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Passmedicine Rheumatology Flashcards

1
Q

What type of arthritis is gout?

A

Inflammatory

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2
Q

How do gout episodes typically present?

A

Episodes lasting several days
Often symptom free between episodes
Acute episodes develop maximal intensity within 12h

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3
Q

What are the symptoms of gout?

A

Pain
Swelling
Erythema

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4
Q

What joint is most affected in gout?

A

MTP (podagra = gout affecting this joint)

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5
Q

Apart from the MTP what joints are also commonly affected by gout?

A

Ankle
Wrist
Knee

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6
Q

What can untreated gout lead to?

A

Repeated acute episodes of gout can damage the joints –> chronic joint problem

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7
Q

What are the radiological features of gout?

A

Joint effusion
Punched out erosions with slcerotic margins in a juxta-articular distribution
Eccentric erosions
Soft tissue tophi

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8
Q

Differences between RA and OA: Aetiology

A

RA: autoimmune
OA: wear + tear –> localised loss of cartilage, remodelling of adjacent bone, assoc. inflammation

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9
Q

Differences between RA and OA: gender

A

RA: more common in women
OA: similar in men and women

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10
Q

Differences between RA and OA: age

A

RA: all ages
OA: elderly more common

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11
Q

Differences between RA and OA: typical joints affected

A

RA: MCP, PIP
OA: large wt bearing joints, CMC, DIP, PIP

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12
Q

Differences between RA and OA: typical hx

A

RA: morning stiffness, improves with use, systemic upset
OA: pain following use, improves with rest, unilateral symptoms, no systemic upset

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13
Q

Differences between RA and OA: x-ray findings

A

RA: loss of joint space, juxta-articular osteoporosis, periarticular erosions, subluxation

OA: loss of joint space, subchondral sclerosis, subchondral cysts, osteophytes forming at joint margins

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14
Q

What is the aetiology of systemic sclerosis?

A

Unknown

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15
Q

What is systemic sclerosis characterised by?

A

Hardened, sclerotic skin, and other connective tissues

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16
Q

In which gender is systemic sclerosis more common?

A

Females

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17
Q

What are the three patterns of disease of systemic sclerosis?

A
  1. Limited cutaneous systemic sclerosis
  2. Diffuse cutaneous systemic sclerosis
  3. Scleroderma (without internal organ involvement)
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18
Q

What are the features of limited cutaneous systemic sclerosis?

A

Can present with Raynaud’s

Scleroderma of face + distal limbs

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19
Q

What antibody is strongly associated with limited cutaneous systemic sclerosis?

A

Anti-centromere Ab

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20
Q

Name a subtype of limited cutaneous systemic sclerosis

A

CREST syndrome

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21
Q

What are the features of CREST syndrome?

A 
Calcinosis
Raynaud's
Oesophageal dysmotility
Sclerodactyly
Telangiectasia
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22
Q

What are the features of diffuse systemic sclerosis?

A

Scleroderma of the trunk + proximal limbs
Commonest cause of death: interstitial lung disease/pulmonary arterial hypertension
HAS POOR PROGNOSIS

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23
Q

What are the commonest complications associated with diffuse systemic sclerosis?

A

ILD, pulmonary arterial hypertension
Renal disease
HTN

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24
Q

What antibody is strongly associated with diffuse cutaneous systemic sclerosis?

