Basics Flashcards

1
Q

ANCA neutrophil cytoplasmic antibodies - small vessel vasculitidies

A

granulomatosis with polyangiitis
eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
microscopic polyangiitis

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2
Q

Common findings of ANCA

A
renal impairment
caused by immune complex glomerulonephritis → raised creatinine, haematuria and proteinuria
respiratory symptoms
dyspnoea
haemoptysis
systemic symptoms
fatigue
weight loss
fever
vasculitic rash: present only in a minority of patients
ear, nose and throat symptoms
sinusitis
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3
Q

General approach to first-line investigations for pANCA testing

A

urinalysis for haematuria and proteinuria
bloods:
urea and creatinine for renal impairment
full blood count: normocytic anaemia and thrombocytosis may be seen
CRP: raised
ANCA testing (see below)
chest x-ray: nodular, fibrotic or infiltrative lesions may be seen

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4
Q

cANCA

A

granulomatosis with polyangitis

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5
Q

pANCA

A

eosinophilic granulomatosis with polyangiitis+ others

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6
Q

Mainstay for ANCA associated vasculitis

A

Immunosuppressive therapy

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7
Q

Features of ank-spon

A

HLA-B27 associated spondyloarthropathy - sex ration (3:1)
Typically a young man who presents with lower back pain and stiffness of insidious onset
stiffness is usually worse in the morning and improves with exercise
the patient may experience pain at night which improves on getting up

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8
Q

Clinical examination for ank-spon

A

reduced lateral flexion
reduced forward flexion - Schober’s test - a line is drawn 10 cm above and 5 cm below the back dimples (dimples of Venus). The distance between the two lines should increase by more than 5 cm when the patient bends as far forward as possible
reduced chest expansion

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9
Q

Other features the A’s

A
Apical fibrosis
Anterior uveitis
Aortic regurgitation
Achilles tendonitis
AV node block
Amyloidosis
and cauda equina syndrome
peripheral arthritis (25%, more common if female)
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10
Q

Later radiograph changes in ank-spon

A

Radiographs may be normal early in disease, later changes include:
sacroiliitis: subchondral erosions, sclerosis
squaring of lumbar vertebrae
‘bamboo spine’ (late & uncommon)
syndesmophytes: due to ossification of outer fibers of annulus fibrosus
chest x-ray: apical fibrosis

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11
Q

Management of ank-spon

A

encourage regular exercise such as swimming
NSAIDs are the first-line treatment
physiotherapy
the disease-modifying drugs which are used to treat rheumatoid arthritis (such as sulphasalazine) are only really useful if there is peripheral joint involvement
the 2010 EULAR guidelines suggest: ‘Anti-TNF therapy should be given to patients with persistently high disease activity despite conventional treatments’
research is ongoing to see whether anti-TNF therapies such as etanercept and adalimumab should be used earlier in the course of the disease

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12
Q

Antiphospholipid syndrome features

A
venous/arterial thrombosis
recurrent fetal loss
livedo reticularis
thrombocytopenia
prolonged APTT
other features: pre-eclampsia, pulmonary hypertension
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13
Q

Antiphospholipid happens secondary to which disease

A

SLE

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14
Q

Associations other than SLE in anti-phospholipid syndrome

A

other autoimmune disorders
lymphoproliferative disorders
phenothiazines (rare)

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15
Q

Antisythetase syndrome features

A

myositis
interstitial lung disease
thickened and cracked skin of the hands (mechanic’s hands)
Raynaud’s phenomenon

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16
Q

Adverse effects of azathioprine

A

bone marrow depression
nausea/vomiting
pancreatitis
increased risk of non-melanoma skin cancer

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17
Q

Behcet’s syndrome features

A

classically: 1) oral ulcers 2) genital ulcers 3) anterior uveitis
thrombophlebitis and deep vein thrombosis
arthritis
neurological involvement (e.g. aseptic meningitis)
GI: abdo pain, diarrhoea, colitis
erythema nodosum

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18
Q

Diagnosis of Behcet’s

A

no definitive test
diagnosis based on clinical findings
positive pathergy test is suggestive (puncture site following needle prick becomes inflamed with small pustule forming)

