia
  • Corticosteroid injections
  • Bursectomy in severe or refractory cases

Year 6 (8 decks)

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