Rheumatology Flashcards
1
Q
cytoplasmic inclusions on muscle biopsy1st line treatment of osteoporosis?
A
alendronate
2
Q
2nd line treatment of osteoporosis if unable to tolerate alendronate due to upper GI side effects?
A
risedronate/ etidronate
3
Q
3rd line treatment of osteoporosis if cannot take bisphosphonates?
A
Strontium Ralenate (dual action: promotes differentiation from pre-osteoblast to osteoblast + inhibits osteoclasts) or Raloxifene (Selective oestrogen receptor modulator)
4
Q
what e.g. is a once only oral bisphosphonate?
A
ibandronate
5
Q
denosumab?
A
human monoclonal antibody: anti-RANK ligand -> inhibits maturation of osteoclasts
6
Q
what protein is defective in Marfans?
A
fibrillin-1
autosomal dominant- defect in FBN1 gene on chr 15
7
Q
oral + genital ulcers + anterior uveitis + thrombophlebitis?
A
Behcet’s syndrome
8
Q
classic triad of Behcet’s syndrome?
A
oral ulcers + genital ulcers + anterior uveitis
9
Q
Features of Behcets?
A
- oral + genital ulcers - anterior uveitis - thrombophlebitis +/- DVT - arthritis - neuro involvement e.g. aseptic meningitis - GI: abdo pain, diarrhoea - erythema nodosum *presumed autoimmune-mediated inflammation of arteries and veins
10
Q
What HLA is assoc w Behcets?
A
HLA-B51 (a split antigen of HLA B5)
11
Q
Diagnosis of Behcets?
A
clinical: no definitive test - positive pathergy test is suggestive (ie. puncture site following needle prick becomes inflamed with small pustule forming)
12
Q
what joints are most commonly implicated in osteoarthritis?
A
carpometacarpal + DIP joints
13
Q
What are Heberden’s and Bouchard’s nodes?
A
Heberden: DIPJ Bouchard: PIPJ result of osteophyte formation.
14
Q
features of osteoarthritis?
A
- usually bilateral - provoked by movement, relieved by resting - stiffness: Worse after long periods of inactivity (only lasts few mins) - Heberdens + Bouchards - squaring of the thumbs
15
Q
What T score suggests osteoporosis? vs osteopenia?
A
> -1.0 = normal
-1.0 to -2.5 = osteopaenia
< -2.5 = osteoporosis
16
Q
most useful investigation in diagnosis of Ankylosing spondylitis?
A
Plain x-ray of the sacroiliac joints
17
Q
features of ankylosing spondylitis on X-ray?
A
- 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
18
Q
Ix of choice if X-ray is negative for Ank spondylitis but clinical suspicion remains high?
A
MRI: Signs of early inflammation involving sacroiliac joints (bone marrow oedema) confirm the diagnosis
19
Q
1st line mx of Ank Spond?
A
NSAIDs + exercise / physio
20
Q
What management should be considered in persistently high disease activity in Ank Spond despite conventional treatment?
A
Anti-TNF therapies e.g. etanercept / adalimumab
21
Q
mix of SLE + Systemic sclerosis + myositis?
A
mixed connective tissue disease ie. Sharp’s syndrome
22
Q
what antibody is most sensitive for mixed connective tissue disease?
A
anti-U1 RNP antibodies RNP = ribonucleoprotein
23
Q
most common features of mixed connective tissue disease?
A
- Raynaud’s often precedes (occurs in 90%) - polyarthralgia/ arthritis - myalgia - dactylitis - other dermatological (e.g. photosensitive rash), oesophageal dysfunction, respiratory, haematological, cardiac, renal, neuropsych features
24
Q
osteogenesis imperfecta - abnormality in what?
A
type 1 collagen - which is main component of the organic part of bone + skin & tendons
25
Disorder in type 5 collagen?
Ehlers-Danlos syndrome - Type 5 collagen found in cell surfaces, hair, placentas
26
Type 4 collagen mutations cause?
Alpert's syndrome, Goodpasture's \*defect in basal lamina
27
Defect in type 2 collagen?
Chondrodysplasias - type 2 collagen is main component of cartilage
28
Defect in type 3 collagen?
can lead to a type of Ehlers-Danlos \*not the classic type Type 3 collagen is main component of reticular fibres
29
features of osteogenesis imperfecta?
- fractures following minor trauma - blue sclera - deafness 2dry to osteosclerosis - dental imperfections
30
Ca/ Phosphate/ PTH/ Alk Phos results in osteogenesis imperfecta?
usually all normal
31
common causes of drug-induced lupus?
