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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
Osteochondroma | features
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
Giant cell tumour features
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
Osteosarcoma features
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
Ewing's sarcoms features
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
Chondrosarcoma features
malignant tumour of cartilage most commonly affects the axial skeleton more common in middle-age
30
What is Codman's triangle?
Triangular area of a new subperiosteal bone- created when a lesion, often a tumour, raises the periosteum away from the bone
31
Denosumab- what is it and how does it work?
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
Treatment for osteoporosis
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
What is dermatomyositis?
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
Skin features of polymyositis
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
Other features
``` proximal muscle weakness +/- tenderness Raynaud's respiratory muscle weakness interstitial lung disease: e.g. Fibrosing alveolitis or organising pneumonia dysphagia, dysphonia ```
36
Investigations of Dermatomyositis
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
What is transient idiopathic osteoporosis
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
What is pubic symphysis dysfunction
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
What is great trochanteric pain syndrome Trochanteric bursitis
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
Myopathies features
symmetrical muscle weakness (proximal > distal) common problems are rising from chair or getting out of bath sensation normal, reflexes normal, no fasciculation
41
Causes of myopathies
inflammatory: polymyositis inherited: Duchenne/Becker muscular dystrophy, myotonic dystrophy endocrine: Cushing's, thyrotoxicosis alcohol
42
Features of Polymyositis
``` proximal muscle weakness +/- tenderness Raynaud's respiratory muscle weakness interstitial lung disease: e.g. fibrosing alveolitis or organising pneumonia dysphagia, dysphonia ```
43
Investigations for Polymyositis
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
Which test helps to differentiate between polymyalgia rheumatica and statin-induced cardiomyopathy
ESR
45
Which antibody is associated with dermatomyositis
ANA
46
When after does reactive arthritis occurs
After an infection where organism cannot be recivered from the joint
47
Septic joint colour on aspirate
Turbid-greu
48
Which type of hypersensitivity reaction is SLE
Type 3
49
The A's of ankylosing spondylitis
``` Apical fibrosis Anterior uveitis Aortic regurgitation Achilles tendonitis AV node block Amyloidosis ```
50
Which condition does temporal arteritis overlap with
Polymyalgia rheumatica
51
Temporal arteritis features
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
Dexa scan T score meaning
> -1.0 = normal -1.0 to -2.5 = osteopaenia < -2.5 = osteoporosis
53
Antisynthetase syndrome features
myositis interstitial lung disease thickened and cracked skin of the hands (mechanic's hands) Raynaud's phenomenon
54
Antisynthetase syndrome - caused by autoantibodies against whhat?
anti-Jo-1
55
First line management for ank-spon
physiotherapy and NSAIDs
56
What is antisynthetase syndrome a subtype fo
Dermatomyositis
57
Joint aspirate appearance in rheumatoid arthritis
shows a high WBC count, predominantly PMNs. Appearance is typically yellow and cloudy with absence of crystals
58
Adjusted calcium, phosphate, parathyroid hormone and ALP results in Osteogenesis imperfecta
NORMAL
59
Still's disease in adults features
``` Arthralgia elevated serum ferritin Salmon-pink, maculopapular rash Pyrexia Lymphadenopathy RF and ANA negative ```
60
CREST syndrome features
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
Antibody for diffuse systemic sclerosis
anti-sc-70
62
antibody for limited cutaneous sclerosis
anti-centromere

Rheumatology (11 decks)

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