Rheumatology Flashcards
(152 cards)
1
Q
What are the extra-articular manifestations of ankylosing spondylitis?
A
- acute iritis
- aortic regurgitation
- apical lung fibrosis
2
Q
What is the most specific antibody for rheumatoid arthritis ?
A
anti-CCP
= anticyclic citrullinated peptide Ab
3
Q
What is Felty’s?
A
- rheumatoid arthritis
- neutropenia
- splenomegaly
4
Q
What antibody would you find in Felty’s?
A
Rheumatiod factor (RhF)
5
Q
What joints are predominantly involved in RA?
A
- MCP
- PIP
- wrist
6
Q
What are the xray signs of RA?
A
‘SOLD’
S - soft tissue swelling
O - osteopenia (peri-articular)
L - loss of joint space
D - deformity
7
Q
What is DAS28 and what does it measure?
A
- disease activity score for RA which looks at 28 joints
- analyses 4 things:
- joint involvement
- serology
- acute phase reactants
- duration of symptoms
8
Q
What are the red flags for RA?
A
- atlantoaxial subluxation
- pericarditis
- monoarticular flare (think septic arthritis)
- eye involvement (scleritis)
9
Q
What are the classic hand signs in RA?
A
- boutonniere deformity
- swan neck deformity
- ulnar deviation of MCP joints
- Z deformity of thumb
- wrist subluxation
10
Q
What is MTX?
A
dihydrofolate reductase inhibitor (anti-folic acid)
11
Q
List DMARDs.
A
- gold
- MTX
- sulfasalazine
- hydroxychloroquine
- leflunomide
- penicillamine
- azathioprine
- cyclophosphamide
- ciclosporin
- tacrolimus
- mycophenylate mofetil
- minocycline
12
Q
List the TNF alpha inhibitors.
A
- infliximab
- etanercept
- adalimumab
- certolizumab
- golimumab
- -> approved by NICE as 1st line biologic therapy after active failure to respond to 2 DMARDs
- -> usually used in combo with MTX
13
Q
What biologic works by B cell depletion?
A
rituximab = anti-CD20
14
Q
What biologic inhibits IL-6?
A
tocilizumab
15
Q
What biologic disrupts T cell function?
A
abatacept = anti CD80 & CD86
16
Q
What biologic inhibits IL-1?
A
anakinra
- -> short half life means you need daily injections
- -> not recommended for treatment of RA
17
Q
What are the SEs of MTX?
A
- teratogenic
- pancytopenia
- pneumonitis
- oral ulcers
- lymphoproliferative disorders
- increased susceptibility to infections
18
Q
List 2 new small molecule DMARDs.
A
- baracitinib
- tofacitinib
–> JAk-STAT pathways targeted
19
Q
What joints are typically affected in OA?
A
- knees
- hips
- thumb bases
- finger interphalangeal joints
20
Q
What are the risk factors for OA?
A
- age
- female sex
- obesity
- joint injury
21
Q
What are the xray findings in OA?
A
‘LOSS’
L - loss of joint space
O - osteophytes
S - subchondral cysts
S - subarticular sclerosis
22
Q
What are Heberden’s nodes?
A
bony enlargements of the distal interphalangeal joints
23
Q
What are the pathways leading to OA?
A
- abnormal stress
- obesity
- trauma
- abnormal anatomy
- altered joint loading - abnormal joint physiology
- genetic factors
- ageing
- inflammation and sepsis
–> lead to joint damage, pain and disability
24
Q
What are the main symptoms of OA?
A
- pain
- stiffness
- reduced range of movement
25
What would you suspect in an OA patient presenting with erythema/acute painful effusion?
co-existent crystal synovitis
--> cartilage matrix changes in OA to promote calcium pyrophosphate, basic calcium phosphate and monosodium urate crystal deposition
26
What are Bouchard's nodes?
bony enlargements of the proximal interphalangeal joints
27
What blood results in RA are suggestive of widespread synovitis?
- raised ESR
- raised CRP
- raised platelets
- raised serum ferritin
28
Why do patients with RA get carpel tunnel syndrome?
secondary to inflammation of the flexor tendon sheath
29
What is the common causative organism in septic arthritis?
staphylococcus aureus
30
What is the most common joint affected by septic arthritis?
knee
31
What is the main differential diagnosis of septic arthritis?
crystal arthopathies
32
How does infection reach a joint leading to septic arthritis?
