Rheumatology Flashcards
1
Q
Which HLA is associated with Behcets Syndrome?
A
HLA B51
2
Q
Which age group is temporal arteritis most common in?
A
Only >50s
3
Q
How does temporal arteritis present?
A
Headache
Temporal artery (palpable) + scalp tenderness
Jaw claudication (pain after 10-15th bite)
Amaurosis fugax/sudden blindness
Around 50% have symptoms of PMR also
4
Q
Investigations for temporal arteritis?
A
Bloods - raised ESR/CRP
Temporal artery biopsy - may be normal d/t skip lesions
Temporal artery US - halo sign, as sensitive as biopsy but often not available
5
Q
Management of temporal arteritis?
A
Steroids @ 40mg
If visual symptoms, @ 60mg or IV methylprednisolone
Treatment often continues for 1-2 years
Urgent ophthalmology review
There should be a dramatic response, if not consider other Dx
6
Q
What is the biggest cause of morbidity in temporal arteritis?
A
Long-term steroid treatment so consider bisphosphonates/PPI
7
Q
Demographic for PMR?
A
Age >50yrs, often women
8
Q
Symptoms of PMR?
A
Subacute onset (<2 weeks) of bilateral aching -> Worse in morning, improves with activity
Tenderness and morning stiffness in shoulders and proximal limb muscles (NO weakness) +/- mild polyarthritis
Tenosynovitis
Low grade fever + fatigue + WL
Carpel tunnel syndrome (10%)
Temporal arteritis symptoms
9
Q
Investigations for PMR?
A
Raised CRP +/- ESR
ALP raised in 30%
CK is normal
10
Q
Management of PMR?
A
Prednisolone 15mg
Dramatic response
Continue for >2yrs + bisphosphonates/PPI
11
Q
What is the pathophysiology of dermato/polymyositis?
A
capillary obliteration causes ischaemia and muscle infarction
12
Q
Symptoms of dermato/polymyositis?
A
Progressive, symmetrical, proximal muscle weakness
Their bulk is greater than expected from weakness – important sign
Skin features (only in Dermatomyositis)
Photosensitive
Macular rash over back and shoulders – shawl distribution
Heliotrope rash – periorbital
Gottron’s sign – roughened red papules over extensor surfaces of fingers
Other - Raynaud’s, ILD, resp muscle weakness
13
Q
Investigations for dermato/polymyositis?
A
Autoantibodies - o Anti-Jo1 (poly), anti-Mi-2 (derm), ANA (most sensitive, not specific)
Raised serum muscle enzymes - CK, aldolase
Muscle biopsy – shows muscle necrosis, phagocytosis of muscle fibres + inflam infiltrates
Screen for malignancy
14
Q
Management of dermato/polymyositis?
A
Systemic steroids - prednisolone
Immunosuppressive drugs – methotrexate
<10% of cases are fatal
15
Q
What is Langerhans cell histiocytosis?
A
Langerhans cell histiocytosis is a rare condition associated with the abnormal proliferation of histiocytes (phagocytic cell). It typically presents in childhood with bony lesions
16
Q
Features of Langerhans cell histiocytosis?
A
bone pain, typically in the skull or proximal femur, cutaneous nodules, recurrent otitis media/mastoiditis
17
Q
Diagnosis of Langerhans cell histiocytosis?
A
Electronmicroscopy - tennis racket-shaped Birbeck granules
18
Q
Is RA more common in M or F?
A
Females >2:1
19
Q
Which HLA is RA associated with?
A
HLA DR4 (main), also HLA DR1
20
Q
What are the RFs for RA?
A
smoking + genetics (combination is v significant)
21
Q
Joint presentation of RA?
A
Symmetrical, swollen, painful and stiff small joints of the hands, wrist and feet
• Worse in the morning
• Larger joints may become involved
• Does not affect DIP joints or spinal involvement
Fatigue, more likely to get carpel tunnel
22
Q
Signs of deformity in RA?
A
Boutonniere deformity
Ulnar deviation of MCP joints
Swan-neck deformity of fingers
Z deformity of the thumb
23
Q
Extra-articular presentation of RA?
A
Splenomegaly (+low WCC and RA = Felty’s syndrome)
Nodules - elbow, lung, heart valve, achilles tendon
Eye - episcleritis/scleritis
Lung - fibrosis, pleuritis, pleural effusion
24
Q
What is RA a significant RF for?