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19
Q

What are bisphosponates

A

Analogues of pyrophosphate, molecule which decreases demineralisation in bone.
Inhibit osteoclasts by reducing recruitment and promoting apoptosis

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20
Q

Adverse effects of bisphosphonates

A

oesophageal reactions: oesophagitis, oesophageal ulcers (especially alendronate)
osteonecrosis of the jaw
increased risk of atypical stress fractures of the proximal femoral shaft in patients taking alendronate
acute phase response: fever, myalgia and arthralgia may occur following administration
hypocalcaemia: due to reduced calcium efflux from bone. Usually clinically unimportant

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21
Q

What should be corrected before giving bisphosphonates

A

Hypocalcaemia/ vitamin D deficiency

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22
Q

Benign bone tumours

A

Osteoma
Osteochondroma- exotosis
Giant cell tumour

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23
Q

Malignant tumours

A

Osteosarcoma
Ewing’s sarcoma
Chondrosarcoma

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24
Q

Osteoma features

A

benign ‘overgrowth’ of bone, most typically occuring on the skull
associated with Gardner’s syndrome (a variant of familial adenomatous polyposis, FAP)

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25
Q

Osteochondroma

features

A

most common benign bone tumour
more in males, usually diagnosed in patients aged < 20 years
cartilage-capped bony projection on the external surface of a bone

26
Q

Giant cell tumour features

A

tumour of multinucleated giant cells within a fibrous stroma
peak incidence: 20-40 years
occurs most frequently in the epiphyses of long bones
X-ray shows a ‘double bubble’ or ‘soap bubble’ appearance

27
Q

Osteosarcoma features

A

Most common malignant brain tumour
In children and adolescents
Femur, tibia and humerus affected
X-ray shows codman’s triangle from periosteal elevation and sunburst pattern

28
Q

Ewing’s sarcoms features

A

small round blue cell tumour
seen mainly in children and adolescents
occurs most frequently in the pelvis and long bones. Tends to cause severe pain
associated with t(11;22) translocation which results in an EWS-FLI1 gene product
x-ray shows ‘onion skin’ appearance

29
Q

Chondrosarcoma features

A

malignant tumour of cartilage
most commonly affects the axial skeleton
more common in middle-age

30
Q

What is Codman’s triangle?

A

Triangular area of a new subperiosteal bone- created when a lesion, often a tumour, raises the periosteum away from the bone

31
Q

Denosumab- what is it and how does it work?

A

Human monoclonal antibody- prevents the development of osteoclasts by inhibiting RANKL

Given SC
Usually for treatment of osteoporosis- NOT FIRST LINE
Given in adults with bone mets for solid tumours

32
Q

Treatment for osteoporosis

A

Oral bisphosphonates given first line- oral ALENDRONATE, then risedronate or etidronate if alendronate not tolerated.
Then dependent on T-score
Raloxifene and strontium ranelate next line

33
Q

What is dermatomyositis?

A

Inflammatory disorder causing symmetrical proximal muscle weakness and characteristic skin lesions
may be idiopathic or associated with connective tissue disorders or underlying malignancy
- screen patients for malignancy

34
Q

Skin features of polymyositis

A

eatures
photosensitive
macular rash over back and shoulder
heliotrope rash in the periorbital region
Gottron’s papules - roughened red papules over extensor surfaces of fingers
‘mechanic’s hands’: extremely dry and scaly hands with linear ‘cracks’ on the palmar and lateral aspects of the fingers
nail fold capillary dilatation

35
Q

Other features

A
proximal muscle weakness +/- tenderness
Raynaud's
respiratory muscle weakness
interstitial lung disease: e.g. Fibrosing alveolitis or organising pneumonia
dysphagia, dysphonia
36
Q

Investigations of Dermatomyositis

A

the majority of patients (around 80%) are ANA positive
around 30% of patients have antibodies to aminoacyl-tRNA synthetases (anti-synthetase antibodies), including:
antibodies against histidine-tRNA ligase (also called Jo-1)
antibodies to signal recognition particle (SRP)
anti-Mi-2 antibodies

37
Q

What is transient idiopathic osteoporosis

A

An uncommon condition sometimes seen in the third trimester of pregnancy
Groin pain associated with a limited range of movement in the hip
Patients may be unable to weight bear
ESR may be elevated

