Rheumatology Flashcards
1
Q
What are the red flags for back pain?
A
age <20 or >55 sphincter disturbance current or recent infection history of malignancy morning stiffness constant or progressive pain neurological disturbance bilateral or alternating leg pain FLAWS thoracic back pain acute onset in elderly people nocturnal pain
2
Q
what does a nerve root lesion at L2 cause?
A
weakness in hip flexion and adduction
3
Q
what does a nerve root lesion at L3 cause?
A
weakness in knee extension and hip adduction
4
Q
what does a nerve root lesion at L4 cause?
A
weakness in foot dorsiflexion + inversion + knee extension
5
Q
what does a nerve root lesion at L5 cause?
A
weakness in: great toe dorsiflexion foot dorsiflexion + inversion knee flexion hip extension + abduction
6
Q
what does a nerve root lesion at S1 cause?
A
weakness in foot plantar flexion + eversion
7
Q
nerve roots of knee jerk reflex?
A
L3,L4
8
Q
nerve roots of ankle jerk reflex?
A
S1,S2
9
Q
Ix lower back pain?
A
MRI only if suspected malignancy, infection, fracture, CES or ank spond
DRE
10
Q
Mx lower back pain?
A
c: physical activity and exercise
M: NSAIDS + PPI if >45
Other: group exercise programme
manual therapy (techniques such as massage)
radio frequency denervation
epidural injections of Land steroid for acute/severe sciatica
11
Q
what is rheumatoid arthritis?
A
chronic systemic inflammatory condition characterised by a symmetrical defaming poly arthritis
12
Q
epidemiology RA?
A
F>M (2:1), 50-60yo, 1% prevalence (higher in smokers)
HLA DR1, DR4
13
Q
what are some poor prognostic indicators of rheumatoid arthritis?
A
\+ve RhF insidious onset early erosions HLA DR4 \+ve anti-CCP extra-articular features
14
Q
S/S rheumatoid arthritis?
A
swollen, painful small joints in hands and feet
ulna deviation of MCP and radial deviation at wrist
stiffness better with exercise/worse in morn
swan neck, boutonniere, z-thumb
15
Q
associated features rheumatoid arthritis?
A
eyes - episcleritis, keratoconjunctivitis sicca
neck - Atlanta-axial subluxation (may cause cord compression)
heart -pericarditis
lungs - fibrosis, rheumatoid nodules
hands - de quervains tenosynovitis, CTS, trigger finger
speen -splenomegaly, felty’s syndrome
kidneys - amyloid
all - rheumatoid nodules
16
Q
what is Felty’s syndrome?
A
SANTA Splenomegaly anaemia neutropenia thrombocytopenia arthritis
17
Q
Diagnostic criteria for rheumatoid arthritis?
A
American College of Rheumatology Criteria
NICE recommend clinical diagnosis
18
Q
ix rheumatoid arthritis?
A
- DAS28 [disease activity score]
- squeeze test positive (pain across MCPJ or MTPJ)
Bloods: FBC (anaemia, raised ESR, CRP), RhF (+ve 70%), anti-CCP (90-95% specific, 80% sensitive), ANA(+ve in 30%)
TJC/SJC
Imaging: XR (baseline), USS (synovitis), MRI, CXR
19
Q
what are TJC and SJC
A
tender joint count and swollen joint count (part of DAS28)
20
Q
What should be monitored in rheumatoid arthritis?
A
DAS28, CRP
if DAS28 >5.1 consider stepping up Mx
21
Q
Mx rheumatoid arthritis?
A
1st line: conventional DMARD
2nd line: combination cDMARD therapy (2x)
3rd line: biological DMARD + cDAMRD
Surgery: ulna stylectomy, joint prosthesis
Flare ups: corticosteroids (PO, IM methylprednisolone)+/-NSAIDs
22
Q
Give some examples of cDMARDs?
A
methotrexate sulfasalazine hydroxychloroquine MMF cyclophosphamide azathioprine
23
Q
Give some examples of bDMARDs?
A
(TNF-a inhibitors, B cell/T depletion) EtanercepT (SE demyelination) Infliximab Adalimumab Rituximab
24
Q
How are methotrexate, sulfasalazine and hydroxychloroquine monitored?
A
M- regular FBC and LFT (risk of myelosuppression and cirrhosis)
S - salicylate so not given if aspirin sensitive
H - annual visual acuity testing. OK IN PREGNANCY.
25
what joints does OA affect?
weight bearing: hip and knee
| hands: CMCJ, DIPJ, PIPJ
26
What joints does RA affect?