1. haematogenous route
2. direct spread from the metaphysis
3. penetrating trauma/surgery
33
What is the most likely causative organism of septic arthritis in young sexually active individuals?
Neisseria gonorrhoea
34
What is ankylosis?
bony fusion across the joint
35
What are the complications of septic arthritis?
1. seeding of infection into spine/other orgnas
2. recurrence of infection
3. long-term joint damage - arthritis/ankylosis
4. avascular necrosis (esp. of the hip)
36
What is a podagra?
gout of the metotarsalphalangeal joint
37
What is the main differential to rule out with acute gout?
septic arthritis
38
What do you see in polarised light microscopy in gout?
negatively birefringent needle shaped urate crystals
39
What do you see in polarised light microscopy in pseudo-gout?
positively birefringent rhomboid shaped calcium pyrophosphate dehydrate crystals
40
What are the RFs for gout?
1. diet
2. obesity
3. age and sex
4. alcohol
5. drugs (thiazide diuretics)
41
What are tophi?
deposits of urate crystals in the skin embedded in a matrix of lipid, protein and calcific debris
--> can contribute to destructive arthropathy or secondary OA
42
What are the characteristic gout changes on xray?
soft tissue swelling
subcortical cysts
geode formation ('punched out lesions')
43
What conditions are associated with childhood gout?
- Lesch-Nyhan syndrome
| - G6PD deficiency
44
Which NSAID would you prescribe for gout?
indomethacin
45
What are the side effects of colchicine?
- severe diarrhoea
- bone marrow dysfunction
- neuromuscular dysfunction
46
What is the therapeutic target of plasma urate for prophylactic treatment of gout?
<0.36mmol/L
OR
if tophi present <0.30mmol/L
47
What is the criteria for prophylactic treatment of gout?
2 or more attacks of gout per year
48
What are the prophylactic treatment options for gout?
1. allopurinol
- reduces serum urate
- typical dose 300mg OD
2. febuxostat
- xanthine oxidase inhibitor
- 80mg OD
- prevents purine breakdown products of xanthine and hypoxanthine being converted into urate
49
Which joints are typically affected by pseudogout?
- knees
- ankles
- elbows
- hands
- wrists
- shoulders
50
What is pseudo-gout associated with/RFs?
1. hypothyroidism
2. hypomagnesium
3. hypercalcaemia
4. hereditary haemochromatosis
51
What is seen on xray with pseudo-gout?
soft tissue calcium deposits = chondrocalcinosis
52
How do you treat pseudo-gout?
acute episode - NSAIDs or intra-articular steroids
| chronic episode - long-term NSAIDS or colchicine
53
List the spondylarthritides.
'PEAR'
P - psoriatic arthritis
E - enteric arthropathy
A - ankylosing spondylitis
R - reactive arthritis
also: undifferentiated spondyloarthritis and childhood spondyloarthritis
- -> inflammatory joint disease of the vertebral column and sacro-iliac joints
- -> mimic rheumatoid conditions but serologically different (usually RhF -ve)
- -> strong association with HLA B27
54
What is enthesitis?
inflammation at site of insertion of tendon/ligament into bone
55
What is dactylitis?
inflammation of an entire digit due to soft tissue oedema and tenosynovial and joint inflammation
56
What is the common MHC II subtype in the spondyloarthritides?
HLA B27
--> tests for RhF, anti-CCP and other autoantibodies are negative
57
What conditions are associated with ankylosing spondylitis?
extra-articular:
1. osteoporosis (predisposes to spinal fractures)
2. aortic valve incompetence
3. pulmonary apical fibrosis
4. enthesitis (achilles tendon bursitis and plantar fascia)
5. acute anterior uveitis (pain+photophobia)
6. dactylitis affecting toe (sausage toe)
58
What is schoeber's test?
- assess flexion of the lumbar spine
- mark at level of posterior superior iliac spine and 10cm above
- on forward flexion this should increase to 15cm
- good test for ankylosing spondylitis
59
What factors worsen the prognosis of ankylosing spondylitis?