A

scl-70 antibodies

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25
What are the features of scleroderma (without internal organ involvement)?
Tightening + fibrosis of the skin | May manifest as plaques (morphoea) or linear
26
What antibodies are associated with systemic sclerosis?
ANA 90% | RF 30%
27
What is Paget's disease of the bone?
Disease of increased but uncontrolled bone turnover
28
What is the aetiology of Paget's disease?
Primary disorder of osteoclasts, with excessive osteoclastic resorption followed by increased osteoblastic activity
29
How does Paget's normally present?
1 in 20 are symptomatic
30
What bones are most commonly affected by Paget's disease?
Skull Spine Pelvis Long bones of lower extremities
31
What are the predisposing factors for Paget's?
Increasing age Male Northern latitude Family history
32
What are the clinical features of Paget's?
Tends to be older male with bone pain + isolated raised ALP
33
What are the classical features of Paget's if it is left untreated?
Bowing of tibia | Bossing of skull
34
What does blood work for Paget's usually show?
Normal Ca, phosp Raised ALP Other markers of bone turnover may be high (e.g. procollagen type 1, N-terminal propeptide, serum C-telopeptide, urinary N-telopeptide + urinary hydroproline)
35
X-ray of a Paget's diseased skull would show...
Thickened vault | Osteoporosis circumscripta
36
What are the indications for the treatment of Paget's?
Bone pain Skull/long bone deformity Fracture Periarticular Paget's
37
How is Paget's treated?
Bisphosphonates (oral risedronate/IV zoledronate) | Calcitonin
38
What are the complications associated with Paget's disease?
``` Deafness (due to cranial nerve entrapment) Bone sarcoma Fractures Skull thickening High output cardiac failure ```
39
What are the new recommendations for the treatment of RA?
Start DMARD ASAP and to include other Rx options, e.g. analgesia, physio + surgery
40
What is the initial therapy for RA?
DMARD monotherapy +/- bridging course of prednisolone
41
How do NICE recommend monitoring response to treatment in RA?
CRP + disease activity (e.g. DAS28)
42
What is the most commonly used DMARD?
Methotrexate
43
What must you do if you put a patient on methotrexate?
Monitor LFTs and FBC due to risk of myelosuppression and liver cirrhosis Repeat weekly until stabilised, thereafter monitor every 2-3m
44
What are the three other DMARDs apart from methotrexate used for RA Rx?
Sufasalazine Leflunomide Hydroxychloroquine
45
When are TNF-inhibitors indicated for Rx of RA?
Inadequate response to 2+ DMARDs including methotrexate
46
Name 3 TNF-inhibitors
Etanercept Infliximab Adalimumab
47
What is etanercept?
Recombinant human protein, acts as a decoy for TNF-a | s/c administration
48
What are the side effectsof etanercept?
Demyelination | Reactivation of TB
49
What is infliximab?
Monoclonal Ab Binds to TNF-a + prevents it fro binding with TNF receptors IV administration
50
What risks are associated with infliximab use?
Reactivation of TB
51
What is adalimumab?
Monoclonal Ab | s/c administration
52
What is rituximab?
Anti-CD20 monoclonal Ab RESULTS IN B CELL DEPLETION Given as two 1g IV infusions 2 weeks apart Infusion reactions common
53
What is abatacept?
Fusion protein that modulates a key signal required for activation of T lymphocytes --> decreased T cell proliferation + cytokine production Given as infusion
54
What is polyarteritis nodosa?
Vasculitis affecting medium sized arteries with necrotising inflammation leading to aneurysm formation
55
In which groups of people is polyarteritis nodosa most common?
In middle aged men | Associated with HEPATITIS B infection
56
What are the features of polyarteritis nodosa?
``` Fever, malaise, arthalgia Wt loss HTN Mononeuritis multiplex, sensorimotor polyneuropathy Testicular pain Livedo reticularis Haematuria, renal failure ```
57
What antibody is associated with polyarteritis nodosa?
pANCA
58
What is pseudogout?
A form of microcrystal synovitis caused by deposition of calcium pyrophosphate dihydrate crystals in the synovium
59
What are the risk factors for pseudogout?
``` Haemochromatosis Hyperparathyroidism Acromegaly Low mg, low phosp Wilson's disease ```
60
Where does pseudogout tend to affect most?
Knee, wrist, shoulders most commonly affected
61
What investigations should you do in pseudogout and what will they find?
Joint aspiration: weakly positive birefringent rhomboid shaped crystals X-Ray: chondrocalcinosis
62
What is the management of pseudogout?
Aspiration of joint fluid to exclude septic arthritis | NSAIDs/IA/IM/oral steroids
63
What is gout?
Form of microcrystal synovitis caused by deposition of monosodium urate monohydrate in the synovium
64
What causes gout?
Chronic hyperuricaemia (>450microm/l)
65
What is the acute management of gout?
First line: NSAIDs/colchicine + PPI if req. Consider oral steroids (e.g. 15mg predn) if NSAIDs/colchicine CI 3rd line: IA steroids Ensure patient continues on allopurinol if already taking
66
In treating an acute attack of gout, when should the patient be advised to stop taking NSAIDs?
1-2 days after symptoms have settled
67
What are the indications for urate lowering therapy?
All patients after their first attack of gout
68
In which patients is urate lowering therapy strongly indicated?
``` >= 2 attacks in 12 months Tophi Renal disease Uric acid renal stones Prophylaxis if on cytotoxics/diuretics ```
69
What is used for urate lowering therapy?
First line: allopurinol (2w after attack) 100mg od initially titrating every few weeks to aim for serum uric acid of <300microm/l Consider colchicine when starting allopurinol (or NSAID if colchicine not tolerated) Second line: febuxpstat
70
How do allopurinol + febuxpstat work?
Xanthine oxidase inhibitors
71
What lifestyle modifications are helpful in gout?
Reduce alcohol intake Lose wt Avoid foods high in purines
72
What foods are high in purines?
``` Liver Kidney Seafood Oily fish Yeast products ```
73
What drugs might you want to stop in someone with gout?
Precipitating drugs, e.g. thiazides
74
How does colchicine work?
Inhibits microtubule polymerization by binding to tubulin, interfering with mitosis. Also inhibits neutrophil motility and activity
75
``` What are the expected values of serum: Calcium Phosphate ALP PTH in someone with osteoporosis? ```
Calcium - normal Phosphate - normal ALP - normal PTH - normal
76
``` What are the expected values of serum: Calcium Phosphate ALP PTH in someone with osteomalacia? ```
Calcium - decreased Phosphate - decreased ALP - increased PTH - increased
77
``` What are the expected values of serum: Calcium Phosphate ALP PTH in someone with primary hyperparathyroidism? ```
Calcium - increased Phosphate - decreased ALP - increased PTH - increased
78
``` What are the expected values of serum: Calcium Phosphate ALP PTH in someone with chronic kidney disease --> secondary hyperparathyroidism? ```
Calcium - decreased Phosphate - increased ALP - increased PTH - increased
79
``` What are the expected values of serum: Calcium Phosphate ALP PTH in someone with Paget's disease of the bone? ```
Calcium - normal Phosphate - normal ALP - increased PTH - normal
80
``` What are the expected values of serum: Calcium Phosphate ALP PTH in someone with osteopetrosis? ```
Calcium - normal Phosphate - normal ALP - normal PTH - normal
81
How do bisphosphonates work?
Analogues of pyrophosphate (a molecule thatdecreases demineralisation in bone) They inhibit osteoclasts by reducing recruitment + promoting apoptosis
82
What are bisphosphonates used to treat?
Prevention and treatment of osteoporosis Hypercalcaemia Paget's disease Pain from bone metatases
83
What are the AEs of bisphosphonates?
Oesophagitis, oesophageal ulcers (esp. alendronate) Osteonecrosis of the jaw Increased risk of atypical stress fractures of proximal femoral shaft Acute phase response: fever, myalgia, arthalgia following administration Hypocalcaemia (due to Ca efflux from bone)
84
How do bisphosphonate tablets need to be taken?
On empty stomach in the morning at least 30m before breakfast Sitting upright/standing for 30m Swallow with plenty of water
85
When should you co-prescribe vitamin D/Ca with bisphosphonates?