38
Q

What is pubic symphysis dysfunction

A

Common in pregnancy
Ligament laxity increases in response to hormonal changes of pregnancy
Pain over the pubic symphysis with radiation to the groins and the medial aspects of the thighs. A waddling gait may be seen

39
Q

What is great trochanteric pain syndrome

Trochanteric bursitis

A

Due to repeated movement of the fibroelastic iliotibial band
Pain and tenderness over the lateral side of thigh
Most common in women aged 50-70 years

40
Q

Myopathies features

A

symmetrical muscle weakness (proximal > distal)
common problems are rising from chair or getting out of bath
sensation normal, reflexes normal, no fasciculation

41
Q

Causes of myopathies

A

inflammatory: polymyositis
inherited: Duchenne/Becker muscular dystrophy, myotonic dystrophy
endocrine: Cushing’s, thyrotoxicosis
alcohol

42
Q

Features of Polymyositis

A
proximal muscle weakness +/- tenderness
Raynaud's
respiratory muscle weakness
interstitial lung disease: e.g. fibrosing alveolitis or organising pneumonia
dysphagia, dysphonia
43
Q

Investigations for Polymyositis

A

elevated creatine kinase
other muscle enzymes (lactate dehydrogenase (LD), aldolase, AST and ALT) are also elevated in 85-95% of patients
EMG
muscle biopsy
anti-synthetase antibodies
anti-Jo-1 antibodies are seen in pattern of disease associated with lung involvement, Raynaud’s and fever

44
Q

Which test helps to differentiate between polymyalgia rheumatica and statin-induced cardiomyopathy

A

ESR

45
Q

Which antibody is associated with dermatomyositis

A

ANA

46
Q

When after does reactive arthritis occurs

A

After an infection where organism cannot be recivered from the joint

47
Q

Septic joint colour on aspirate

A

Turbid-greu

48
Q

Which type of hypersensitivity reaction is SLE

A

Type 3

49
Q

The A’s of ankylosing spondylitis

A
Apical fibrosis
Anterior uveitis
Aortic regurgitation
Achilles tendonitis
AV node block
Amyloidosis
50
Q

Which condition does temporal arteritis overlap with

A

Polymyalgia rheumatica

51
Q

Temporal arteritis features

A

typically patient > 60 years old
usually rapid onset (e.g. < 1 month)
headache (found in 85%)
jaw claudication (65%)
visual disturbances
vision testing is a key investigation in patients with suspected temporal arteritis
secondary to anterior ischemic optic neuropathy
tender, palpable temporal artery
around 50% have features of PMR: aching, morning stiffness in proximal limb muscles (not weakness)
also lethargy, depression, low-grade fever, anorexia, night sweats

52
Q

Dexa scan T score meaning

A

> -1.0 = normal
-1.0 to -2.5 = osteopaenia
< -2.5 = osteoporosis

53
Q

Antisynthetase syndrome features

A

myositis
interstitial lung disease
thickened and cracked skin of the hands (mechanic’s hands)
Raynaud’s phenomenon

54
Q

Antisynthetase syndrome - caused by autoantibodies against whhat?

A

anti-Jo-1

55
Q

First line management for ank-spon

A

physiotherapy and NSAIDs

56
Q

What is antisynthetase syndrome a subtype fo

A

Dermatomyositis

57
Q

Joint aspirate appearance in rheumatoid arthritis

A

shows a high WBC count, predominantly PMNs. Appearance is typically yellow and cloudy with absence of crystals

58
Q

Adjusted calcium, phosphate, parathyroid hormone and ALP results in Osteogenesis imperfecta

A

NORMAL

59
Q

Still’s disease in adults features

A
Arthralgia
elevated serum ferritin 
Salmon-pink, maculopapular rash
Pyrexia
Lymphadenopathy 
RF and ANA negative
60
Q

CREST syndrome features

A

Calcinosis (white deposits)
Raynaud’s (cold, white fingertips precipitated by cold weather)
oEsophogeal dysmotility (dysphagia)
Sclerodactyly (thickened skin on top of hands and inability to straighten fingers)
Telangiectasia (excessive number of spider naevi)

61
Q

Antibody for diffuse systemic sclerosis

A

anti-sc-70

62
Q

antibody for limited cutaneous sclerosis

A

anti-centromere