1. disease onset <16y/o
2. ESR >30
3. males
4. smoking
5. HLA 27 +ve
6. early hip involvement
7. syndesmophytes at presentation
8. poor response to NSAIDs
60
What are the typical presenting complaints for patients with AS?
1. young male
2. insidious onset back pain lasting >3 months
3. pain awakens pt from sleep
4. accompanied early morning stiffness
5. stiffness improved with exercise (big part of Mx)
6. pain radiates from sacroiliac joints to hips/buttocks
61
What are the xray findings in AS?
early: sacroiliitis and squaring of the vertebral bodies
later: syndesmophytes, facet joint fusions
'bamboo' spine appearance
62
How can AS be assessed?
1. The Bath ankylosing spondylitis disease activity index
- assesses disease activity
2. The Bath ankylosing spondylitis functional index
- assess over patient function
3. The Bath ankylosing spondylitis metrology index
- assesses spinal mobility
63
What are the shared features of the spondyloarthritides?
1. seronegative
2. HLA B27 association
3. axial arthritis; spine and sacroiliac joints affected
4. enthesitis
5. dactylitis
6. asymmetrical large joint oligoarthritis/monoarthritis
7. extra-articular manifestations:
- iritis
- psoriasis
- aortic valve incompetence
- oral ulcers
- IBD
64
What medication is indicated in severe AS?
TNF alpha blockers
- etanercept
- adalimumab
- golimumab
65
What is arthritis mutilans and what spondyloarthitidy is it seen in?
psoriatic arthritis
osteolysis leads to destruction of the small joints of the digits with shortening
66
What is onycholisis?
painless detachment of nail from the nail bed
67
What are the nail changes seen with psoriatic arthritis?
1. pitting
2. onycholysis
3. hyper-keratosis
68
What is the classic xray finding in psoriatic arthritis?
'pencil-in-cup' deformity in severe cases
69
What is Reiter's syndrome?
1. reactive arthritis
2. conjunctivitis (usually bilateral)
3. urethritis
(can't see, can't pee, can't bend your knee!)
70
What are the common causative organisms of reactive arthritis?
GU
- chlamydia trachomatis
GI
- campylobacter jejuni
- salmonella typhimurium
- Yersinia enterocolitica
- shigella flexneri
71
What is the most important differential of reactive arthritis?
septic arthritis
72
What are the clinical features of reactive arthritis?
1. acute asymmetrical oligoarthritis affecting large joints e.g. ankle and knee
2. painless mouth ulcers
3. keratoderma blennorrhagia & circinate balanitis
73
What conditions is enteropathic arthritis associated with?
1. inflammatory bowel disease
- Crohn's
- Ulcerative colitis
2. coeliac disease
3. Whipple's disease
74
How do you treat sacroiliitis and spondylitis?
first-line:
- regular physio and exercise
- NSAIDS e.g. naproxen/diclofenac
- COX-2 inhibitors e..g etoricoxib
second line:
- PO/IM corticosteroids
third-line:
- anti TNF alpha (etanercept, adalimumab, golimumab) and anti-IL17
adjunctive
- bisphosphonates, calcium and vitamin D supplements to improve bone density
- low dose amitriptyline at night for pain
75
Why should NSAIDs be used with caution in enteropathic arthropathy?
they may worsen gut symptoms
76
What is the treatment of oligoarthritis and/or enthesitis?
first-line:
- NSAIDs
- intraarticular/intra-lesion corticosteroid injections
second-line:
- DMARDs e.g. MTX
77
What is SLE?
systemic lupus erythematous
| = a multisystem autoimmune disease associated with genetic and environmental risk factors
78
What is the pneumonic for SLE?
'SOAP BRAIN MD'
Serositis - pleuritis and pericarditis
Oral ulcers - painless
Arthirits - non-erosive involving 2 or more joints
Photosensitivity
Bloods - all low; thrombocytopenia, haemolytic anaemia (Coomb's +ve), leukopenia, neutropenia, lymphopenia (most common manifestation other than +ve ANA)
Renal - lupus nephritis with proteinuria and red cell casts
ANA +ve
Immunology - anti-dsDNA, anti-Sm, antiphospholipid antibody positive
Neurology - seizures and psychosis
Malar rash
Discoid rash
79
What HLA associations are there with SLE?