A
CVS disease - many of RA patients are on NSAIDs (increased risk), more likely to be inactive (increased risk)
25
What investigation should you do prior to surgery for RA?
X-ray of cervical spine to check for atlantoaxial subluxation - impt for intubation
26
Autoantibodies for RA?
RhF +ve 70% (not specific, cheaper test than anti-CCP)
Anti-CCP 98% (also +ve up to 10yrs before getting RA)
Worse prognosis with +ve RhF and anti-CCP
27
XR findings in RA?
SOLE
Soft tissue swelling
Osteopaenia
Loss of joint space
Erosions
28
What advice should you give to women taken methotrexate who want to conceive?
methotrexate is not safe in pregnancy and needs to be stopped at least 6 months before conception
29
Which RA medications are safe in pregnancy?
sulfasalazine, azathioprine and hydroxychloroquine
30
If a patient has an allergy to aspirin, which DMARD medication should be avoided?
Sulfasalazine
- should also be cautious giving sulfasalazine to patients with G6PD
31
Which HLA is ank spon associated with?
HLA B27
32
Symptoms of ank spon?
Backache and stiffness
- worse after inactivity
- begins in lumbar region -\> thoracic
- may develop question mark posture in longterm
33
Signs of ank spon?
Schober's test - \<5cm increase
Reduced lateral flexion
Chest expansion \<5cm
34
7A's of extra-articular features of ank spon?
- Atlanto-axial subluxation (C1 + C2 move out of shape)
- Anterior uveitis
- Apical lung fibrosis
- Aortic regurgitation
- Amyloidosis
- Achilles tendonitis
- Autoimmune bowel disease
35
Investigations in ank spon?
Raised ESR/CRP
Hypergammaglobulinaemia
XR:
Sacroiliac joints - erosion, sclerosis
Squaring of the lumbar spine - give bamboo spine (becomes osteoporotic and risk of microfractures on movement)
Syndesmophytes
36
Scoring systems in ank spon?
BASDAI (disease activity index)
BASFI (functional index)
Scores need to be \>4 on 2 occasions 3 months apart despite NSAIDS
37
Management of ank spon?
General - patient education, stop smoking, exercise, PT
Therapy - NSAIDS for 3 months, local steroids (systemic steroids won't help spine but may help surrounding features), Anti-TNF therapy
38
Which category of medication will not help in ank spon?
DMARDs
39
Male to Female ratio for Gout?
M:F 4:1
40
Cause of Gout?
Deposition of monosodium urate crystals in and near joints
41
Causes of increased crystal deposition in Gout?
Increased production – hereditary, leukaemia/lymphomas (tumour lysis syndrome), cytotoxic drugs, increased dietary purines
Impaired excretion - diuretics, CKD, alcohol excess (beer)
-\> Diuretics may make your body fluid more concentrated (so higher conc of uric acid), some diuretics also impair the excretion of uric acid
42
What can precipitate Gout flare ups?
Trauma, surgery, starvation, infection or diuretics
43
RFs for Gout?
Male, meat + seafood (high in purines), alcohol, diuretics, obesity, HTN, coronary heart disease, DM, CKD, heart failure
44
Presentation of Gout?
Acute pain, swollen, hot, erythematous joint, reaching a peak after **_6-12hrs_**
Often with fever + malaise
Primary site – MTP joint
Other sites – knee, midtarsal joints, wrists, ankles, small hand joints
45
What type of crystals will you see on polarised light microscopy following aspiration of a joint with Gout vs Pseudogout?
Crystals:
Strongly negatively birefringent needles = gout
Rhomboid positively birefringent = pseudogout
46
What happens to the urate levels in your body in Gout?
Serum urate - may be raised/normal, useful to see response to Tx
Urine uric acid - may be raised
47
XR findings in chronic Gout?
punched-out lesions may be seen, juxta-articular erosions
48
USS/CT/MRI findings in Gout?
can identify urate deposition, structural joint damage and joint inflammation
49
Management of Gout?
Untreated, resolves over 5-15 days
With treatment, lasts about 3-5 days
General - RICE, continue prophlaxis therapy
NSAIDs - 1st line
Colchicine - if NSAIDs poorly tolerated/HF/on anticoagulation
Steroids - useful if NSAIDs + Colchicine are CI
50
Prevention of Gout?
urate lowering therapy: allopurinol (do not start until after acute attack) or febuxostat (more potent and expensive)
Also lifestyle modifications
51
What is the main SE of colchicine?