Vit D always | Ca if dietary intake is inadequate
86
When should you stop bisphosphonates?
After 5 years if the patient is <75, femoral neck T score of >2.5 + low risk according to FRAX/NOGG
87
What is temporal arteritis?
Large vessel vasculitis
88
What condition overlaps with temporal arteritis?
Polymyalgia rheumatica
89
What are the features of temporal arteritis?
``` Patient usually >60 Rapid onset, <1m Headache Jaw claudication Visual disturbances (secondary to anterior ischaemic optic neuropathy) Tender, palpable temporal artery 50% have features of PMR Lethargy, depression, low grade fever, anorexia, night sweats ```
90
What are features of PMR?
Aching | Morning stiffness in proximal limb muscles
91
What investigations should you do for temporal arteritis?
Inflammatory markers, expect ESR >50, elevated CRP Temporal artery biopsy (NB skip lesions may be present) CK and EMG normal
92
How do you treat temporal arteritis?
High dose prednisolone (should be dramatic response if not reconsider diagnosis) Urgent ophthalmology review for those with visual symptoms (on the day)
93
What conditions are associated with cANCA antibodies?
Granulomatosis with polyangiitis (wegener's)
94
What conditions are associated with pANCA?
Churg strauss syndrome, primary sclerosing cholangitiis, sometimes granulomatosis with polyangiitis Others: UC, connective tissue dx (e.g. RA, SLE, Sjogren's), autoimmune hepatitis
95
What is the most common target for cANCA?
Serine protease 3
96
What is the most common target for pANCA?
Myeloperoxidase
97
What is Marfan's syndrome?
Autosomal dominant connective tissue disorder
98
What is the aetiology of Marfan's syndrome?
FBN1 gene defect on chromosome 15 that encodes for the protein fibrillin 1
99
What are the features of Marfan's?
Tall stature with arm span to height ratio >1.05 High arched palate Arachnodactyly Pectus excavatum Scoliosis of >20 degrees Heart: dilatation of aortic sinuses (may --> aortic aneurys, aortic dissection, aortic regurg, mitral valve prolapse) Lungs: repeated pneumothoraces Eyes: upward lens dislocation, blue sclera, myopia Dural ectasia
100
What is dural ectasia?
Ballooning of the dural sac at the lumbrosacral level
101
What treatments have extended the life expectancy of those with Marfan's?
Regular echos, beta-blockers, ACEi
102
What is osteomalacia?
Normal bony tissue but decreased mineral content Rickets if growing, osteomalacia after epiphysis fusion
103
What can cause osteomalacia?
``` Vit D deficiency, e.g. malabsorption, lack of sunlight, diet Renal failure Drug induced, e.g. anticonvulsants Vitamin D resistant, inherited Liver disease, e.g. cirrhosis ```
104
What are the features of Rickets?
Knock-knee, bow legs, features of hypocalcaemia
105
What are the features of osteomalacia?
Bone pain, fractures, muscle tenderness, proximal myopathy
106
What investigations should you do in suspected ostemalacia?
25(OH) vitamin D: low Alk phos: raised Ca, phos: low X-Ray: children: cupped, ragged metaphyseal surfaces, adults: translucent bands (Looser's zones or pseudofractures)
107
How do you treat osteomalacia?
Ca with vit D supplements
108
What does the histology of PMR show?
Vasculitis with giant cells | Characteristically skips certain sections of affected artery while damaging others
109
What arteries are most affected in PMR?
Muscle bed arteries
110
What are the typical features of PMR?
Patient typically >60 Usually rapid onset (<1m) Aching, morning stiffness in proximal limb muscles Also mild polyarthalgia, lethargy, depression, low grade fever, anorexia, night sweats
111
What investigations should you do in PMR?
ESR > 40 mm/hr | Note CK and EMG normal
112
How do you treat PMR?
Prednisolone e.g. 15mg od Expect dramatic response
113
How does azathioprine work?
It is metabolised to the active compound mercaptopurine, a purine analogue that inhibits purine synthesis
114
How do you test for azathioprine toxicity?
Thiopurine methyltransferase test - TPMT deficiency predisposes to azathioprine related pancytopenia
115
What adverse effects are associated with azathioprine?
``` Bone marrow depression NV Pancreatitis Increased risk of non-melanoma skin cancer Agranulocytosis/myelosupression ```
116
What drug should you be careful about prescribing alongside azathioprine?
Allopurinol A significant reaction may occur Use lower doses of azathioprine
117
What is Sjogren's syndrome?
An autoimmune disorder affecting exocrine glands --> dry mucosal surfaces Can be primary/secondary to RA/other connective tissue dx
118
In which gender is Sjogren's syndrome more common?
Females
119
For what condition is there a marked increased in risk of in Sjogren's syndrome?
Lymphoid malignancy
120
What are the features of Sjogren's syndrome?
``` Dry eyes (keratoconjunctivitis sicca) Dry mouth Vaginal dryness Arthalgia Raynaud's, myalgia Sensory polyneuropathy Recurrent episodes of parotitis Renal tubular acidosis ```
121
What antibodies are associated with Sjogren's syndrome?
RF in nearly 100% ANA 70% AntiRo, AntiLa
122
What test can do you for Sjogren's syndrome?
Schirmer's test (filter paper near conjunctival sac to measure tear formation)
123
What does the histology show in Sjogren's syndrome?
Focal lymphocytic infiltration
124
How do you manage Sjogren's syndrome?
Artificial saliva + tears | Pilocarpine may stimulate saliva production
125
How does methotrexate work?
It is an antimetabolite that inhibits dihydrofolate reductase (an enzyme essential for the synthesis of purines and pyrimidines)
126
What are the indications for methotrexate use?
Inflammatory arthritis (esp. RA) Psoriasis ALL
127
What are the adverse effects of methotrexate?
``` Mucositis Myelosupression Pneumonitis Pulmonary fibrosis Liver fibrosis ```
128
What is the BNFs advice on methotrexate + pregnancy?
Avoid pregnancy for at least 6m after treatment cessation | Men should use effective contraception until 6m after treatment
129
What should you co-prescribe with methotrexate?
Folic acid 5mg
130
What is the recommended starting dose of methotrexate?
7.5mg
131
Only one strength of methotrexate should be prescribed - what is this?
2.5mg
132
What drugs should you avoid co-prescribing with methotrexate?
Trimethoprim (increases risk of marrow aplasia as trimethoprim + methotrexate are both folate antagonists so --> pancytopenia/myelosuppression putting pt at risk of severe infection/bleeds) Aspirin (increases risk of methotrexate toxicity due t reduced excretion)
133
How do you treat methotrexate toxicity?
Folinic acid
134
Which HLA allele is reactive arthritis associated with?
HLA-B27
135
What is Reiter's syndrome?
Triad of urethritis, conjunctivitis and arthritis Was originally following dysenteric illness in WW2, but most commonly due to STIs now
136
What is reactive arthritis?
Arthritis that develops following an infection where the organism cannot be recovered from the joint
137
What are the features of reactive arthritis?
Develops after 4-6 weeks of initial infection Symptoms generally last about 4-6m Asymmetrical oligoarthritis of lower limbs Dactylitis Urethritis Conjuncitivitis, anterior uveitis Circinate balanitis, keratoderma blenorrhagica
138
What is keratoderma blenohagica?
Waxy brown/yellow papules on palms/soles
139
What HLA allele is ankylosing spondylitis associated with?
HLA-B27
140
In which group of people is ankylosing spondylitis most common?
Men, 20-30yo
141
What investigations can you use in ankylosing spondylitis?
ESR, CRP typically raised but normal levels do not exclude AS HLA-B27 not useful as it is +ve in 10% of normal patients Plain X-Ray of the SI joint is most useful for diagnosis
142
What are some later changes of ankylosing spondylitis you may see on X-Ray?
Sacroilitis - subchondral erosions, sclerosis Squaring of lumbar vertebrae Bamboo spine Syndesmophytes (due to ossification of outer fibres of annulus fibrosus) CXR - atypical fibrosis
143
What might spirometry show in ankylosing spondylitis?
Restrictive pattern due to pulmonary fibrosis, kyphosis, ankylosis of costovertebral joints
144
What is the management for ankylosing spondylitis?