HLA B8, DR2, DR3
80
What are the 3 best tests for SLE?
1. anti-dsDNA
2. complement - low C3 and low C4
- they fall in a flare due to complement consumption - particularly in patients with renal disease
3. ESR raised - CRP normal
81
Which viral infections are associated with SLE?
EBV and CMV
82
What is Jacoud's arthropathy?
ligamentous laxity seen in SLE patients
83
What is neonatal lupus?
syndrome occurring in 10% babies born to mothers with anti-Ro/anti-La antibodies
PC: rash induced by UV light
84
How do you treat severe flares of SLE?
IV cyclophosphamide + high dose prednisolone
85
What is the maintenance therapy for SLE?
NSAIDs + hydroxychloroquine (compatible with pregnancy and breastfeeding and may even improve outcomes)
86
How do you treat lupus nephritis?
mycophenolate mofetil
87
What conditions is Sjogren's associated with?
- thyroid disease
- autoimmune hepatitis
- primary biliary cirrhosis
- increased risk of non-Hodgkin's B cell lymphoma
88
What clinical syndrome is characterised by sicca symptoms and what are these?
- Sjogren's syndrome
| - sicca symptoms: dry eyes, dry mouth (xerostomia), preceded by parotid swelling
89
In which conditions might Sjogren's develop as a secondary disorder?
- RA
- SLE
- systemic sclerosis
- PBC
90
What is Schirmer's test?
measures conjunctival dryness
| <5mm in 5 minutes is positive
91
What autoantibodies are associated with Sjogren's syndrome?
- Anti-Ro
- Anti-La
- RhF
- ANA
92
What blood results are indicative of Sjogren's?
- v high total IgG levels (hypergammaglobulinaemia)
| - raised ESR
93
How do you treat Sjogren's?
- arthralgia: hydroxychloroquine + NSAIDs
- pilocarpine
- artificial tears: hypromellose
94
Describe polymyositis and dermatomyositis.
rare conditions characterised by insidious onset of progressive symmetrical proximal muscle weakness and auto-immune mediated striated muscle inflammation
associated with myalgia and arthralgia
+/- derm manifestations
95
What is the diagnostic criteria for polymyositis?
1. proximal muscle weakness
2. elevated muscle enzymes - ALT, AST, LDH, CK, aldolase
3. myopathic changes on muscle biopsy
4. EMG - classic fibrillation potentials
96
What are the characteristic skin signs in dermatomyositis?
1. shawl sign
- photosensitive macular rash over back and shoulders
2. heliotrope rash
- lilac purple rash on eyelids +/- oedema
3. Gottron's papules
97
What autoantibodies are associated with polymyositis and dermatomyositis?
1. anti-Mi2
| 2. anti-Jo1 (associated with acute onset and interstitial lung fibrosis)
98
How do you treat poly-/dermato-myositis?
- screen/rule out malignancy
- prednisolone
- DMARDs in resistant cases
- hydroxychloroquine and topical tacrolimus for skin manifestations
99
What is myositis?
inflammation of the muscle
100
What is polymyositis?
inflammation affecting many muscles especially shoulders, thighs and hips
101
What is dermatomyositis?
muscle inflammation with particular skin rashes
102
What do anti-phospholipid antibodies cause?
'CLOTS'
C - coagulation defects
L - livedo reticularis
O - obstetric - recurrent miscarriages in 2nd and 3rd trimester
T - thrombocytopenia
103
What antibodies are found in antiphospholipid syndrome?
'antiphospholipid antibodies'
= anticardiolipin antibodies and beta-2-glycoprotein
only 20% have lupus coagulant
104
What antibody is found in drug-induced lupus?
antihistone antibodies
105
List some examples of drugs which cause drug-induced lupus.
- isoniazid
- hydralazine
- procainamide
- quinidine
106
What are the clinical features of limited cutaneous systemic sclerosis?
formerly CREST syndrome:
```
C - calcinosis of subcutaneous tissue
R - Raynaud's phenomenon
E - oesophageal and gut dysmotility
S - sclerodactyly
T - telangiectasia (on face and digits)
```
- -> skin involvement limited to hands, face and feet
- -> 40% also affected by interstitial lung disease
107
How do you treat Raynauds?