Diarrhoea
52
Most common demographic in SLE?
F:M 9:1, commoner in Afro-Caribbean + Asians, age 20-40yrs
53
What may trigger SLE?
Infection, injury, stress, EBV, drug induced
54
Which drugs may cause drug-induced SLE and which Ab is it associated with?
SHIPP (Sulfonamide (abx), hydralazine (HF), isoniazid (TB), phenytoin (seizures), procainamide (arrhythmia))
Anti-histone Abs in 100%
55
Symptoms of SLE?
Discoid rash
Oral ulcers (usually painless)
Photosensitivity
ANA (\>95%)
Malar rash (butterfly rash – occurs in 50%)
Immunological phenomenon (anti-smith, anti-dsDNA, anti-phospholipid, Ro, La)
NEurological (altered mental state, seizures, stroke, headache, depression)
Renal (persistent proteinuria/cell casts)
Arthralgia/non-erosive arthritis (occurs in 90%, involves 2+ peripheral joints)
Serositis (pleuritis/pericarditis)
Haematological (pancytopaenia)
56
Investigations for SLE?
3 best tests
1) Anti-dsDNA antibody titres
2) Complement: low C3+C4
3) raised ESR
57
Management of SLE?
Severe flares -\> req urgent IV cyclophosphamide and high-dose prednisolone
Maintenance -\> NSAIDs and hydroxychloroquine, low-dose steroids may be used if chronic
Cutaneous symptoms -\> Topical steroids, suncream
Biologicals -\> Target B cells with rituximab/belimumab
Lupus nephritis -\> immunosuppression; steroids + cyclophosphamide/mycophenolate
58
Symptoms of antiphospholipid syndrome?
Antiphospholipid Abs cause CLOTS:
Coagulation defect (arterial and venous thrombosis)
Livedo reticularis
Obstetric (recurrent miscarriages)
Thrombocytopaenia (decreased platelets)
59
Investigations of antiphospholipid syndrome?
Measure anti-phospholipid antibodies
o Lupus anticoagulant Ab
o Anticardiolipin Ab
Causes a paradoxical rise in APTT (measures the speed of clotting)
60
Who would you screen for anti-phospholipid syndrome?
\<50 with stroke + woman with 3+ pregnancy losses before 10 weeks
61
Management of anti-phospholipid syndrome?
Healthy lifestyle advice
```
One VTE/arterial thrombosis: Warfarin lifelong (aim INR 2-3)
Recurrent VTE (whilst on warfarin): Add in aspirin (aim INR 3-4)
```
Seek advice in pregnancy
62
Which features of SLE are less common in drug-induced lupus?
Renal and nervous system involvement
63
What are the classical features of raynauds disease vs phenomenon?
Raynaud's disease typically presents in young women (e.g. 30 years old) with bilateral symptoms
Raynaud's phenomenon (secondary causes):
connective tissue disorders
**scleroderma (most common)**
RA, SLE, leukaemia
type I cryoglobulinaemia, cold agglutinins
use of vibrating tools
drugs: oCP, ergot
cervical rib
64
How should you manage Raynaud's?
Suspected secondary Raynaud's -\> referred to secondary care
first-line: CCB e.g. nifedipine
IV prostacyclin (epoprostenol) infusions: effects may last several weeks/months
65
What percentage of patients with psoriasis develop psoriatic arthropathy?
10-20%
All patients with psoriasis should be offered an annual assessment
66
How can you differentiate between psoriatic arthritis and RA?
Psoriatic: DIP joints, no RF, no skin nodules
RA: MCP joints, RF in 70%, Skin nodules
67
What are the features of psoriatic arthritis?
Stiffness, swelling and pain in joints, ligaments and tendons
Arthritis is relapsing and remitting
Can be symmetrical polyarthritis/asymmetrical oligoarthritis, usually in hands and feet
Usually preceded by the rash by a few years
Nail changes in 80% – pitting, yellowing, onycholysis
Dactylitis
68
XR changes in psoriatic arthritis?
Unusual combination of coexistence of erosive changes and new bone formation
Periostitis
'pencil-in-cup' appearance
69
How do you treat psoriatic arthritis?
Same as for RA
• NSAIDs – to relieve MSK symptoms
• Corticosteroid injections
• DMARDs at an early stage – e.g. Methotrexate
• TNF-alpha inhibitors
70
What is the most common causative organism in septic arthritis in young sexually active people? vs most common organism overall?