Most common: procainamide hydralazine
less common: isoniazid minocycline phenytoin
32
most sensitive antibodies for drug induced lupus?
anti-histone abs (in 80-90%) \*ANA+ (100%), dsDNA -ve, anti Ro/ Smith +ve in 5%
33
xray changes in rheumatoid arthritis?
Early x-ray findings: - loss of joint space - juxta-articular osteoporosis - soft-tissue swelling Late x-ray findings: - periarticular erosions - subluxation
34
most specific antibodies for dermatomyositis?
anti-Mi-2 antibodies
35
most specific antibodies for polymyositis?
anti-Jo1 antibodies
36
Management of dermatomyositis?
prednisolone
37
anti-La antibodies?
Sjogrens
38
Anti-Ro antibodies?
Sjogrens, SLE, Congenital Heart block
39
SEs of bisphosphonates?
oesophageal reactions: ulcer/ oesophagitis osteonecrosis of the jaw increased risk atypical stress #s of proximal femoral shaft acute phase response: fever, myalgia, arthralgia following administration hypoCa: reduced ca efflux from bone, usually clincally unimportant
40
management of vit D deficiency?
Oral Vit D loading then maintenance
41
features of Antiphospholipid syndrome?
recurring fetal loss, venous/ arterial thrombosis
- livedo reticularis
- thrombocytopenia
- prolonged APTT
- pulmonary HTN, pre-eclampsia
42
primary thromboprophylaxis in antiphospholipid syndrome?
low dose aspirin
43
secondary thromboprophylaxis in antiphospholipid syndrome?
lifelong warfarin w target INR 2-3 to start
44
what type of hypersensitivity reaction is SLE?
type 3 immune complex dysregulation -\> immune complex formation -\> deposition can affect any organ (skin, joints, kidney, brain)
45
SLE assoc w which HLA?
HLA B8, DR2, DR3
46
SE of raloxifene?
increased risk of VTE may worsen menopausal symptoms
47
raloxifene and risk of breast ca?
decreases risk
48
causes of decreased excretion of uric acid? thus predisposing to gout
drugs: diuretics CKD lead toxicity Lesch-Nyhan syndrome
49
Gout: causes of increased production of uric acid?
myeloproliferative/ lymphoproliferative disorders cytotoxics severe psoriasis
50
IBD drugs: which are safe to use in pregnancy?
azathioprine, mesalazine and sulfasalazine (w folic acid) safe to use in pregnancy and breastfeeding
51
SE of Azathioprine?
bone marrow suppression N+V pancreatitis
52
azathioprine increases risk of what type of ca?
non melanoma skin cancer
53
azathioprine may interact w which drug?
allopurinol - lower doses of azathioprine should be used if taking allopurinol
54
management of methotrexate toxicity?
folinic acid
55
What medication may interact with methotrexate to increase the risk of methotrexate toxicity secondary to reduced excretion?
high dose aspirin
56
what antibody is found in polyarteritis nodosa?
assoc w p-ANCA perinuclear-antineutrophil cytoplasmic abs
57
what infection is assoc w polyarteritis nodosa?
hep B
58
CK and EMG findings in polymyalgia rheumatica?
Normal! Pts may have raised inflammatory markers e.g. ESR\>40
59
features of pseudoxanthoma elasticum?
- retinal angioid streaks
- 'plucked chicken skin' appearance - small yellow papules on the neck, antecubital fossa and axillae
- cardiac: mitral valve prolapse, increased risk of ischaemic heart disease
- GI haemorrhage
60
most common organism causing iliopsoas abscess?
staph aureus
61
most common secondary cause of iliopsoas asbcess?
crohns disease
62
examination findings of iliopsoas abscess?
hyperextension of hip -\> pain as psoas muscle is stretched
ask pt to lift thigh against hand -\> pain due to contraction of psoas muscle
63
ix of choice for iliopsoas abscess?