MCPJ, PIPJ
27
What is swan neck deformity?
stretching of the volar plate
PIPJ hyperextension
DIPJ flexion
28
what is seen on XR in OA?
```
LOSS
loss of joint space
osteophytes
subchondral cysts
subchondral sclerosis
```
29
what is seen on XR in rheumatoid?
```
LESS
loss of joint space
erosions (periarticular)
soft tissue swelling
subluxation and deformity
```
30
aetiology of gout?
monosodium urate crystals deposition in and around joints -> erosive arthritis
31
epidemiology of gout?
M>F 5:1
32
gout associations?
HTN
IHD
Metabolic syndrome
33
Causes of gout?
hereditary
drugs (loop diuretics, thiazide diuretics,aspirin,)
decreased excretion (primary gout, renal impairment)
increased cell turnover (lymphoma, leukaemia, haemolysis
ETOH XS
Purine rich foods
34
S/s gout?
```
mono arthritis with severe joint inflammation (ankle, foot, hand, wrist elbow)
60% get podagra
trophy
radiolucent kidney stones
interstitial nephritis
```
35
Ix gout?
```
polarised light microscopy (-ve birefringent needle shaped crystals)
XR (late; para-articular punched out erosions, reduced joint space)
serum urate (increased OR normal)
```
36
Acute Mx gout?
Acute:
1st line = colchicine, NSAIDs (not aspirin) +/- PPI
renal impairment = PO steroids (prednisolone 15mg /d)
intraarticular steroid injection
follow up in 4-6w and check BP, HbA1c, serum urate, U&E and lipids -> consider urate lowering therapy
- do not stop allopurinol if already established
- do not stop aspirin 75 if for cardioprotective
37
Chronic mx for gout?
conservative: WL, avoid ETOH XS, avoid prolonged fasting
urate lowering therapy: started after inflammation stopped
- XO inhibitors: 1. allopurinol, 2. febuxostat
- uricosuric drugs: probenecid, losartan
- recombinant urate oxidase: rasbicurase
38
what are the S/S of pseudo gout?
mono arthritis (knee, wrist, hip - BIGGER JOINTS)
39
RFs for pseudo gout?
```
increased age
OA
DM
hypothyroidism
hyperparathyroidism
hereditary haemochromatosis
Wilsons
```
40
Ix pseudo gout?
polarised light microscopy (+ve birefringent, rhomboid shaped crystals)
XR chondrocalcinosis
41
Mx pseudo gout?
analgesia
NSAIDs
PO/IM/intra-articular steroids
42
what are seronegative spondyloarthropathies?
a group of inflammatory arthritidies affecting the spine and peripheral joints with no production of rheumatoid factors and associated with HLA-B27 allele
43
Give some examples of seronegative spondyloarthropathies?
```
PEAR HEADS
Psoriatic arthritis
Enteropathic arthritis
Ankylosing spondylitis
Reactive arthritis
HLA B27 allele
Enthesitis
Axial, asymmetrical, oligoarthritis
Dactylitis
Seronegative
```
44
what is the key difference between ankylosing spondylitis and psoriatic arthritis?
lack of hand S/S in AS
45
What are the S/S of ankylosing spondylitis?
```
back pain relieved by exercise
anterior chest pain (costochondritis)
SOB (pulmonary fibrosis)
morning stiffness
eye pain
osteoporosis (60%)
```
46
epidemiology of ankylosing spondylitis?
M>F (6:1); 18-25
47
Ank Spond associated features?
```
AAAAAA
anterior uveitis
apical lung fibrosis
aortic regurgitation
AV node block
achilles tenoditis
amyloidosis
```
48
Ankylosing spondylitis investigations?
```
Graded by New York Criteria
Schobers test (<5cm increase = positive)
1st: XR (late changes)
2nd: MRI (if XR normal as more sensitive)
HLA-B27 testing
ESR +/- CRP
```
49
what is seen on XR/MRI of ankylosing spondylitis?
sacroilitis > vertebrae (corner erosions, syndesmophytes)
scerlosis
ankylosis (fusion)
bamboo sign (squaring of lumbar vertebrae)
dagger sign (supraspinatus ossification)
50
what is the management of ankylosing spondylitis?
conservative: exercise/physiotherapy
medical: NSAID, anti-TNFa (etanercept), secukinumab
surgical: hip replacement to decrease pain and increase mobility
51
what % of those with psoriatic arthritis are affectedly psoriasis?