1. physical protection e.g. gloves
| 2. CCB e.g. nifedipine
108
What antibodies are associated with limited cutaneous systemic sclerosis?
anticentromere antibodies
- signify a risk of pulmonary HTN but not fibrosis
- associated with a relatively good prognosis
109
What is Raynaud's phenomenon?
peripheral digit ischaemia due to paroxysmal vasospasm, precipitated by cold/emotion
110
What is scleroderma?
hardening of the skin
111
What is the most frequent cause of death in systemic sclerosis?
pulmonary disease
112
What antibodies are associated with diffuse cutaneous systemic sclerosis?
antitopoisomerase-1 (Scl-70)
113
What do patients with systemic sclerosis typically present with?
1. beak-like nose
| 2. microstomia (small mouth)
114
Systemic sclerosis patients at risk of lung fibrosis should be treated with what?
cyclophosphamide
115
List the 2 main autosomal dominant connective tissue disorders.
1. Ehler danlos syndrome
| 2. Marfan's syndrome
116
What are cANCA wtih proteinase 3 antibodies associated with?
Granulomatosis with polyangiitis (GPA)
117
What are pANCA with myeloperoxidase antibodies associated with?
microscopic polyangiitis
118
List the 2 main large vessel vasculidites.
1. Takayasu's
| 2. Giant cell arteritis
119
List the 2 main medium vessel vasculidites.
1. polyarteritis nodosa
| 2. Kawasaki's disease
120
Give some examples of small vessel vasculidites.
ANCA negative:
- Goodpasture's (anti-GBM)
- HSP (IgA vasculitis)
ANCA positive:
- Granulomatosis with polyangiitis (cANCA)
- Churg strauss (eosinophilic granulomatosis with polyangiitis)
- microscopic polyangiitis
121
List some causes of secondary vasculitis.
- RA
- SLE
- infection: hep B and C, HIV
122
What is the maintenance therapy for systemic vasculitis?
azathioprine/MTX + glucocorticoids
123
What infection is polyarteritis nodosa associated with?
hepatitis B
124
What is the treatment of Kawasaki disease?
- immunoglobulins
| - aspirin
125
What investigations should be done for systemic vasculitis?
1. urine analysis
- most important investigation
- severity of renal involvement key determinant of prognosis
2. blood tests
- leucocytosis suggests primary cause
- eosinophils suggest churg strauss/drug reaction
3. LFTs
- abnormal suggest viral infection
4. immunology
- cANCA/pANCA
- RhF and ANA suggestive of CTD cause
5. biopsy
- confirm diagnosis
6. other
- echo for kawasaki/exclude infection
126
What is microscopic polyangitis?
- vasculitis that commonly affects the kidneys
- biopsy shows focal segmental necrotizing glomerulonephritis with some immune deposits (pauciimmune)
- lung disease presents with haemoptysis
127
What is Behcet's?
- systemic vasculitis
- unknown aetiology
- characterised by orogenital ulceration
- common in Turkey and Japan
- ocular involvement early on (affects 50%)
128
What are the clinical features of polyarteritis nodosa?
- constitutional illness
- rash
- mononeuritis multiplex
- vascular hypertension
- organ infarction
129
What are the major toxicities of cyclophosphamide?
- haemorrhagic cystitis
- formation of bladder tumours
- infertility
- infection
130
What condition frequently occurs with GCA?
polymyalgia rheumatica
131
Describe polymyalgia rheumatica.
It is characterised by bilateral pain and stiffness of the hips and shoulders +/- mild polyarthritis, tenosynovitis and carpel tunnel syndrome
typically associated with elevated inflammatory markers
132
What distinguishes polymyalgia rheumatica from myositis/myopathies?
creatinine kinase levels in polymyalgia rheumatica are normal
133
What are the differential diagnoses of polymyalgia rheumatica?