``` Regular exercise, e.g. swimming NSAIDs 1st line Physio DMARDs if peripheral joint involvement Anti-TNF therapy should be given to patients with persistently high dx activity despite conventional Rx (e.g. etanercept) ```
145
What antibodies tend to be positive in SLE?
ANA 99% RF 20% anti-dsDNA very specific to SLE Anti-Sm - even more specific Others - anti-u1, SS-A (anti-Ro), SS-B (anti-La)
146
How do you monitor disease progression in SLE?
ESR (during active dx, CRP characteristically normal) C3, C4 levels low during active disease (formation of complexes leads to consumption of complement) Anti-dsDNA titres can be used to monitor disease
147
What is the different between Raynaud's disease and Raynaud's phenomenon?
Raynaud's disease = primary | Raynaud's phenomenon = secondary
148
In which group of people does Raynaud's most commonly present?
Women in their 30s with bilateral symptoms
149
In someone presenting with Raynaud's disease, what factors would suggest underlying connective tissue disease?
``` Onset after 40 years Unilateral symptoms Rashes Presence of Abs Features which may suggest SLE, RA (e.g. recurrent miscarriages, arthritis) Digital ulcers, calcinosis Chillblains ```
150
What are some secondary causes of Raynauds?
``` Connective tissue dx (scleroderma, RA, SLE) Leukaemia Type 1 cyroglobulinaemia, cold agglutins Use of vibrating tools COCP, ergot Cervical rib ```
151
How do you manage Raynaud's?
First line: CCB, e.g. nifedipine | IV prostacycline infusions
152
In which group of people is SLE more common?
Women | Afro-carribbean origin
153
What are the general features of SLE?
Fatigue Fever Mouth ulcers Lymphadenopathy
154
What are the skin features of SLE?
Malar (butterfly) rash that spares the nasolabial folds Discoid rash (scaly, erythematous, well demarcated in sun-exposed areas) Photosensitivity Raynaud's phenomenon Livedo reticularis Non-scarring alopecia
155
What are the MSK features of SLE?
Arthalgia | Non-erosive arthritis
156
What are the CV features of SLE?
Pericarditis | Myocarditis
157
What are the respiratory features of SLE?
Pleurisy | Fibrosing alveolitis
158
What are the renal features of SLE?
Proteinuria | Diffuse proliferative GN
159
What are the neuropsychiatric features of SLE?
Anxiety, depression Psychosis Seizures
160
What is rheumatoid factor?
Circulating antibody (usually IgM) that interacts with the Fc portion of the patients own IgG
161
How can RF be detected?
Rose-Waaler test: sheep red cell agglutination | Latex agglutination test (less specific)
162
What % of RA patients have a +ve RF?
70-80% High titre levels assoc. w. severe progressive dx
163
In which conditions is RF commonly +ve?
``` Sjogren's 100% Felty's syndrome 100% Infective endocarditis 50% SLE 20% Systemic sclerosis 30% General pop 5% Rarely: TB, HBV, EBV, leprosy ```
164
When can you detect anti-cyclic citrullinated peptide antibody in those with RA?
May be detectable up to 10 years before development of RA
165
When should you test for anti-CCP antibodies?
In those with suspected RA but negative RF
166
What are the risk factors for osteoporosis as included in the FRAX risk assessment tool?
``` Hx of glucocorticoid use RA Alcohol excess Hx of parental hip fracture Low BMI Current smoking ```
167
Apart from the ones included on FRAX, what other risk factors are there for osteoporosis?
``` Sedentary lifestyle Premature menopause Caucasians and asians Endocrine disorders (e.g. hyperthyroidism, hypogonadism (e.g. Turner's), growth hormone deficiency, hyperparathyroidism, DM Multiple myeloma, lymphoma IBD, malabsorption, gastrectomy, liver disease CKD Osteogenesis imperfecta, homocystinuria ```
168
What medications can worsen osteoporosis?
``` Glucocorticoids SSRIs Anti-epileptics PPIs Glitazones Long term heparin therapy Aromatase inhibitors, e.g. anastrozole ```
169
Why should you investigate osteoporosis for secondary causes?
To exclude diseases that mimic osteoporosis (e.g. osteomalacia) To identify the cause of osteoporosis + contributory factors Assess the risk of subsequent fractures To select the most appropriate form of treatment
170
What investigations are recommended for diagnosing osteoporosis?
``` Hx, Ex FBC, ESR/CRP Serum Ca, albumin, creatinine, phosphate, alk phos, liver transaminases TFTs DXA ```
171
What other investigations may be useful in osteoporosis for specific indications?
Lateral radiographs of lumbar and thoracic spine/DXA-based vertebral imaging Protein immunoelectrophoresis and urinary Bence-Jones proteins 25OHD PTH Serum testosterone, SHBG, FSH, LH (in men), Serum prolactin 24 hour urinary cortisol/dexamethasone suppression test Endomysial and/or tissue transglutaminase antibodies (coeliac disease) Isotope bone scan Markers of bone turnover, when available Urinary calcium excretion
172
What are the minimum baseline bloods that should be ordered for all patients with suspected osteoporosis?
``` FBC UE LFTs Bone profile CRP TFTs ```
173
What is Behcet's syndrome?
A complex multisystem disorder associated with presumed autoimmune mediated inflammation of the arteries and veins
174
What is the aetiology of Behcet's syndrome?
Unknown
175
What is the classic triad of Behcet's syndrome?
Oral ulcers Genital ulcers Anterior uveitis
176
In which groups of people is Behcet's most common?
Eastern Mediterranean countries Men 20-40yos
177
What HLA allele is Behcet's associated with?
HLA B51
178
What are the features of Behcet's syndrome?
``` 1. oral ulcers, 2. genital ulcers, 3. anterior uveitis Thrombophlebitis and DVT Arthritis Neurological involvement Abdo pain, diarrhoea, colitis Erythema nodosum ```
179
What is used to diagnose Behcet's syndrome?
No definitive test Based on clinical findings Positive pathergy test is suggestive (puncture site following needle prick becomes inflamed with small pustule forming)
180
Where is the gene for HLA antigens found?
Chromosome 6
181
What diseases are strongly associated with HLA-A3 antigen?
Haemochromatosis
182
What diseases are strongly associated with HLA-B51 antigen?
Behcet's disease
183
What diseases are strongly associated with HLA-B27 antigen?
Ankylosing spondylitis Reiter's syndrome Acute anterior uveitis
184
What diseases are strongly associated with HLA-DQ2/8 antigen?
Coeliac disease
185
What diseases are strongly associated with HLA-DR2 antigen?
Nacrolepsy | Goodpasture's
186
What diseases are strongly associated with HLA-DR3 antigen?
Dermatitis Herpetiformis Sjogren's syndrome Primary biliary cirrhosis
187
What diseases are strongly associated with HLA-DR4 antigen?
T1DM | RA
188
What is chronic fatigue syndrome?
At least 4 months of disabling fatigue affecting mental + physical function more than 50% of the time in the absence of other disease which may explain symptoms
189
What are some other features of chronic fatigue syndrome?
``` Sleep problems Muscle/joint pains Headaches Painful lymph nodes without enlargement Sore throat Cognitive dysfunction (e.g. difficulty thinking, inability to concentrate...) Physical/mental exertion makes worse General malaise, flu like symptoms Dizziness Nausea Palpitations ```
190
What investigations should you do in chronic fatigue syndrome and why?
To exclude other pathology | FBC, UE, LFTs, TFTs, CRP, ESR, Ca, ferritin, coeliac screening, urinalysis
191
How do you manage chronic fatigue syndrome?
CBT Graded exercise therapy Pacing (organising activities to avoid tiring) Low dose amitriptyline may be useful for poor sleep Referral to pain management if it is a predominant feature
192
What is Ehler-Danlos syndrome?
AD connective tissue disorder that mostly affects type III collagen Leads to tissue being more elastic than normal --> joint hypermobility + increased elasticity of skin
193
What are the features of Ehler-Danlos syndrome?
Elastic, fragile skin Joint hypermobility + recurrent dislocation Easy bruising Aortic regurgitation, mitral valve prolapse, aortic dissection SAH Angioid retinal streaks
194
What is osteogenesis imperfecta?
Group of disorders of collagen metabolism resulting in bone fragility + fractures
195
How is osteogenesis imperfecta inherited?
AD
196
What is the aetiology of osteogenesis imperfecta?