10-40%
| 40-60yo
52
S/S of psoriatic arthritis?
```
psoriasis
nail (posh = pitting, onycholysis, sublingual hyperkeratosis)
enthesitis
dactylitis
arthritis
affects DIP
```
53
what is oligoarthritis ?
<= 4 joints
54
what arthritic features are seen in psoriatic arthritis?
```
asymmetrical hligoarthritis (20-30%)
distal arthritis of the DIPJ
symmetrical poly arthritis (30-40%, RA but w DIPJ)
arthritis mutilans
spinal (50%)
```
55
what is the investigations for psoriatic arthritis?
XR: pencil in cup deformity
56
Mx psoriatic arthritis?
```
NSAIDs
methotrexate
ciclosporin
sulfasalazine
anti-TNF
```
57
what is reactive arthritis/reiters?
sterile arthritis 1-4w after urethritis or dystentery
58
what causes urethritis?
chlamydia, ureaplasma (not gonorrhoea)
59
what causes dysentery?
campylobacter, salmonella, shigella, yersinia
60
what are the S/S of reactive arthritis?
cant see, pee or climb a tree
- lower limb oligarthritis
- conjunctivitis/uveitis
- urethritis
```
keratoderma blenorrhagicum (plaques on soles/palms)
circinate balanitis (penile ulceration)
enthesitis, mouth ulcers
```
61
Ix reactive arthritis?
raised ESR
raised crp
stool culture if diarrhoea, urine chlamydia PCR
62
Mx reactive arthritis?
NSAIDs, PO steroids
| relapsing -> sulfasalazine
63
what is enteropathic arthritis?
arthritis occurs in 15% of those with UC or Crohn's
64
S/S of enteropathic arthritis?
asymmetrical lower limb oligoarthritis, sacroilitis
65
Mx enteropathic arthritis?
treat IBD
nsaids
local steroids
66
what are some autoimmune CTDs?
```
SLE
Sjogrens
Mixed CTD
systemic sclerosis
myositis
relapsing polychondritis
Behcets
```
67
Which conditions are anti rheumatoid factor +ve?
sjogrens (50%)
feltys (100%)
RhA (70%)
SLE (20%)
68
Which conditions are ANA +ve?
SLE (99%)
autoimmune hepatitis (75%)
sjogrens (70%)
RhA (30%)
69
Which conditions are anti dsDNA +ve?
SLE (70%)
70
Which conditions are anti CCP +ve?
RhA (80%)
71
Which conditions are anti histone +ve?
drug induced SLE (100%)
72
which conditions are anti centromere +ve?
CREST (limited sclerosis)
73
which conditions are ENA +ve?
```
SLE
sjogrens
MCTD
polymyositis
CREST
diffuse systemic sclerosis
```
74
which conditions are anti-Ro?
SLE, sjogrens (70%), heart block
75
which conditions are anti-La?
SLE
| sjogrens (30%)
76
which conditions are anti-smith?
SLE (30%)
77
which conditions are anti-RNP ?
SLE
| MCTD
78
which conditions are anti-jO-1?
polymyositis>dematomyositis
79
which conditions are anti Mi-2?
dermatomyositis > polymyositis
80
which conditions are anti SCL70/topoisomerase?
diffuse systemic sclerosis
81
which conditions are anti RNA pol 1,2,3?
diffuse systemic sclerosis
82
what are the indications for urate lowering therapy?
the British Society of Rheumatology Guidelines now advocate offering urate-lowering therapy to all patients after their first attack of gout
ULT is particularly recommended if:
>= 2 attacks in 12 months
tophi
renal disease
uric acid renal stones
prophylaxis if on cytotoxics or diuretics
(allopurinol 1st line)
83
what is atlantoaxial subluxation and how is it screened for?
rare complication of rheumatoid arthritis
Anteroposterior and lateral cervical spine radiographs preoperatively screen for this complication
ensuring the patient goes to surgery in a C-spine collar and the neck is not hyperextended on intubation.
84
What is hyperparathyroidism a risk factor for?
development of calcium pyrophosphate dihydrate deposition (CPPD) or pseudogout
chonedrocalcinosis
85
What is Antisynthetase syndrome?
Antisynthetase syndrome is caused by autoantibodies against aminoacyl-tRNA synthetase e.g. anti-Jo1.
It is characterised by
- myositis
- interstitial lung disease
- thickened and cracked skin of the hands (mechanic's hands)
- Raynaud's phenomenon
86
what is temporal arteritis?