CT abdomen
64
management of iliopsoas abscess?
abx
percutaneous drainage initial approach
surgery if: failure of percutaneous draining/ presence of another intra-abdo pathology which requires surgery
65
when are topical NSAIDs indicated in osteoarthritis?
for OA of knee/ hand
66
what antibody titre can be used for disease monitoring of SLE?
anti-dsDNA titres
67
what is the major target within the cell of p-ANCA?
myeloperoxidase
68
what is the main target within the cell for cANCA?
proteinase-3
69
What is the minimum steroid intake a patient should be taking before they are offered osteoporosis prophylaxis?
equivalent of pred 7.5mg or more for 3 months or longer
70
proximal myopathy + mechanic hands + interstitial lung disease?
anti-synthetase syndrome
71
SE of hydroxychloroquine?
bulls eye retinopathy - might result in severe and permanent visual loss
baseline exam + annual screening recommended
72
management of patients at risk of corticosteroid- induced osteoporosis?
if pt is \>65 OR had fragility fracture in the past
offer bone protection ie. bisphosphonates
73
management of patients at risk of corticosteroid-induced osteoporosis?
offer bone density scan
if T score bone protection
if between 0 and -1.5 -\> repeat bone density scan in 1-3 years
74
MOA of mycophenolate mofetil?
inhibits inosine-5-monophosphate dehydrogenase which is needed for purine synthesis
- \> reduces lymphocyte production
- \> used in organ transplant to preven rejection/ autoimmune conditions
75
sjogrens - increased risk of which malignancy?
lymphoid (40-60 fold)
76
osteoporosis in males - check what hormone level?
testosterone
- testosterone deficiency
77
muscle pain and stiffness following exercise, cramps, myoglobinuria, low lactate levels during exercise
McArdle's disease
- autosomal recessive type V glycogen storage disease
- caused by myophosphorylase deficiency
78
allergy to which other classes should caution you to starting sulfasalazine?
aspirin, sulphonamides
79
SEs of sulfasalazine?
* oligospermia
* Stevens-Johnson syndrome
* pneumonitis / lung fibrosis
* myelosuppression, Heinz body anaemia, megaloblastic anaemia
* may colour tears → stained contact lenses
80
key features of relapsing polychondritis?
ears: auricular chondritis, hearing loss, vertigo
nasal: saddle nose deformity
Resp tract: e.g. wheeze/ stridor/ hoarseness
ocular: episcleritis/ scleritis/ iritis
arthalgia
less commonly: cardiac valvular regurg, cranial n palsies, peripheral neuropathies, renal dysfunction
81
What is the greatest predictor of future thrombosis in patients with anti-phospholipid syndrome?
Lupus anticoagulant
Lupus anticoagulants have an odds ratio for thrombosis 5 to 16 times higher than controls.
82
most useful characteristic sign of gout on US?
double contour sign
- a hyperechoic, irregular band over the superficial margin of the joint cartilage, produced by the deposition of monosodium urate crystals on the surface of the hyaline cartilage
83
most common cardiac abnormality of Marfans syndrome?
aortic root dilatation
84
what management might you start in Adult onset stills disease if symptoms persist despite initial management?
anti IL1 e.g. anakinra
/ TNF therapy
or methotrexate
85
monitoring while pt is on leflunomide (a DMARD)?
FBC/ LFTs and BP
86
topical NSAIDs used for OA of..?
knees/ hand
87
ank spond, what type of pulmonary fibrosis?
apical
88
Which one of the following cells secretes the majority of tumour necrosis factor in humans?
macrophages
89
lens dislocation in Marfans's?
UP
90
MOA of azathioprine?
inhibits purine synthesis
91
osteopetrosis - dysfunction in?
defective osteoclast function -\> failure of normal bone resorption
-\> results in dense, thick bones that are prone to fracture
aka Marble bone disease
92
features of osteopetrosis?
bone pain/ neuropathies common
prone to fractures
Ca, PO4, ALP normal
stem cell transplant and IFN-gamma have been used for treatment
93
Pseudogout associated factors?
increasing age
haemochromatosis
hyperparathyroidism
low magnesium, low phosphate
acromegaly, Wilson's disease
94
Ehlers-Danlos: what type of collagen is affected?
type 3 collagen
95
complications of Paget's disease?
deafness (cranial nerve entrapment)
bone sarcoma (1% if affected for \> 10 years)
fractures
skull thickening
high-output cardiac failure
96
SEs of TNFa inhibitors e.g. etanercept/ infliximab?
reactivation of TB
etanercept: can cause demyelination
97
which anti-TB medication causes drug induced SLE?
isoniazid
98
pathophysiology of discoid lupus erythematous?
- benign disorder, rarely progresses to SLE
- characterised by follicular keratin plugs
- autoimmune aetiology
99
Radial tunnel syndrome vs Lateral epicondylitis?