Abnormality in type 1 collagen due to decreased synthesis of pro-alpha 1 or pro-alpha 2 collagen polypeptides
197
If the X-Ray is negative for SI joint involvement in AS but suspicion of AD is high what should be the next step in investigation?
MRI
198
What imaging findings confirm the diagnosis of AS?
Signs of inflammation in the SI joints (bone marrow oedema)
199
Name one of the most important risk factors for osteoporosis
Use of corticosteroids
200
The risk of osteoporosis is thought to rise significantly once an individual is taking what dose of steroids?
Equivalent of prednisolone 7.5mg a day for 3m+
201
If a patient is going t be taking steroids >3m and are at risk of osteoporosis when should bone protection start?
ASAP
202
What are the two groups that patients at risk of corticosteroid induced osteoporosis are split into?
1. >65/previously had a fragility fracture --> offer bone protection 2. <65 --> offer bone scan + results dependent on T score >0 - reassure; 0-->-1.5 - repeat bone density scan in 1-3y,
203
What is the first line treatment for corticosteroid induced osteoporosis?
Alendronate | Ensure Ca and vit D replete
204
When is treatment for osteoporosis indicated?
Following osteoporotic fragility fracture in a post-menopausal women confirmed to have osteoporosis (T score -2.5 or less) (DEXA scan be not be necessary if clinician considers inappropriate/unfeasible)
205
Who with osteoporosis should be offered vit D/Ca supplements?
All, expect those who are replete
206
What is the first line treatment for osteoporosis?
Alendronate
207
What % of pts cannot tolerate alendronate + why is this usually?
25% | Upper GI problems
208
What is second line treatment for osteoporosis?
Risedronate or etidronate
209
What treatments are recommended for those with osteoporosis who cannot take bisphosphonates?
Stronium ranelate | Raloxifene
210
What evidence is there for alendronate/risedronate/etidronate?
Reduce risk of vertebral and non-vertebral fractures
211
What is the option for a once a month oral bisphosphonate?
Ibandronate
212
What kind of drug is raloxifene?
Selective oestrogen receptor modulator
213
What is the evidence behind raloxifene?
Reduces vertebral fractures, prevents bone loss + increases bone density in spine + femur
214
What are the AEs of raloxifene?
May worsen menopausal symptoms | Increases risk of TE events
215
What is an additional benefit of raloxifene?
May reduce risk of breast cancer
216
What is the action of strontium ranelate?
Increases deposition of new bone by osteoblasts (promotes differentiation of pre-osteoblasts to osteoblasts) Reduces resorption of bone by inhibiting osteoclasts
217
What is the issue with strontium ranelate?
Concerns re safety profile | Must only be prescribed by a specialist if there are no other treatments
218
What are CIs for strontium ranelate?
Hx of CV dx or significant CV dx or prev. VTE
219
What are the AEs of strontium ranelate?
Increased risk of CV event Increased risk of TE events Serious skin reactions, e.g. SJS
220
What is the action of denosumab?
Human monoclonal antibody that inhibits RANK ligand, which in turn inhibits the maturation of osteoclasts
221
How is denosumab given?
Single sc injection every 6m
222
What is teriparatide?
Recombinant form of parathyroid hormone
223
What is teriparatide good at?
Increasing bone mineral density
224
What evidence does HRT have for osteoporosis management?
Reduces incidence of vertebral fractures + non-vertebral fractures
225
When is the ONLY situation you would use HRT for osteoporosis management?
If pt also having vasomotor symptoms
226
What non-medication treatments may be useful in the management of osteoporosis?
Hip protectors - compliance is an issue | Falls risk assessment - consider for high risk pts
227
What is the most common organism causing septic arthritis?
Staph aureus
228
What organism should be considered for causing septic arthritis in young sexually active individuals?
Neisseria gonorrhoea
229
In adults, where is the most common location for septic arthritis?
Knee
230
What criteria is used to assess the probability of septic arthritis in kids?
``` Kocher's criteria - 1 point for each of: - Non wt bearing - Fever >38.5 - WCC >12x10^9/L - ESR >40mm/hr ``` (2 points = 40% risk, 3 = 93%, 4 = 99%)
231
How is septic arthritis managed?
``` IV antibiotics (flucloxacillin/clindamycin) for 6-12w Needle aspiration to decompress the joint Athroscopic lavage may be req. ```
232
What should be done before treating septic arthritis?
Take sample of synovial fluid before antibx are started!
233
What is dermatomyositis?
Inflammatory disorder causing SYMMETRICAL, proximal muscle weakness + skin lesions
234
What is the aetiology of dermatomyositis?
Idiopathic Associated with connective tissue dx Associated with underlying malignancy
235
What malignancies are most commonly associated with dermatomyositis?
Ovarian, breast, lung
236
What is polymyositis?
Variant of dermatomyositis where skin manifestations are not prominent
237
What are the skin features in dermatomyositis?
``` Photosensitive Macular rash over back + shoulders Helitrope rash in periorbital region Gottron's papules Mechanics hands - v. dry and scaly, with cracks on palmar + lateral aspects of fingers Nail fold capillary dilatation ```
238
What are Gottron's papules?
Roughened red papules over extensor surface of fingers
239
What are the non-skin manifestations of dermatomyositis?
``` Proximal muscle weakness +/- tenderness Raynaud's Respiratory muscle weakness Interstitial lung dx, e.g. fibrosing alveolitis/organising pneumonia Dysphagia, dysphonia ```
240
What antibody do 80% of those with dermatomyositis have?
ANA
241
What antibody do 30% of those with dermatomyositis have?
Aminoactyl-tRNA synthases (e.g. Jo-1, Abs to signal recognition particle, anti-Mi-2 antibodies)
242
What antibody is generally associated with RA?
Anti-CCP antibody
243
What antibody is generally associated with SLE?
Anti-dsDNA
244
What antibody is generally associated with diffuse systemic sclerosis?
Anti-Scl-70 antibodies
245
What is anti-mitochondrial antibodies (AMAs) associated with?
Primary biliary cirrhosis
246
What two conditions is hydroxychloroquine used in?
RA | Systemic/discoid lupus erythematosus
247
What adverse effect is associated with hydroxychloroquine?
Bull's eye retinopathy - my result in severe + permanent vision loss
248
What 1 examination/investigation is required before starting a patient on hydroxychloroquine?
Baseline ophthalmological examination | thereafter annual screening
249
Can hydroxychloroquine be used in pregnancy?
Yes
250
What HLA allele is associated with ankylosing spondylitis?
HLA-B27
251
What is the typical history of someone presenting with ankylosing spondylitis?
Young man who presents with low back pain + stiffness of insidious onset Stiffness worse in morning + improves with exercise Pt may experience pain at night, which improves on getting up
252
What will you see on examination of someone with AS?
Reduced lateral flexion Reduced forward flexion (Schober's test) Reduced chest expansion
253
What is Schober's test?
Draw line 10cm above + 5cm below back dimples (L5) | The distance between the two lines should increase by >5cm when the pt bends as far forward as possible
254
What are other features associated with AS?
``` The A's: Apical fibrosis Anterior uveitis (in 1/3rd) Aortic regurgitation Achilles tendonitis AV node block Amyloidosis ``` Cauda equina syndrome Peripheral arthritis
255
When is the typical onset of SLE?
20-40y
256
What kind of hypersensitivity reaction is SLE?
Type 3
257
What HLA alleles are associated with SLE?
HLA B8, DR2, DR3
258
What is thought to cause SLE?
Immune system dysregulation leading to immune complex formation Immune complexes can deposit in any organ incl. skin, joints, kidneys, brain
259
What should all pts with OA be offered?
Help with wt loss | Advice re. local muscle strengthening exercises + general fitness
260
What are the first line analgesics for OA?
Paracetamol + topical NSAIDs (for knee/hand)
261
What are second-line analgesics for OA?
NSAIDs/COX-2 inhibitors, opioids, capsaicin cream, IA corticosteroids
262
What must be co-prescribed with COX-2 inhibitors/NSAIDs?