Temporal arteritis is large vessel vasculitis which overlaps with polymyalgia rheumatica (PMR) (50%)
87
what is seen on histology of temporal arteritis?
Histology shows changes that characteristically 'skips' certain sections of the affected artery whilst damaging others.
88
what are the features of temporal arteritis?
typically patient > 60 years old
usually rapid onset (e.g. < 1 month)
headache (found in 85%)
jaw claudication (65%)
visual disturbances:
- amaurosis fugax
- blurring
- double vision
- 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
89
ix temporal arteritis?
raised inflammatory markers
ESR > 50 mm/hr (note ESR < 30 in 10% of patients)
CRP may also be elevated, alp raised, plts raised
USS temporal artery (halo sign, if -ve do biopsy)
temporal artery biopsy
skip lesions may be present
note creatine kinase and EMG normal
90
Mx temporal arteritis?
- urgent high-dose glucocorticoids should be given as soon as the diagnosis is suspected and before the temporal artery biopsy (40-60mg prednisolone) -> PPI AND alendronate for 2y
- no visual loss: high-dose prednisolone is used
- evolving visual loss: IV methylprednisolone is usually given prior to starting high-dose prednisolone
- urgent ophthalmology review same day, visual damage is often irreversible
other treatments:
bone protection with bisphosphonates is required as long, tapering course of steroids is required
low-dose aspirin is sometimes given to patients as well, although the evidence base supporting this is weak
91
What are the 3 patterns of disease for systemic sclerosis?
1. limited cutaneous systemic sclerosis
2. diffuse cutaneous systemic sclerosis
3. scleroderma
92
Which antibodies are present in systemic sclerosis?
ANA positive in 90%
RF positive in 30%
anti-scl-70 antibodies associated with diffuse cutaneous systemic sclerosis
anti-centromere antibodies associated with limited cutaneous systemic sclerosis
93
Where does limited cutaneous systemic sclerosis affect?
face and distal limbs predominately
94
where does diffuse cutaneous systemic sclerosis affect?
trunk and proximal limbs predominately
95
What are the adverse effects of methotrexate?
```
mucositis
myelosuppression
pneumonitis
pulmonary fibrosis
liver fibrosis
```
96
what should be monitored whilst on methotrexate?
FBC, U&E and LFTs need to be regularly monitored.
'FBC and renal and LFTs before starting treatment and repeated weekly until therapy stabilised, thereafter patients should be monitored every 2-3 months'
97
What should be co-prescribed with methotrexate
folic acid 5mg once weekly should be co-prescribed, taken more than 24 hours after methotrexate dose
98
What is antiphospholipid syndrome?
Antiphospholipid syndrome is an acquired disorder characterised by a predisposition to both venous and arterial thromboses, recurrent fetal loss and thrombocytopenia
99
What does antiphospholipid commonly occur secondary to
SLE
also:
other autoimmune disorders
lymphoproliferative disorders
phenothiazines (rare)
100
features of antiphospholipid?
```
venous/arterial thrombosis
recurrent fetal loss
livedo reticularis
thrombocytopenia
prolonged APTT
other features: pre-eclampsia, pulmonary hypertension
```
101
Mx antiphospholipid?
primary thromboprophylaxis - low dose aspirin
secondary -
- initial venous thromboembolic events: lifelong warfarin with a target INR of 2-3
- recurrent venous thromboembolic events: lifelong warfarin; if occurred whilst taking warfarin then consider adding low-dose aspirin, increase target INR to 3-4
- arterial thrombosis should be treated with lifelong warfarin with target INR 2-3
102
which autoantibody is most linked to sjogrens
anti-Ro
103
What is behcets?
rare and poorly understood inflammatory condition that mostly occurs in Turkish, Mediterranean and Japanese
104
what are the S/S of behcets?
recurrent oral and or genital ulceration
uveitis
erythema nodosum
VTE
105
Ix behcets?
skin pathergy test (pinprick - papule formation)
106
Mx behcets?
immunosuppression
107
Epidemiology sjogrens?
F>M 9:1 (40-50yo)
108
Types of sjogrens?
primary: associated with AI (thyroid, AIH, PBC), MALT lymphoma
secondary: secondary to RhA, SLE, systemic sclerosis
109
S/S sjogrens?