- similar presentation
- Radial tunnel syndrome: pain tends to be around 4-5cm distal to the lateral epicondyle
- symptoms may be worsened by extending the elbow and pronating the forearm
- compression of posterior interosseous branch of the radial nerve
100
most common cause of death in sustemic sclerosis?
respiratory involvement
(80%)
- interstitial lung disease and pulmonary arterial hypertension
101
causes of avascular necrosis of hip?
long-term steroid use
chemotherapy
alcohol excess
trauma
102
what autoantibody is most likely to be positive in dermatomyositis?
ANA
- positive in 60%
Anti-Mi-2 only seen in ~25%
103
1st step management of Rheumatoid arthritis?
DMARD monotherapy (most often methotrexate) +/- short course of bridging prednisolone
104
Rheumatoid arthritis: monitoring response to treatment?
CRP + DAS28 score to monitor disease activity
105
Features of polyarteritis nodosa?
fever, malaise, arthralgia
weight loss
hypertension
mononeuritis multiplex, sensorimotor polyneuropathy
testicular pain
livedo reticularis
haematuria, renal failure
- pANCA +ve in 20%, Hep B +ve in 30%
106
Dural ectasia in Marfan's syndrome?
dural ectasia = ballooning of the dural sac at the lumbosacral lvl
may cause lower back pain associated with neurological problems such as bladder and bowel dysfunction.
107
Features of McArdle's disease?
- decreased muscle glycogenolysis. auto recessive. myophosphorylase deficiency.
muscle pain and stiffness following exercise
muscle cramps
myoglobinuria
low lactate levels during exercise
108
pathophysiology of Langerhans cell histiocytosis?
abnormal proliferation of histiocytes
typically presents in childhood with bony lesions
109
Features of Langerhans cells histiocytosis?
bone pain, typically in the skull or proximal femur
cutaneous nodules
recurrent otitis media/mastoiditis
tennis racket-shaped Birbeck granules on electromicroscopy
110
main target of pANCA?
myeloperoxidase (MPO)
111
main target of cANCA?
serine proteinase 3 (PR3)
112
Mx of discoid lupus erythematous?
1st line: topical steroid cream
oral antimalarials may be used second-line e.g. hydroxychloroquine
avoid sun exposure
113
poor prognostic features of Rheumatoid arthritis?
rheumatoid factor +, anti-CCP antibodies
poor functional status at presentation
X-ray: early erosions (e.g. after \< 2 years)
extra articular features e.g. nodules
HLA DR4
insidious onset
114
Which biochemical marker (other than alk phos) could be tested to support a diagnosis of Paget's disease of the bone?
serum/ urine hydroxypoline (raised)
- markers of bone turnover
115
What is used for disease monitoring of SLE?
- ESR
- C3/4 = low
- anti-dsDNA titres
116
Autoantibodies in adult onset Still's disease?
rheumatoid factor and ANA
are NEGATIVE
- its a diagnosis of exclusion
117
Features of adult onset Still's disease?
arthralgia
raised ferritin
macpap, salmon-pink rash
pyrexia: typically rises late afternoon
lymphadenopathy
118
mx of familial mediterranean fever?
colchicine may help
119
what conveys poor prognosis in polymyositis?
interstitial lung disease
- major risk factor for premature death
120
Features of Polymyositis?
proximal muscle weakness +/- tenderness
Raynaud's
respiratory muscle weakness
interstitial lung disease: e.g. fibrosing alveolitis or organising pneumonia
dysphagia, dysphonia
121
Wegeners/ Granulomatosis with polyangiitis: treatment of choice?
cyclophosphamide and streroids
122
what type of GN does Churg-strauss (Eosinophilic GPA) usually cause?
focal segmental glomerulosclerosis
123
What renal abnormalities are associated with Henoch-Schonlein purpura?
glomerular IgA deposition
- usually managed with NSAIDs. if severe: steroids
124
Ankylosing spondylitis: earliest sign on X-ray
sacroiliitis: subchondral erosions and sclerosis
125
Antiphospholipid syndrome: what autoantibodies are associated?
anti-cardiolipin
lupus anticoagulant
anti-B2 glycoprotein 1
126
most common type of psoriatic arthropathy at presentation?
peripheral asymmetric oligoarthropathy
127
Sjogren's syndrome: what medication can stimulate saliva production?
pilocarpine
128
Features of Familial Mediterranean Fever?