Neisseria gonorrhoeae
overall: Staphylococcus aureus
71
Treatment of septic arthritis?
IV flucloxacillin or clindamycin if penicillin allergic
For 6-12 weeks
72
How many people with reactive arthritis have HLA association?
HLA B27, 75%
73
What is the demographic for patients with reactive arthritis?
Young white adults
74
What causes reactive arthritis?
Occurs 1-4 weeks after either:
• GU infection from STI (e.g. chlamydia)
• GI infection (campylobacter, salmonella, shigella or Yersinia)
It may be chronic or relapsing
75
Which joints are affected in reactive arthritis and what are the other symptoms?
- Asymmetrical
- Lower extremities
- Oligoarthritic (usually no more than 6 joints)
“I knee’d to pee”
I = conjunctivitis/anterior uveitis
Knee’d = arthritis
Pee = urethritis
(organism is not present in arthrocentesis of joint)
```
May also see these skin changes:
keratoderma blenorrhagica(waxy yellow/brown papules on palms and soles)
Circinate balanitis (painless vesicles on the coronal margin of the prepuce)
```
76
Management of reactive arthritis?
No specific cure
Splint joints
NSAIDs or intra-articular steroid injections
Consider sulfasalazine or methotrexate if symptoms \>6 months
Symptoms rarely last \>12 months
77
Name a SE caused by hydroxychloroquine?
Bull's-eye retinopathy
78
What is the pathophysiology of osteoporosis?
Skeletal disease characterised by:
- Low bone mass
- Microarchitectural deterioration of bone tissue
- \> increase in bone fragility and susceptibility to fracture
79
Name of treatments that can be used for osteoporosis?
Bisphosphonates, HRT, Denosumab, Teriparatide, Ca + vit D
80
MOA/SEs of bisphosphonates?
MOA - decrease osteoclast activity, acts on the cholesterol synthesis pathway - inhibits the enzyme 'Farnesyl Pyrophosphate Synthase'
SEs - GI irritation (alendronate), sit up and take half an hour before food
Osteonecrosis of the jaw (rare)
81
HRT, MOA/SEs?
MOA - Decrease osteoclast activity
Benefits – reduce fractures, reduce risk of CC, prevent menopausal symptoms
Negatives – increase breast cancer risk, stroke, CVS disease, VTE
82
MOA/SEs of Denosumab?
MAb to RANK ligand, decreases osteoclast
Negative – rebound increase of bone turnover when stopped
83
MOA/CIs of Teriparatide?
Increases osteoblast activity (ANABOLIC), PTH analogue
Reduced risk of fractures by \>50%
Increases bone density and improves trabecular structure
CI – Hx of bone cancer/mets
84
What are the FRAX and DEXA score?
FRAX - assess risk over next 10 years, if \>10% warrants a DEXA scan
DEXA – T\>-1 = normal, T-1 to -2.5 = osteopaenia, T-2.5 = osteoporosis
o T score – compares your BMD to that of a young person with peak BMD
o Z score – compares your BMD to an average person of the same age, sex + ethnicity
85
RFs for osteoporosis?
SHATTERED
Steroids
Hyperthyroidism/hyperparathyroidism
Alcohol + smoking
Thin BMI \<22
Testosterone deficiency
Early menopause
Renal/liver failure
Erosive bone disorders (Inflammatory cytokines increase bone resorption: RA, spondyloarthritis, SLE, IBD)
Dietary Ca deficiency
86
Management of patients with Osteopaenia?
DEXA T score between -1 and -2.5
- Diet (adequate Calcium + Vit D)
- Regular weight-bearing exercises - dancing/skipping/gym
- Reduce drinking + stop smoking
Rescan in 3 years
Management of patients at risk of corticosteroid-induced osteoporosis
87
What is the classic triad in Behcets disease and what other symptoms may occur?
AI inflammation of vessels
Oral ulcers, genital ulcers, anterior uveitis
thrombophlebitis, DVT, arthritis, aseptic meningitis, erythema nodosum
88
What demographic does Behcet's disease most commonly occur in?
Young men from eastern Med (Turkey), FH
89
What simple test can be used to assist diagnosis of Behcet's disease?
Positive pathergy test is suggestive (the puncture site of a needle prick becomes inflamed with small pustules forming)
90
Management of Behcet's disease?
Topical steroids – for ulcers + uveitis
Systemic steroids
Steroid-sparing drugs – e.g. azathioprine
91
What is McArdle's Disease?