```
decreased tear production and dry eyes
bilateral parotid swelling
decreased salivation (xerostomia)
vaginal dryness (dyspareunia)
systemic: polyarthritis, raynaud's, bibasal pulmonary fibrosis, vasculitis, myositis
sensory polyneuropathy
```
110
Ix sjogrens?
bloods: abs (anti-ro [70%], anti La [30%], RhF [50%], hypergammaglobulinaemia
special: schirmers test, parotid biopsy
111
Mx Sjogrens?
artificial tears, saliva replacement
NSAIDS,hydroxychloroquine, immunosuppression
N.B. Abs may cause heart block in baby ->need O&G input
112
Epidemiology SLE?
0.2% 9:1 F, child bearing age, afro-carribean, asian
113
S/S SLE?
```
SOAP BRAIN MD
serositis
oral ulcers, hair loss
arthritis
photosensitivity
```
```
blood (all counts low)
renal (proteinuria, haematuria)
ANA (>95%)
Immunological (anti-dsDNA, AIHA)
neurological (psych, seizures)
```
malar rash
discoid rash
pulmonary involvement is very common with pleuritic being the most frequent manifestation
114
What are the causes of drug induced lupus?
```
Hydralazine PIMP
Hydralazine
Procainamide
Isoniazid
Minocycline
Phenytoin
```
115
S/S of drug induced lupus?
arthralgia, myalgia, recent serositis
116
Ix drug induced lupus?
anti histone Abs (100%)
117
Classification of APLS?
1st (70%)
| 2nd to SLE (30%)
118
Which antibodies are present in APLS?
anti-cardioliptin
| lupus anticoagulant
119
S/S of APLS?
```
CLOTS
Coagulation (venous and arterial thromboembolism; INCREASED APTT)
Livedo reticularis (web like rash)
Obstetric complications
Thrombocytopenia
```
120
Management of APLS?
No prev VTE -> OD low dose aspirin
| prev VTE -> OD warfarin
121
Investigations SLE/APLS?
```
Antibodies:
ANA (99% sensitive, poorly specific)
Anti-dsDNA (99% specific, 70% sensitive)
Anti-smith (99% specific, 70% sensitive)
RF (20% sensitive)
```
Disease activity monitoring:
Anti-dsDNA titres
C4 reduction (Mod active lupus), C3 reduction (very active lupus)
ESR levels
122
Management of SLE?
SEVERE FLARE - prednisolone + IV cyclophosphamide
proteinuria -> ACEi
aggressive lupus nephritis -> immunosuppression
Maintenance:
hydroxychloroquine +/- DMARDs (MMF, azathioprine) +/- low dose steroids
severe disease: biologicals (belimumb, rituximab)
sun protection: sun cream, low dose steroids
123
what is mixed connective tissue disease?
SLE, scleroderma, polymyositis, RhA
| Ix: anti-RNP
124
what is relapsing polychondritis?
an inflammatory disease of the cartilage (tenderness, inflammation and destruction of cartilage)
125
what are the associations with relapsing polychondritis?
aortic valve disease
polyarthritis
vasculitis
126
S/S relapsing polychondritis?
floppy ears
saddle nose
larynx (stridor)
127
Mx relapsing polychondritis?
immunosuppression
128
Aetiology raynaud's phenomenon?
peripheral digit ischaemia precipitated by cold or emotion
129
Classification of raynauds?
primary: idiopathic, Raynaud's disease
secondary: Raynaud's syndrome
- rheumatological (systemic sclerosis, SLE, RhA, sjogrens)
- haematological (thrombocytosis, pv)
- drugs (COCP, non cardio selective beta blockers)
- congenital (cervical rib -> thoracic outflow syndrome)
130
S/S raynauds?
digit pain, triphasic colour change, digital ulceration and gangrene
131
Mx raynauds?
gloves/avoid cold
CCB (nifedipine)
PDE V inhibitor (sildenafil)
IV prostacyclin
132
what are the types of systemic sclerosis
1. Limited systemic sclerosis (crest = 70%)
| 2. diffuse systemic sclerosis (30%)
133
What is CREST?
```
Calcinosis
Raynaud's
Oesophageal dysmotility
Sclerodactyly
Telangiectasia
```
```
Skin involvement (beak nose, microstomia, pul HTN)
limited to face, hands, feet
```
134
what is diffuse systemic sclerosis?
diffuse skin involvement extends past the wrists and up the arms
135
What are the features of diffuse systemic sclerosis?
organ fibrosis
GI: GOR, aspiration, dysphagia, anal incontinence
lung 80%: fibrosis, pul HTN
cardiac: arrhythmias, conduction defects
renal: acute HTN crisis (most common cause of death)
136
Ix Systemic sclerosis?
bedside: urine dip, PCR
bloods: FBC, U&Es, abs
limited CREST -> anti-centromere
diffuse -> anti-SCL70, anti RNA polymerase 1,2,3
imaging:
CXR - cardiomegaly, bibasal fibrosis
Hands - calcinosis
Ba swallow - impaired oesophageal motility
HRCT - fibrosis
Echo - fibrosis
ECG - pulmonary HTN, conduction blocks, arrhythmias
137
Mx systemic sclerosis?