PPI
263
What drug that if the pt was already on would CI prescribing COX-2 inhibitors/NSAIDs?
Aspirin
264
What are non-pharmacological treatments for OA?
Supports + braces TENS Shock absorbing insoles/shoes
265
If all conservative measures for OA management fail what are the options?
Refer for consideration of joint replacement
266
What is glucosamine?
Normal constituent of glycosaminoglycans in cartilage and synovial fluid
267
What is the evidence re glucosamine use in treatment of OA?
Significant improvement in symptoms + reduces joint space narrowing However NICE don't recommend it
268
What is osteoporosis?
Disorder characterised by loss of bone mass
269
Define osteoporosis
Bone mineral density of less than 2.5 standard deviations below the young adult mean density
270
What does osteoporosis put pts at risk of?
Fragility fractures (e.g. neck of femur fracture, which carries significant morbidity + mortality)
271
What % of post-menopausal women will suffer an osteoporotic fracture at some point?
50%
272
What are the major risk factors for osteoporosis?
Age, female sex
273
What are other risk factors for osteoporosis?
``` Corticosteroids use Smoking Alcohol Low BMI FH ```
274
What screening tools can you use for osteoporosis? What do these assess?
FRAX/QFracture | Assess 10 year risk of pt developing a fragility fracture
275
Which pts should be assessed for osteoporosis?
Those who have sustained a fragility fracture
276
How is BMD assessed?
Dual energy X-Ray absorptiometry (DEXAscan) from which a T score can be worked out
277
What bones does the DEXA scan use?
Lumbar spine | Hip
278
What T score in either the hip or the lumbar spine warrants starting treatment for osteoporosis?
279
What is the first line treatment for osteoporosis?
Bisphosphonate, e.g. alendronate
280
When are bisphosphonates contraindicated?
If eGFR <35
281
What are some respiratory extra-articular complications of RA?
Pulmonary fibrosis, pleural effusion, pulmonary nodules, bronchiolitis obliterans, methotrexate pneumonia, pleurisy
282
What are some ocular extra-articular complications of RA?
Keratoconjunctivitis sicca, episcleritis, scleritis, corneal ulceration, keratitis, steroid induced cataracts, chloroquine retinopathy
283
What is an MSK extra-articular complications of RA?
Osteoporosis
284
What is a CV extra-articular complications of RA?
IHD (has similar risk as T2DM)
285
What is a psychological extra-articular complications of RA?
Depression
286
What is an immunological extra-articular complications of RA?
Increased risk of infection
287
What two conditions are less commonly associated with RA?
Felty's syndrome | Amyloidosis
288
What are the features of Felty's syndrome?
RA Splenomegaly Low WCC
289
What organisms can cause the post-dysenteric form of reactive arthritis?
``` Shigella flexneri Salmonella typhimurium Salmonella enteritidis Yersinia enterocolitica Campylobacter ```
290
What organisms can cause the post-STI form of reactive arthritis?
Chlamydia trachomatis
291
What is the management of reactive arthritis?
Analgesics, NSAIDs, IA steroids | Sulfasalazine, methotrexate sometimes used for persistent symptoms
292
How long does it usually take for reactive arthritis to clear up?
Usually within 1 y
293
What would you expect to find of the synovial fluid in reactive arthritis?
Cloudy yellow colour Culture negative No crystals WCC 20, 000/mm
294
What is the most common Ig?
IgG
295
What is the action of IgG?
Enhances phagocytosis of viruses/bacteria | Fixes complement + passes to foetal circulation
296
What is the most predominant Ig in breast milk?
IgA
297
Where is IgA mostly found?
Secretions of digestive, respiratory and urogenital tracts/systems
298
What is the action of IgA?
Provides localised protection on mucous membranes
299
What is the action of IgM?
First Ig to be secreted in response to an infection | Fixes complement b
300
Does IgM pass to the foetal circulation?
No
301
What is the role of IgD antibodies?
Largely unknown but involved in activation of B cells
302
What is the action of IgE?
Mediates T1 hypersensitivity reactions by binding to Fc receptors on the surface of mast cells + basophils Also provides immunity to parasite, e.g. helminths
303
What is the least abundant Ig in the serum?
IgE
304
What is the structure of IgE?
Monomer
305
What is the structure of IgD?
Monomer
306
What is the structure of IgM?
Penamer
307
What is the structure of IgA?
Monomer/dimer
308
What is the structure of IgG?
Monomer
309
What can predispose to gout?
Decreased excretion of uric acid Increased production of uric acid Lesch-Nyhan syndrome
310
What things can cause decreased excretion of uric acid?
Drugs, e.g. diuretics CKD Lead toxicity
311
What things can cause increased production of uric acid?
Myeloproliferative/lymphoproliferative disorder Cytotoxic drugs (e.g. chemotherapy) Severe psoriasis
312
What is the pathophysiology of Lesch-Nyhan syndrome?
Hypoxanthine-guanine phosphoribosyl transferase (HGPRTase) deficiency
313
How is Lesch-Nyhan syndrome inherited?
X-linked recessive
314
What are the features of Lesch-Nyhan syndrome?
``` Gout Renal failure Neurological deficits Learning difficulties Self-mutilation ```
315
What kind of muscle weakness do you get in PMR?
No true weakness of limb girdles | Weakness of muscles is due to myalgia (pain inhibition)
316
How would you describe the pain in OA?
Exacerbated by exercise, relieved by rest
317
What movement is affected first in hip OA?
Internal rotation
318
What are risk factors for hip OA?
Age Obesity Prev. joint problems
319
When is the pain from inflammatory arthritis most prominent?
Morning
320
What other features will be present in inflammatory arthritis?
Systemic features, raised inflammatory markers
321
What features may you see in referred lumbar spine pain?
Femoral nerve compression may cause referred hip pain | Femoral nerve stretch test +ve
322
What is the femoral nerve stretch test?
Lie pt prone, extend hip joint with straight leg when bend the knee This stretches the femoral nerve and causes pain if it is trapped
323
What causes greater trochanteric pain syndrome (Trochanteric bursitis)?
Repeated movement of the fibroelastic iliotibial band
324
What are the features of greater trochanteric pain syndrome (Trochanteric bursitis)?
Pain + tenderness over lateral thigh
325
What people tend to get greater trochanteric pain syndrome (Trochanteric bursitis)?
Women aged 50-70
326
What causes meralgia paraesthetica?
Compression of lateral cutaneous nerve of thigh
327
What are the symptoms of meralgia paraesthetica?
Burning over anterolateral aspect of thigh
328
What may avascular necrosis follow?
High dose steroid therapy or hip fracture or dislocation
329
When is pubic symphysis dysfunction common?
In pregnancy - ligament laxity increases in response to hormonal changes
330
What symptoms do you get in pubic symphysis dysfunction?
Pain over pubic symphysis with radiation to the groins + medial aspect of thighs
331
What kind of gait may you see in pubic symphysis dysfunction?
Waddling
332
When is transient idiopathic osteoporosis seen?
Uncommon condition but more seen in 3rd trimester of pregnancy
333
How does transient idiopathic osteoporosis present?
Groin pain + limited range of movement in the hip May be unable to wt bear ESR may be elevated
334
What is a T score based off of?
Bone mass of a young reference population
335
What scores a T score of -1 mean?
Bone mass of one SD below the young reference population
336
What is the Z score adjusted for?
Age, gender, ethnic factors
337
T score >-1 =?
Normal
338
T score of -1 to -2.5 =?
Osteopenia
339
T score >-2.5
Osteoporosis
340
What usually results in lateral epicondylitis?
Unaccustomed activity, e.g. playing tennis or house painting
341
What are the features of lateral epicondylitis?
Pain/tenderness at lateral epicondyle | Worse on wrist extension against resistance/supination of forearm with elbow extended
342
How long do bouts of lateral epicondylitis tend to last?
6m-2y
343
What is involved in the management of lateral epicondylitis?
Advice re avoiding muscle overload Simple analgesia Steroid injection Physio
344