AR disease - inability to release glucose from glycogen
Characterised by reversible exercise intolerance, fatigue and acute crisis (severe fatigue and acute muscle cramps)
'Second wind' phenomenon
92
Investigations for McArdle's Disease?
Clinical
Elevated CK
Myoglobinuria in urine
93
Which cells secretes the majority of tumour necrosis factor in humans?
Macrophages
94
What is Rugger Jersey spine and when is it seen?
Prominent endplate densities at multiple vertebral levels to produce an alternating sclerotic-lucent-sclerotic appearance
Hyperparathyroidism
95
What causes Marfans Syndrome?
Autosomal dominant connective tissue disorder.
Caused by a defect in the FBN1 gene on chromosome 15 that codes for the protein fibrillin-1
(think FBI/MI5)
96
What is included in the Major/Minor criteria for Marfans syndrome?
Major criteria (\<2):
Lens dislocation
Aortic dissection or dilatation (risk rises in pregnancy)
Dural ectasia (dilation)
Skeletal features: arachnodactyly
(long spider fingers), armspan \> height,
pectus deformity, scholiosis
Minor signs:
MV prolapse, joint hypermobility, high-arched palate
97
Management of Marfans Syndrome?
B-blockers to slow dilation of the aortic root
Annual echos
98
Pathophysiology of Ehler Danlos Syndrome?
Autosomal dominant CTD – affects T3 collagen
99
Presentation of Ehler Danlos?
Presentation:
Joint hypermobility – recurrent dislocation
Elastic, fragile skin
Easy bruising
Aortic regurgitation, mitral valve prolapse, aortic dissection
SAH
Retinal streaks
100
Fibromyalgia presentation?
9 pairs of tender points on the body – if tender in 11/18 of these points, Dx is more likely
More commonly on neck, shoulders, spine
101
Management of fibromyalgia?
Explanation
Aerobic exercise
CBT
Medication: pregabalin, duloxetine, amitriptyline, replace vit D
102
What is the most common pathogen if sickle-cell patents develop osteomyelitis?
Salmonella
In everyone else: Staph aureus
103
How to treat osteomyelitis?
Flucloxacillin for 6 weeks
Clindamycin if allergic
104
What do you need to test before commencing azathioprine?
Thiopurine methyltransferase (TPMT) deficiency is present in about 1 in 200 people and predisposes to azathioprine toxicity (pancytopaenia)
105
What three categories of people should be offered bisphosphonates?
Patients \>65 yrs or those who've previously had a fragility fracture
Patients \<65 yrs with osteoporosis Dx from DEXA scan
Patients taking/anticipated to be taking steroids at 7.5mg daily for \>3m
106
Management of RA?
DMARDs - should start within 3/12 of persistent symptoms, can take 6-10wks to work (hydroxychloroquine, methotrexate - try alone first, sulfasalazine)
Steroids - can be used as a bridging agent waiting for DMARDs to work
Biological agents - TNF-a inhibitors (first-line when no response to 2xDMARDs), then B cell depletion (rituximab), IL1/6 inhibitors (tocilizumab), disruption of T cell function
107
When should you stop biological treatment in RA?
When someone becomes unwell
108
What should you check before starting immunosuppressants?
Hep B, HIV, TB
109
How should you assess response to treatment in RA?
NICE recommends using a combination of CRP and disease activity (using a composite score such as DAS28) to assess response to treatment
110
What symptoms do you get in De Quervain's tenosynovitis?
Pain on the radial side of the wrist/tenderness over the radial styloid process
111
How to manage De Quervain's tenosynovitis?
analgesia
steroid injection
immobilisation with a thumb splint (spica) may be effective
surgical treatment is sometimes required
112
What is discoid lupus erythematous?
Only the rash:
erythematous, raised rash, sometimes scaly,
may be photosensitive,
more common on face, neck, ears and scalp,
lesions heal with atrophy, scarring (may cause scarring alopecia), and pigmentation
113
Treatment of discoid lupus erythematous?
topical steroid cream
Hydroxychloroquine (use this DMARD is SLE also)
avoid sun exposure
Very rarely progresses to SLE (5%)
114
Which sex is more associated with a poor prognosis in RA?
Female
115
Which medications may worsen osteoporosis (other than glucocorticoids)?
SSRIs
antiepileptics
proton pump inhibitors
glitazones
long term heparin therapy
aromatase inhibitors e.g. anastrozole
116
What is Pseudoxanthoma elasticum?