1. inflammatory rheum disorders:
- RA - GCA
2. non-inflamm rheum:
- OA - fibromyalgia
3. infection
- bac endocarditis
4. malignancy
- leukaemia
5. endocrine
- hypothyroidism
6. other
- drug induced e,g, statins
134
What is the gold standard diagnostic test for GCA?
temporal artery biopsy
- lymphocytic and macrophage infiltrate with the presence of giant and epithelioid cells
Rx should not be delayed for the sake of obtaining a biopsy
- temporal artery USS may be a useful adjunct
135
What is the mainstay treatment for both PMR and GCA?
oral gulcocorticoids
- lower for PMR which should be titrated down over weeks-months
- higher for GCA 30-40mg or 60mg if jaw claudication
136
What are the side-effects of steroids?
- weight gain
- bruising
- infections
- glaucoma
- myopathy
- osteoporosis
- diabetes
- HTN
- depression
- dyspepsia
137
What blood results would you expect in GCA?
- raised ESR (>50)
- new headache
- temporal artery tenderness/ decreased pulsation
- abnormal artery biopsy
138
What are the differential diagnoses of GCA?
- migraine
- tension headache
- trigeminal neuralgia
- takayasu's arteritis
- polyarteritis nodosa
139
What is the childhood polarteritis nodosa variant?
Kawasaki's
140
What is polyarteritis nodosa?
A necrotising vasculitis that causes aneurysms and thrombosis in medium sized arteries
typically affects SKIN (rash with 'punched out' ulcers' and RENAL (main cause of death)
141
How do you treat polyarteritis nodosa?
corticosteroids and cyclophosphamide
treat hep B with antivirals
142
What are the speculated trigger factors for fibromyalgia?
- disruption of sleep pattern
- emotional stress
- systemic disease/infection
- anxiety/depression
143
What other somatic syndromes is fibromyalgia associated with?
- chronic fatigue syndrome
- IBS
- chronic headache syndromes
144
What are the 2 key features of fibromyalgia?
1. allodynia:
- pain in response to a non-painful stimulus
2. hyperaesthesia:
- exaggerated perception of pain in response to a mild stimulus
- CSF of these patients show increased levels of substance P
- PET imaging suggests abnormal central dopamine response to pain
145
What are the diagnostic features of fibromyalgia?
1. widespread pain
2. chronic pain (>3mths)
3. absence of inflammation
4. PLUS presence of pain on palpation of at least 11/18 tender points
146
What is the management of fibromyalgia?
AIM: to optimise physical, psychological and social functioning
1. education
2. graded exercise
3. pacing activities (breaking down everyday activities into small realistic goals)
4. psychotherapeutic interventions (CBT, midfulness-based treatment and relaxation therapy)
5. limited drug therapy (amitryptalline(10-50mg)/pregabalin (150-450mg) may improve pain and sleep)
147
List the physical, psychological and cognitive symptoms of fibromyalgia.
1. physical
- urinary frequency and urgency
- migraine
- IBS
- muscle spasm
- paraesthesia
- dizziness
- temp change
2. psychological
- panic attacks
- anxiety
- depression
3. cognitive:
- memory lapses
- word mix-ups
- reduced concentration
148
Define JIA.
chronic arthritis of unknown aetiology, lasting at least 6 weeks with an onset before 16y/o. The onset type is defined by type of disease in first 6months
149
What are the 7 subgroups of JIA?
1. systemic
2. oligoarthritis (persistent/extended)
3. polyarthritis RhF -ve
4. polyarthritis RhF +ve
5. psoriatic arthritis
6. enthesitis-related arthritis
7. indifferentiated (fits no other category/fits more than one other category)
150
Amyloidosis can be subdivided into 3 categories. What are they?
1. plasma cell dyscrasia (AL amyloid)
- abnormal production of amyloidogenic immunoglobulins
2. reactive amyloid (AA)
- increased production of serum amyloid A
- associated with chronic inflammatory and infective conditions e.g. RA
3. familial amyloidosis
151
Describe the histological diagnosis in amyloidosis.
- staining with congo red/ under polarized light
- fibrillar proteins exhibit and apple-green birefringence
-
152
What are the antibodies in poly-/dermato-myositis?
- myositis specific antibodies (MSA)
| - myositis associated autoantibodies (MAA)