- auto recessive
- attacks lasting 1-3 days
- pyrexia
- abdo pain (due to peritonitis)
- pleurisy
- pericarditis
- arthritis
- erysipeloid rash on lower limbs
129
Features of Scheuermann's disease?
usually affects 13-16yo
- affects normal ossification of ring epiphyses of several thoracic vertebrae
- Vertebrae narrower anteriorly -\> Kyphosis
130
Gout: prophylaxis in those where allopurinol is not tolerated?
febuxostat
(also a xanthine oxidase inhibitor)
131
Gout: what are other prophylactic medications in refractory cases?
1. uricase
2. pegloticase (polyethylene glycol modified mammalian uricase) \> given as an infusion once every two weeks
132
in which group of patients would you start alendronate before DEXA scan confirms osteoporosis?
in adults ≥75 with fragility #
133
Perthes disease: management?
- keep the femoral head within the acetabulum: cast, braces
* If \< 6 years: observation
* Older: surgical management with moderate results
* Operate on severe deformities
134
scleroderma renal crisis causing hypertensive emergency: mx?
oral ACEi
* aim gradual reduction of blood pressure at a rate of 10-15 mmHg per day
135
most definitive way of confirming the diagnosis of primary Sjogren’s syndrome?
Salivary gland biopsy
* will show focal lymphocytic sialadenitis
136
management of methotrexate if pt develops new oral ulceration?
hold MTX + discuss with rheum
137
Fibromyalgia: pharmacological therapies?
pregabalin, duloxetine, amitriptyline
138
Ix of Paget’s disease that reflects increased bone turnover?
Serum C-telopeptide (CTx) - useful in monitoring disease progression or treatment efficacy Others: - procollagen type I N-terminal propeptide (PINP) - urinary N-telopeptide (NTx) - urinary hydroxyproline
139
Features of familial Mediterranean fever aka recurrent polyserositis?
more common in people of Turkish, Armenian and Arabic descent Attacks lasting 1-3 days: - fever - abdo pain (peritonitis) - Pleurisy - pericarditis - arthritis - erysipeloid rash on lower limbs
140
Mx of familial Mediterranean fever aka recurrent polyserositis?
Colchicine
141
Features of jaccouds arthropathy?
'reversible' joint deformities such as swan neck, thumb subluxation, ulnar deviation, 'boutonniere' and hallux valgus + NO articular erosions on XR
142
Rheumatoid arthritis: When is a third agent required?
starting of biologic therapy when the patient has been on at least 2 DMARDs, including methotrexate \> with two DAS 28 scores of \> 5.1 at least one month apart
143
What ix will lead to diagnosis of polyarteritis nodosa?
Angiography Ie renal angiogram
144
Tx of choice in SLE?
Hydroxychloroquine
145
Tx to consider in SLE if f internal organ involvement e.g. renal, neuro, eye?
prednisolone, cyclophosphamide
146
What autoantibody is assoc with high risk of scleroderma renal crisis?
anti-RNA polymerase III
147
What antibodies are assoc with high risk of pulmonary hypertension in systemic sclerosis?
anti-U3-RNP
148
What autoantibodies are assoc with Systemic sclerosis assoc myositis?
anti-PM-Scl
149
Management of stills disease?
1st line: NSAIDs (to manage fever, joint pain, serositis) - trial for at least 1 wk before adding steroids if symptoms persist, the use of methotrexate, IL-1 or anti-TNF therapy can be considered
150
Steroid sparing agent for management of giant cell arteritis?
Methotrexate
151
First line management of adult onset stills disease?
NSAIDs first
152
First line management of adult onset stills disease?
NSAIDs first
153
Medications to treat LUPUS nephritis?
Mycophenolate mofetil
154
Ocular features in ehler Danlos?
angioid retinal streaks
155
Muscle biopsy: Endomysial lymphocytic infiltrates that invade nonnecrotic muscle fibres
Polymyositis
156
Muscle biopsy: Perimysial inflammation of lymphocytes and parafascicular atrophy
Dermatomyositis
157
Muscle biopsy: cytoplasmic inclusions
Inclusion body myositis
158
Features of Langerhans cell histiocytosis ? (abnormal proliferation of histiocytes)
1. bone pain, typically in the skull or proximal femur 2. cutaneous nodules 3. recurrent otitis media/mastoiditis 4. tennis racket-shaped Birbeck granules on electromicroscopy
159
first line tx of psoriatic arthropathy?
Same as rheumatoid arthritis Ie DMARD like methotrexate
160
What medication might precipitate a scleroderma renal crisis?
Steroids
161
Ankylosing spondylitis: cardiac abnormalities?
AV node block Aortic regurgitation