An inherited condition (usually AR) characterised by an abnormality in elastic fibres
117
What are the symptoms 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
gastrointestinal haemorrhage
MAGIC
118
What is the most worrying condition that can occur in patients with Pseudoxanthoma elasticum?
Cardiac problems: IHD, mitral valve prolapse
Progressive narrowing of small/medium vessels from changes in the elastic properties of vessels
119
What is the most common infective organism in an iliopsoas abscess?
Staph aureus
120
What can cause a secondary iliopsoas abscess?
Crohn's (commonest cause in this category)
Diverticulitis, colorectal cancer
UTI, GU cancers
Vertebral osteomyelitis
Femoral catheter, lithotripsy
Endocarditis
intravenous drug use
121
Symptoms of an iliopsoas abscess?
Fever
Back/flank pain
Limp
Weight loss
122
How to treat an iliopsoas abscess?
Antibiotics + percutaneous drainage -\> successful in around 90% of cases
Surgery is indicated if:
1. Failure of percutaneous drainage
2. Presence of an another intra-abdominal pathology which requires surgery
123
Symptoms of limited cutaneous systemic sclerosis?
Calcinosis (calcium deposits in the skin)
Raynaud’s (long Hx)
oEsophageal and gut dysmotility
Sclerodactyly (swollen tight digits)
Telangiectasia
124
Which Abs is most associated with limited cutaneous systemic sclerosis?
Anti-centromere Abs 70-80%
125
What is the difference between limited and diffuse cutaneous systemic sclerosis?
Limited - limited to the skin on the hands/face/feet
Diffuse - diffuse skin involvement and early organ fibrosis (lung, heart, GI, renal)
126
Which Abs is most associated with diffuse cutaneous systemic sclerosis?
Antitopoisomerase-1 (anti-Scl70) Abs in 40% and anti-RNA polymerase in 20%
127
Management of systemic sclerosis?
Currently no cure
Can be managed with steroids initially
Immunosuppression – IV cyclophosphamide (for organ involv or progressive skin disease)
Treat specific features of the disease e.g. oesophageal reflux
Monitor BP and renal function and echos
ACE-i/ARBs to decrease risk of renal crisis
Tx Raynauds -\> hand warmers, battery-powered gloves, CCB/ACEi (vasodilators)
128
What is the most common cause of death in diffuse cutaneous systemic sclerosis?
interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH)
Other complications include renal disease and HTN
129
Which medication should not be prescribed alongside methotrexate?
Trimethoprim - can cause bone marrow suppression and severe pancytopaenia
High-dose aspirin increases the risk of methotrexate toxicity secondary to reduced excretion
130
How often do you take methotrexate?
Weekly
131
What should you take alongside methotrexate?
Folic acid, at least 24hrs after methotrexate
132
How to treat methotrexate toxicity?
Folinic acid
133
What is Paget's disease?
Increased bone turnover from increased numbers of osteoclasts and resulting osteoblasts, resulting in:
- Disorganised remodelling (bone = weaker, bulkier, less compact and more vascular)
- Bone enlargement
- Deformity
- Weakness
134
What causes Paget's disease of the bone and who is more at risk?
both genetic and environmental factors (e.g. infections)
Over 40s, male, commoner in temperate climates (lacking extremes in temperatures)
135
What are the symptoms of Paget's disease?
Commonly asymptomatic (~70%)
Deep ‘boring’ bone pain
Deformity or bone enlargement -\>Commonly in the pelvis, lumbar spine, skull, femur and tibia
136
What would you see on an initial blood profile and urine in Paget's?
raised ALP, Ca2+ and PO43- normal
Urinary hydroxyproline
137
What would you see on XR in Paget's disease?
- Localised enlargement of bone
- Osteolysis (bone degradation) and sclerosis (hardening)
- Specific findings in Paget’s:
o Classic ‘blade of grass’ lesion between healthy and diseased long bones
o ‘Cotton wool’ pattern in the skull
138
Complications of Paget's Disease?
* Pathological fractures
* Osteoarthritis
* Nerve compression from bone overgrowth e.g. CN8 (deafness/tinnitus), spinal nerves
* Hypercalcaemia (Vomiting, polyuria, polydipsia)
* Osteosarcoma
139
Management of Paget's Disease?
Analgesia (NSAIDs/paracetamol)
If not enough, **bisphosphonates**
May need surgery for bone deformity, osteoarthritis, fractures and nerve compression
Monitoring by _ALP_ d/t risk of osteosarcoma
140
What is Von Gierke Disease?