What are anti-jo antibodies also known as?
Ab against aminoacyl-tRNA
345
What features are associated with antisynthase syndrome?
Myositis Interstitial lung disease Thickened + cracked skin of hands Raynaud's
346
What is antisynthase syndrome?
Subtype of dermatomyositis
347
What are poor prognostic features in rheumatoid arthritis?
``` RF +ve Poor functional status at presentation HLA DR4 X-ray - early erosions Extra-articular features, e.g. nodules Insidious onset Anti-CCP antibodies Female gender ```
348
What are the features of drug induced lupus?
Arthalgia Myalgia Skin (e.g. malar rash) + pulmonary involvement (e.g. pleurisy)
349
What antibodies are positive in drug induced lupus?
ANA 100% Antihistone in 80-90% Anti-Ro/anti-Smith less commonly +ve
350
What are the most common causes of drug induced lupus?
Procainamide | Hydralazine
351
What are the less common causes of drug induced lupus?
Isoniazid Minocycline Phenytoin
352
In which patients may you consider colchicine instead of NSAIDs for treating an acute attack of gout?
Elderly pts on warfarin (puts them at risk of GI haemorrhage)
353
What are the rotator cuff muscles?
``` SItS - small t for teres minor Supraspinatus Infraspinatus teres minor Subscapularis ```
354
What is the action of supraspinatus?
Abducts arm before deltoid (first 20 degrees)
355
What is the action of infraspinatus?
Rotates arm laterally
356
What is the action of teres minor?
Adducts + rotates arm laterally
357
What is the action of subscapularis?
Adducts + rotates arms medially
358
What does calcinosis look like?
White deposits
359
What symptoms do patients get with oesophageal dysmotility?
Dysphagia
360
What does sclerodactyly look like?
Thickened skin on tops of hands an inability to straighten fingers
361
What is telangiectasia?
Excessive number of spider naevi
362
What is the mechanism of type 1 hypersensitivity reactions?
Antigen reacts with IgE bound to mast cells
363
Give examples of T1 HS reactions
Anaphylaxis | Atopy (e.g. asthma, hayfever, eczema)
364
What is the mechanism of type 2 hypersensitivity reactions?
IgG or IgM binds to antigen on cell surface
365
Give examples of T2 HS reactions
``` Autoimmune haemolytic anaemia ITP Goodpasture's syndrome Pernicious anaemia Acute haemolytic transfusion reactions Rheumatic fever Pemphigus vulgaris / bullous pemphigoid ```
366
What is the mechanism of type 3 hypersensitivity reactions?
Free antigen + antibody combine
367
Give examples of T3 HS reactions
Serum sickness Systemic lupus erythematosus Post-streptococcal glomerulonephritis Extrinsic allergic alveolitis (especially acute phase)
368
What is the mechanism of type 4 hypersensitivity reactions?
T-cell mediated
369
Give examples of T4 HS reactions
``` TB/tuberculin skin reaction Graft versus host disease Allergic contact dermatitis Scabies Extrinsic allergic alveolitis (especially chronic phase) MS Guillain-Barre syndrome ```
370
What is the mechanism of type 5 hypersensitivity reactions?
Antibodies that recognise and bind to cell surface receptors and stimulate them or block ligand binding
371
Give examples of T5 HS reactions
Grave's disease | Myasthenia gravis
372
What drug should those with RA be given as soon as diagnosed?
DMARD + short dose of bridging prednisolone
373
What are other treatment options for RA?
Analgesia Physio Surgery
374
What should be used to monitor response to treatment in RA?
CRP and disease activity (e.g. DAS28)
375
How are flares in RA managed?
Corticosteroids (oral/intramuscular)
376
What is the most widely used DMARD in RA?
Methotrexate
377
What monitoring is req. for pts on methotrexate?
LFTs + FBC (due to risk of liver cirrhosis + myelosuppression)
378
What are 3 other DMARDs apart from methotrexate that may be used to treat RA?
Sulfasalazine Leflunomide Hydroxychloroquine
379
If a pt with RA is having an inadequate response to DMARDs what is the next step in Rx?
If inadequate response to at least 2 DMARDs can start TNF-inhibitor
380
What are the four TNF-inhibitors used in the treatment of RA?
Etanercept Infliximab Adalimumab Abatacept
381
How does etanercept work?
Recombinant human protein (anti-CD20 monoclonal Ab), acts as a decoy for TNF-a
382
What are AEs of etanercept?
Demyelination | Reactivation of TB
383
How is etanercept administered?
SC
384
How does infliximab work?
Monoclonal Ab, binds to TNF-a and prevents it binding with TNF receptors
385
How is infliximab administered?
IV
386
What are the AEs of infliximab?
Reactivation of TB
387
What is adalimumab?
Monoclonal Ab
388
How is adalimumab administered?
SC
389
What cell does rituximab lead to depletion in?
B cells
390
How does abatacept work?
Fusion protein that modulates a key signal required for activation of T lymphocytes --> decreased T cell proliferation + cytokine production
391
Does psoriatic arthritis tend to occur before or after the skin changes?
Tends to precede
392
What % of pts with psoriasis skin lesions develop an arthropathy?
10-20%
393
What are the types of psoriatic arthropathy?
``` Rheumatoid like polyarthritis Asymmetric oligoarthritis (typically affects hands/feet) Sacroilitis DIP joint disease Arthritis mutilans ```
394
What is arthritis mutilans?
Severe deformity of fingers/hands - 'telescoping fingers'
395
How should you treat psoriatic arthropathy?
Like RA
396
What deformity may seen in hand x-ray of someone with psoaritic arthritis?
Pencil in cup deformity | Plantar spur
397
When is it appropriate to start trying to use anti-TNFa drugs in someone with axial AS?
If they have tried and failed on 2 different NSAIDs and meets criteria for active disease on 2 occasions 12w apart
398
List secondary causes of Raynaud's
Connective tissue dx (e.g. scleroderma most common) Leukaemia Type 1 cryoglonulinaemia, cold agglutinins Use of vibrating tools Drugs, e.g. COCP, ergot Cervical rib
399
What kind of crystals are seen in gout?
Monosodium urate | Needle-shaped, negatively birefringent under polarised light
400
What kind of crystals are seen in pseudogout?
Calcium pyrophosphate | Rhomboid positively birefringent under polarised light
401
What crystals are seen in OA?
Calcium phosphate due to degeneration of cartilage no birefringence
402
What crystals are see in RA?
Cholesterol crystals | Rhomboid + negatively birefringent
403
What mnemonic can be used to remember some causes of gout?
DART - diuretics, alcohol, kidney disease, trauma
404
If a patient on allopurinol for gout prophylaxis has an acute attack, should their allopurinol be stopped?
No - continue it
405
List possible differentials for polyarthritis
``` rheumatoid arthritis SLE seronegative spondyloarthropathies Henoch-Schonlein purpura sarcoidosis tuberculosis pseudogout viral infection: EBV, HIV, hepatitis, mumps, rubella ```
406
What are interferons and cytokines in the body released in response to?
Viral infections | Tumours
407
INF-a and INF-b bind to what type of receptors?
Type 1
408
What type of receptors does IFN-gamma bind to?
Type 2
409
What cells produce INFa?
Leucocytes
410
What is the action of INFa?
Antiviral action
411
In which conditions is INFa helpful?
Hepatitis B and C Kaposi sarcoma Metastatic renal cancer Hairy cell leukaemia
412
What are adverse effects of INFa?
Depression | Flu like symptoms
413
What cells produce INFb?
Fibroblasts
414
What is the action of INFb?
Antiviral
415
What condition is treated with INFb?
MS (can reduce freq. of exacerbations in RRMS)
416
What cells produce INF-g?
Natural killer cells mostly + T helper cells
417
What is the action of INF-g?
Antiviral action, but more of a role in immunomodulation, esp. macrophage activation
418
In what diseases may INF-g be helpful?
Chronic granulomatous disease, osteopetrosis
419
Why can TA cause visual symptoms?
Can cause occlusion of ophthalmic artery --> blindness (irreversible) Transient visual problems may precede complete occlusion
420
What is dactylitis?
Inflammation ofa digit
421
What can cause dactylitis?
Spondyloarthritis: psoriatic/reactive Sickle cell disease Rarer - TB, sarcoidosis, syphillis
422
What score is used to assess for hypermobility?
Beighton score
423
What beighton score is +ve in kids?
6/9
424
What beighton score is +ve in adults?
5/9