Conservative: exercise, skin lubricants, hand warmers
M: immunosuppression
Renal crisis - intensive BP control (ACEi)
oesophageal - PPI, pro kinetics
PHT: sildenafil, bosentan
138
what is polymyositis?
striated muscle inflammation
139
S/S myositis?
progressive symmetrical proximal muscle weakness (associated myalgia and arthralgia)
wasting of shoulder and pelvic girlde
dysphagia, dysphonia, respiratory weakness
malignancy- paraneoplastic phenomenon (lung, pancreas, ovarian, bowel)
140
what are the features of dermatomyositis?
myositis + skin signs
- periorbital heliotrope rash on eyelids +/- oedema
- Gottron's papules on knuckles, elbows, knees
- mechanics hands
- macular rash (shawl sign +ve: over back and shoulders)
- nail fold erythema
- retinopathy (haemorrhages and cotton wool spots)
- subcutaneous calcifications
141
What are the extra articular features of dermatomyositis?
```
fever
arthritis
basal pulmonary fibrosis
raynaud's phenomenon
myocardial involvement: myocarditis, arrhythmias
```
142
Ix myositis?
EMG
Muscle enzymes: Raised CK (in 1000s), raised AST, ALT and LDH
Antibodies: anti-Jo1, Anti-Mi2 [DM>PM], Anti-SRP [PM]
Malignancy screen (tumour markers, CXR, mammogram, USS, CT)
143
Mx myositis?
Screen for malignancy
| immunosuppression (steroids, cytotoxic agents: azathioprine, methotrexate)
144
which criteria are used to classify vasculitides?
Chapel hill criteria
145
Give some large vessel vasculitis?
GCA/temporal arteritis
| Takayasu arteritis
146
Give some medium vessel vasculitis?
Polyarteritis nodosa
| Kawasaki disease
147
Give some small vessel vasculitis?
p-ANCA: Churg-Strauss (eGPA), microscopic polyangitis
cANCA: Wegener's granulomatosia (GPA)
ANCA -ve: HSP, good pasture's, cryoglobulinaemia
148
epidemiology of PMR?
>50yo, association to GCA
149
Which arteries are affected in polymyalgia rheumatica?
temporal
maxillary
ophthalmic
central retinal
150
what are the S/S of pMR?
pain/stiffness in shoulder, neck and hips (no weakness)
| polyarthritis, tenosynovitis, CTS
151
Ix PMR?
raised ESR, CRP, ALP, normal CK
152
Mx PMR?
15 mg PO prednisolone
| taper down to 5 mg + PPI + alendronate
153
What is the epidemiology of takayasu arteritis?
Japanese/asian females
| 20-40yo (granulomatous vasculitis similar to GPA/eGPA)
154
S/S Takayasu arteritis?
```
weak/unequal upper limb pulses
HTN
Large vessel blockage - COMMONLY AORTA (HF, CRF)
Intermittent limb claudication
AR (20%)
```
155
mx takayasu?
corticosteroids
156
Epidemiology polyarteritis nodosa?
young male adults (not common in UK)
157
s/s polyarteritis nodosa?
```
systemic symptoms
skin (rash)
GIT (melaena, abdo pain)
renal (HTN)
liver dysfunction (HBV)
```
158
Ix polyarteritis nodosa?
HBV serology, rosary bead sign on renal angiogram
159
Mx polyarteritis nodosa?
prednisolone and cyclophosphamide
160
Epidemiology Kawasaki?
6m-5yo, 8/100000, Japanese and Black Caribbean ethnicity
161
S/S kawasaki?
```
CRASH and BURN
Conjunctivitis
Rash
Adenopathy
Strawberry tongue
Hands/feet swollen
Fever >5 days
```
162
Ix Kawasaki disease?
clinical (echocardiogram done in OPD)
163
Mx Kawasaki?
IVIG and high dose aspirin
164
What are some ANCA +ve conditions?
GPA/wegeners
eGPA (Churg-Strauss)
Microscopic polyangitis
165
what are some ANCA negative conditions?