T1 glycogen storage disease - unable to break down glycogen into glucose
Usually presents in first few months of life with complications of hypoglycaemia
141
What causes osteomalacia?
Vitamin D deficiency
142
What does the initial blood work look like in osteomalacia?
Low Ca2+, low PO43-, raised ALP, raised PTH
143
What might you see on XR in osteomalacia?
- Loss of cortical (hard) bone
- Partial fractures
- Pseudofractures: ‘Looser’s zones’ – linear areas of low density surrounded by sclerotic borders
144
How to treat osteomalacia?
Vitamin D
Monitor calcium levels (at risk of hypercalcaemia)
145
What is carpel tunnel syndrome?
compression of median nerve in the carpal tunnel
146
Symptoms of carpel tunnel syndrome?
Pain/parasthesia in thumb, index + middle finger
Weakness of thumb abduction, wasting of thenar eminence
Tinel + Phalen's test positive
147
Causes of carpel tunnel syndrome?
idiopathic
pregnancy
oedema e.g. heart failure
lunate fracture
rheumatoid arthritis
RF: obesity
148
Management of carpel tunnel syndrome?
```
corticosteroid injection
wrist splints at night
surgical decompression (flexor retinaculum division)
```
149
What is osteopetrosis?
Opposite of osteoporosis
also known as marble bone disease, is a rare condition characterised by bone hardening due to the malfunction of osteoclasts which don’t absorb the bone correctly
results in dense, thick bones that are prone to fracture
150
What are the symptoms and blood work for osteopetrosis?
bone pains and neuropathies are common,
calcium, phosphate and ALP are normal
151
Which drugs can cause gout?
diuretics: thiazides, furosemide
ciclosporin
alcohol
cytotoxic agents
pyrazinamide
aspirin
CAT FLAP
152
What can cause/precipitate a flare of pseudo gout?
dehydration, intercurrent illness, haemochromatosis (high iron), hyperparathyroidism, long-term steroids
153
What would you use to diagnosis pseudogout and what would it show?
XR - linear opacification of articular cartilage (collection of chondrocalcinosis)
Joint aspiration - raised WCC, predominantly neutrophils, weakly positive birefringent crystals, joint fluid often looks purulent and SA must be excluded
154
Management of pseudogout?
No specific treatment
Acute attacks: cool packs, rest, aspiration
Mx: intra-articular steroids, NSAIDs/colchicine
155
Male to female ratio of systemic sclerosis?
4x more common in females
156
What other non-specific antibodies are present in systemic sclerosis?
ANA positive in 90%
RF positive in 30%
157
The presence of anti-Ro antibodies in SLE can lead to what defects in a foetus?
heart block
158
What is Still's disease?
The adult form of systemic juvenile idiopathic arthritis
A rare inflammatory disorder than can affect the whole body
159
What are the symptoms of Still's disease?
triad of joint pain, spiking fevers (typically in early eve daily), and a pink (salmon) bumpy rash is very characteristic of adult-onset Still’s disease
160
Blood test result in Still's disease?
Elevated serum ferritin
161
Management of Still's disease?
NSAIDs for first few weeks
Steroids if not managed effectively
Mtx, IL-1 or anti-TNF
162
What is frozen shoulder?
Aetiology not fully understood
Known as adhesive capsulitis
163
What is frozen shoulder associated with?
Diabetes (20%)
164
Features of frozen shoulder?
External rotation is affected - both active + passive
Bilateral in up to 20%
165
How long does frozen shoulder typically last?
Between 6 months - 2yrs
166
Treatment of frozen shoulder?
NSAIDs, physio, PO or IA steroids
167
Demographic of osteoarthritis?
More common in females, \>50yrs, obesity, DDH
168
Symptoms of osteoarthritis?
Pain worse at end of day
Most commonly in hip/knee
Crepitus, joint instability
Heberden's nodes (DIPs), bouchard's nodes (PIPs)
Most commonly affects the CMC/DIP joints
Squaring of the thumb
169
XR findings for osteoarthritis?
LOSS
Loss of joint space
Osteophytes
Subchondral cysts
Subarticular sclerosis
170
Management of osteoarthritis?
Exercise, WL, physio
Analgesia - paracetamol, NSAIDs, IA injections
Joint replacement surgery
171
Causes of avascular necrosis of the hip?
long-term steroid use, chemotherapy, alcohol, trauma
172
What might you see on XR, if at all, in avascular necrosis of the hip?
Crescent sign (shows osteonecrosis along the edge of the femur head)
173
Management in avascular necrosis of the hip?
Joint replacement
174
In ank spon, what will TNF-inhibitors not have an effect on?
Radiological progression of disease
175
Which drug may cause a significant interaction with azathioprine and constitute a never event?
Allopurinol
176
Is Azathioprine safe in pregnancy?
Yes
177
What is the cause of De Quervain's tendinosis and who gets it?
De Quervain's tendinosis may be caused by overuse. It also is associated with pregnancy and rheumatoid disease. It is most common in middle-aged women.
178
Poor prognostic factors for RA?
RF + anti-CCP +ve
Poor functional BL
XR - early erosions
HLA DR4
Extra-articular features
Slow onset
179
What is a simplistic general rule to remember whether conditions are AR or AD?
dominant = structural, recessive = metabolic
180
Examples of large cell vasculitis?
Giant cell arteritis, Takayasu’s arteritis
181
Examples of medium cell vasculitis?
Polyarteritis nodosa, Kawasaki disease
182
Examples of small cell vasculitis?
- ANCA +ve vasculitis – p-ANCA associated microscopic polyangiitis, glomerulonephritis and Churg-Strauss syndrome, and c-ANCA associated granulomatosis with polyangiitis
- ANCA -ve vasculitis – HSP, Goodpasture’s syndrome and cryoglobulinemia
183
Who gets PAN?
Hep B
Middle aged men
184
What are the features of PAN?
Fever, malaise, arthralgia
WL
HTN
Mononeuritis multiplex – sensorimotor polyneuropathy
Testicular pain
Livedo reticularis
Renal failure + haematuria
185
Which autoAb is positive in PAN and how likely is this?
pANCA in 20%
186
What is the most common target of pANCA?
Myeloperoxidase (MPO)
187
What is the most common target of cANCA?
Serine proteinase 3 (PR3)
188
Which autoAb is specific for mixed connective tissue disease and what are the symptoms?
anti-RNP
R Raynauds
N swollen hands with No synovitis
P Pain in muscle and joints
189
Which drug is associated with atypical stress fractures of the proximal femoral shaft?
Bisphosphonates (essential because a reduction in osteoclast activity means bones last longer and are therefore more prone to fractures)
190
Which nerve passes through the cubital tunnel?
Ulnar nerve
191
Poor prognostic factor in polymyositis?
Anti-Jo Abs, because this is nearly always associated with ILD which gives a poorer prognosis
192
What happens to the hair follicles in discoid lupus?
Characterised by follicular keratin plugs (keratin forms over the top of the follicle)
193
Which cytokines is the most important in the pathophysiology of rheumatoid arthritis?
Tumour necrosis factor (TNF)
194
What is the mechanism of action of azathioprine?
azathio'purine' = purine synthesis inhibition
195
What is familial mediterranean fever?
Familial Mediterranean Fever (FMF, also known as recurrent polyserositis) is an autosomal recessive disorder causing autoinflammation which typically presents by the second decade
196
What is the mode of inheritance of FMF?
AR
197
How long do attacks of FMF usually last and what symptoms do you get?
attacks typically last 1-3 days
pyrexia
abdominal pain (due to peritonitis)
pleurisy
pericarditis
arthritis
erysipeloid rash on lower limbs
198
How to treat FMF?
Colchicine
199
What is meralgia paraesthetica?
paraesthesia or anaesthesia in the distribution of the lateral femoral cutaneous nerve (LFCN)
200
What can cause meralgia paraesthetica?
Obesity
Pregnancy
Tense ascites
Trauma
Iatrogenic - e.g. surgery
Various sports
201
How to manage meralgia paraesthetica?
Injection of the nerve with local anaesthetic
202
What type of immunoglobulin is RF and what type of immunoglobulin in the body does it react to?
Rheumatoid factor (RF) is a circulating antibody (usually IgM) that reacts with the Fc portion of the patients own IgG
203
What is the gender divide in psoriatic arthritis?
EQUAL, hallelujah
204
What is radial tunnel syndrome?
Thought to be compression of the posterior interosseous branch of the radial nerve
205
What are the symptoms of radial tunnel syndrome?
pain tends to be around 4-5 cm distal to the lateral epicondyle
symptoms may be worsened by extending the elbow and pronating the forearm