HSP
Goodpastures
Cryoglobulinaemia
166
what are the S/S of wegeners/GPA?
URT - rhinitis, epistaxis, saddle nose
LRT - haemoptysis, cough
Renal - rapidly progressive glomerulonephritis (RPGN), nephritic syndrome
167
what are the Ix for GPA?
cANCA (pr3)
dipstick (p+, h+)
CXR (nodules)
168
what is eGPA/Churg Strauss?
rare disease occurs in atopic individuals
169
What are the S/S of eGPA?
eosinophilia
asthma (late onset)
vasculitis (incl. RPGN)
170
What are the Ix for eGPA?
pANCA (MPO n.b. also +ve in UC/TB)
171
What are the S/S of microscopic polyangitis?
RPGN
palpable purpura
haemoptysis
172
Ix for microscopic polyangitis?
pANCA (MPO)
173
What are the features of HSP?
Most common childhood vasculitis
| often preceded by URTI (2-3 days)
174
S/S HSP?
```
purpuric rash (urticarial, maculopapular, spares trunk)
arthralgia (and periarticular oedema, large joints)
Abdominal pain (haematemesis, melena, intussusception)
Glomerulonephritis (micro/macroscopic haematuria, nephrotic syndrome (rare))
```
175
Ix hsp?
Urinalysis (RBCs, protein, casts)
| FBC, clotting screen, U&E,
176
Mx HSP?
```
follow up (weekly for 1m, 2 weekly for 2m, 3m, 6m, 12m)
BP measurements
Urine dip (haematuria)
Most cases will resolve spontaneously within 4 weeks
```
177
what is good pastures?
antiGBM
178
What are the S/S of good pastures?
RPGN
| haemoptysis
179
what are the investigations for good pastures?
Anti-gbm antibody
| CXR (bilateral lower zone infiltrates)
180
Mx goodpastures?
immunosuppression + plasmapheresis
181
What is simple cryoglobulinaemia?
```
Monoclonal IgM -hyperviscosity:
visual disturbances
bleeding from mucous membranes
thrombosis
headaches and seizures
```
182
What is mixed cryoglobulinaemia?
```
Polyclonal IgM - immune complex mediated disease
GN
palpable purpura
arthralgia
peripheral neuropathy
```
183
aetiology of simple cryoglobulinaemia?
20%
| secondary to: myeloma, CLL, Waldenstroms macroglobulinaemia
184
aetiology of mixed cryoglobulinaemia?
```
80%
secondary to SLE
sjogrens
HCV
mycoplasma
```
185
epidemiology of fibromyalgia?
10% of new rheumatology referrals
| F>M (10:1)
186
RFs fibromyalgia?
```
neurosis (depression, anxiety, stress)
work dissatisfaction
overprotective family/lack of support
middle age
low income
divorced
low educational status
```
187
Associations fibromyalgia?
chronic fatigue syndrome
IBS
chronic headache syndrome
188
S/S fibromyalgia?
```
chronic widespread musculoskeletal pain and tenderness
fatigue
sleep disturbance
morning stiffness
poor concentration
low mood
```
189
Ix fibromyalgia
all normal
190
Mx fibromyalgia?
educate
CBT
graded exercise programmes
amitriptyline, pregabalin, venlafaxine
191
Management of chronic pain?
all diabetics get peripheral neuropathy
| Amitriptyline Duloxetine Gabapentin Pregabalin
192
1st line for neuropathic pain?
amitriptyline, pregabalin
193
1st line for diabetic neuropathy?
duloxetine
| tramadol as rescue therapy
194
1st line for trigeminal neuralgia?
carbamazepine
195
Epidemiology still's disease?
15-25yo, 35-45 yo
196
S/S stills disease?
Arthralgia
Salmon pink rash (maculopapular)
Pyrexia (rises in late evening in daily pattern alongside arthralgia)
197
Investigations Stills disease?
RhF, ANA -ve
Raised ferritin
Yamaguchi criteria (sensitivity of 93.5%)
198
Management of Still's disease?
1st line: NSAIDs (for fever, joint pain, serositis)
2nd line: after 1 week of NSAIDs + steroids
3rd line: methotrexate, IL-1, anti-TNF
199
Epidemiology of pagets?
Paget's disease is common (UK prevalence 5%) but symptomatic in only 1 in 20 patients.
200
which bones are affected in Paget's?
The skull, spine/pelvis, and long bones of the lower extremities
201
Paget's RF?
increasing age
male sex
northern latitude
family history
202
Clinical features of Paget's?
```
only 5% of patients are symptomatic
the stereotypical presentation is an older male with bone pain and an isolated raised ALP
bone pain (e.g. pelvis, lumbar spine, femur)
classical, untreated features: bowing of tibia, bossing of skull
```
203
Ix pagets?
bloods:
raised alkaline phosphatase (ALP)
calcium and phosphate are typically normal. Hypercalcaemia may occasionally occur with prolonged immobilisation
```
other markers of bone turnover include:
procollagen type I N-terminal propeptide (PINP)
serum C-telopeptide (CTx)
urinary N-telopeptide (NTx)
urinary hydroxyproline
```
x-rays:
osteolysis in early disease → mixed lytic/sclerotic lesions later
skull x-ray: thickened vault, osteoporosis circumscripta
bone scintigraphy:
increased uptake is seen focally at the sites of active bone lesions
204
Management of paget's?
```
indications for treatment include:
bone pain
skull or long bone deformity
fracture
periarticular Paget's
```
bisphosphonate (either oral risedronate or IV zoledronate)
calcitonin is less commonly used now
205
Complications of Paget's?
```
deafness (cranial nerve entrapment)
bone sarcoma (1% if affected for > 10 years)
fractures
skull thickening
high-output cardiac failure
```
206
What is Marfan's and what is it caused by?
Marfan's syndrome is an autosomal dominant connective tissue disorder
defect in the FBN1 gene on chromosome 15 that codes for the protein fibrillin-1
207
What are the features of Marfan's?
tall stature with arm span to height ratio > 1.05
high-arched palate
arachnodactyly
pectus excavatum
pes planus
scoliosis of > 20 degrees
heart: dilation of the aortic sinuses (seen in 90%) which may lead to aortic aneurysm, aortic dissection, aortic regurgitation, mitral valve prolapse (75%),
lungs: repeated pneumothoraces
eyes: upwards lens dislocation (superotemporal ectopia lentis), blue sclera, myopia
dural ectasia (ballooning of the dural sac at the lumbosacral level)
208
leading causes of death in marfan's?
aortic dissection
209
Mx Marfan's?
regular echocardiography monitoring and beta-blocker/ACE-inhibitor therapy
210
Blood results in osteogenesis imperfect?
Adjusted calcium, PTH, ALP and PO4 results are usually NORMAL in osteogenesis imperfecta
211
Features of osteogenesis imperfecta?
```
presents in childhood
fractures following minor trauma
blue sclera
deafness secondary to otosclerosis
dental imperfections are common
```
212
What is the management for secondary prevention for osteoporotic fractures in post menopausal women?
start a bisphosphonate before waiting for a DEXA
- vitamin D and calcium supplementation should be offered to all women unless the clinician is confident they have adequate calcium intake and are vitamin D replete
- alendronate is first-line
- around 25% of patients cannot tolerate alendronate, usually due to upper gastrointestinal problems. These patients should be offered risedronate or etidronate
- strontium ranelate and raloxifene are recommended if patients cannot tolerate bisphosphonates
213
Interpretation of DEXA?
```
T = no of SDs away from youthful average
Z = no of SDs away from age matched average
```
T>-1 = normal
T-1 - -2.5 = osteopenia
T
214
what is ANA antibody seen in?
(SSSA)
systemic lupus erythematous (SLE)
systemic sclerosis
autoimmune hepatitis
Sjogren's syndrome
215
What is AMA antibody raised in?
primary biliary cholangitis
autoimmune hepatitis
idiopathic cirrhosis
216
What is anti-GBM seen in?
Good pastures
217
What is pANCA seen in?
eosinophilic granulomatosis with polyangiitis
microscopic polyangiitis
Goodpasture's disease.
218
Features of rheumatoid arthritis?
```
LESS
Loss of joint space
Erosions
Subchondral Cysts
Subchondral sclerosis
```
ie erosions differentiate from rheumatoid
219
What is HLA-B27 associated with?
Reactive arthritis
Ankylosing spondylitis
seronegative spondyloarthropathies
220
What HLA is behcets associated with?
HLA-B51
221
What is HLA-DQ2 associated with?
coeliac
222
what is HLA-DR3 associated with?
```
Addison's disease
systemic lupus erythematosus
type 1 diabetes mellitus
Grave's disease
myasthenia gravis
dermatitis herpetiformis
Sjogren's syndrome
primary biliary cirrhosis
```
223
What is HLA-DR1 associated with?
T1DM
| RA