425
Why might you get a single central radiodense line in the spine of someone with AS?
Ossification of the supraspinous and infraspinous ligaments | Known as dagger sign
426
What is fibromyalgia?
Syndrome characterised by widespread pain throughout the body with tender points in specific areas
427
What is the causes of fibromyalgia?
Unknown
428
Which gender does fibromyalgia affect more?
Women
429
What are the features of fibromyalgia?
Chronic pain - at multiple sites or sometimes all over Lethargy Cognitive impairment - 'fibro fog' Sleep disturbance, headaches, dizziness
430
How is fibromyalgia diagnosed?
Clinically - American college of rheumatology classification criteria lists 9 pairs of tender points on body, if pt tendering at least 11 points fibromyalgia more likely
431
What is involved in the management of fibromyalgia?
Explanation Aerobic exercise CBT Medications - pregabalin, duloxetine, amitriptyline
432
What two things are required for diagnosis of AS?
Radiological feature - sacroiliitis Clinical feature NOTE: squaring of vertebrae and bamboo spine are all signs but are not part of the diagnostic criteria
433
How long may anti-CCP antibodies may detectable for before diagnosis of RA?
10 years
434
What test can you do to differentiate between statin induced myopathy and PMR?
ESR - | Raised in PMR, normal in statin induced myopathy
435
What ages tend to get Still's disease?
Bimodal age distribution - 15-25y and 35-46y
436
What are the features of Still's disease?
``` Arthalgia Elevated serum derritin Salmon pink, maculopapular rash Pyrexia Lympadenopathy RF and ANA -ve ```
437
What is the pattern of the pyrexia in Still's disease?
Typically rises in late afternoon/early evening in daily pattern + accompanies worsening of joint symptoms + rash
438
What criteria can be used to help diagnose still's disease?
Yamaguchi criteria
439
What is the management of Still's disease?
NSAIDs - first line to manage fever, joint pain + serositis Trial at least a week before steroids added If symptoms persist then consider methotrexate or IL-1/anti-TNF
440
What is the mechanism of action of penicillamine?
Thought to reduce IL-1 synthesis and prevent maturation of newly synthesized collagen
441
When might penicillamine be used?
RA Mx
442
What are adverse effects of penicillamine?
Rashes Taste disturbance Proteinuria
443
What can penicillamine cause in patients with Wilson's disease?
Membranous glomerulonephropathy
444
Offer prophylactic bisphosphonates to those with a T score of _____ if they are on steroids/going to be on steroids for 3+ months (even if <65y)
1.5
445
What does joint aspirate in RA show?
High WCC (predominantly PMNs) Yellow and cloudy No crystals
446
What are typical features of RA?
Swollen, painful joints in hands + feet Stiffness worse in morning Gradual gets worse with larger joints becoming involved Presentation usually insidious over a few m Positive squeeze test
447
What is a squeeze test?
Discomfort on squeezing across the metacarpal or metatarsal joints
448
What deformities are late features of RA?
Swan neck deformity and boutonniere deformities
449
What is palindromic rheumatism?
Relapsing/remitting monoarthritis of different large joints
450
What is chondrocalcinosis?
Calcification of the cartilage
451
What is antiphospholipid syndrome?
Acquired disorder characterised by predisposition to both venous and arterial thromboses, recurrent foetal loss, thrombocytopenia
452
What is the aetiology of antiphospholipid syndrome?
Primary disorder | Secondary to other conditions, e.g. SLE
453
Antiphospholipid syndrome causes a paradoxical increase in what?
APTT + low platelets - due to ex-vivo reaction of lupus anticoagulant autoantibodies with phospholipids involved in the coagulation cascade
454
What are the features of antiphospholipid syndrome?
``` Venous/arterial thrombosis Recurrent foetal loss Livedo reticularis Thrombocytopenia Prolonged APTT Pre-eclampsia, pulmonary HTN ```
455
What conditions are associated with antiphospholipid syndrome?
SLE - most common Other autoimmune disorders Lymphoproliferative disorders Phenothiazines
456
How should you manage antiphospholipid syndrome?
Initial VTE: warfarin with INR target of 2-3 for 6m Recurrent VTE - lifelong warfarin (increase target INR to 3-4 if VTE occur while on warfarin) Arterial thrombosis treat with lifelong warfarin aiming for INR 2-3
457
What might you see on the skull x-ray of someone with paget's?
Thickening of calvarium | Ill-defined sclerotic and lucent areas
458
What antibody is associated with antiphospholipid syndrome?
Anti-cardiolipin antibody and lupus anticoagulant (La)
459
What is sulfasalazine used to treat?
IBD | RA
460
What kind of drug is sulfasalazine?
5-ASA
461
How does sulfasalazine work?
Decreases neutrophil chemotaxis alongside supressing proliferation of lymphocytes + pro-inflammatory cytokines
462
What are cautions for sulfasalazine use?
G6PD deficiency | Allergy to aspirin/sulphonamides (e.g. co-trimoxazole)
463
What adverse effects are associated with sulfasalazine?
Olgiospermia SJS Pneumonitis/lung fibrosis Myelosupression, Heinz body anaemia, megaloblastic anaemia May colour tears --> stained contact lenses
464
Is sulfasalazine safe to use in pregnancy and breastfeeding?
Yes
465
When does OI tend to present?
Childhood
466
What are the features of OI?
Fractures following minor trauma Blue sclera Deafness secondary to otosclerosis Dental imperfections common
467
What things do you have to take into consideration when a patient with RA is undergoing surgery under GA?
Do lateral and anteroposterior cervical spine radiographs to screen for alantoaxial subluxation - to ensure pt goes into surgery with a Cspine collar and spine is not hyperextended on intubation
468
Why might you get a normal temporal biopsy in TA?
Skip lesions
469
What should be prescribed in RA to reduce need for NSAIDs/COX2 inhibitors?
Simple analgesia, e.g. paracetamol, codeine
470
What two blood tests must be negative to diagnose Still's disease?
RF and ANA
471
What is a very common SE of colchicine?
Diarrhoea
472
What are vasculitides?
Group of conditions characterised by inflammation of BV walls
473
What vasculitides affect the aorta and its branches?
Takayasu's arteritis Buergers disease Giant cell arteritis
474
What vasculitides affect large and medium sized arteries?
Buergers disease Giant cell arteritis Polyarteritis nodosa
475
What vasculitides affect medium sized muscular arteries?
Polyarteritis nodosa | Wegeners granulomatosis
476
What vasculitides affect small muscular arteries?
Wegeners granulomatosis | Rheumatoid vasculitis
477
What is Takayasu's arteritis?
Inflammatory, obliterative arteritis affecting aorta and its branches
478
What are features of Takayasu's arteritis?
Upper limb claudication Diminished/absent pulses ESR raised in acute phase
479
What is Buergers disease?
Segmental thrombotic occlusions of small and medium sized lower limb vessels
480
Who is Buergers disease most common in?
Young male smokers
481
What are the features of Buergers disease?
Proximal pulses usually present but pedal pulses loss | Acute hypercellular occlusive thrombus often present
482
What might you see on angiography in Buergers disease?
Tortuous corkscrew shaped collateral vessels
483
What kind of lesions might you see in GCA temporal artery biopsy?
Granulomatous lesions
484
What might you see on angiography in polyarteritis nodosa?
Saccular/fusiform aneurysms and arterial stenoses
485
What is Wegeners granulomatosis?
Systemic necrotising granulomatous vasculitis
486
What cutaneous lesions might you see in Wegeners granulomatosis?
Ulceration Nodules Purpura
487
What might you see in sinus imaging in Wegeners granulomatosis?
Mucosal thickening and air fluid levels
488
What are the main treatments for vasculitides?
Immunosupression
489
What HLA allele is associated with seronegative spondyloarthropathies?
HLA-B27
490
What does seronegativemean in seronegative spondyloarthropathies?
RF -ve
491
What are the common features of seronegative spondyloarthropathies?
Peripheral arthritis, usually asymmetrical Sacroilitis Enthensopathy, e.g. Achilles tendonitis, plantar fasciitis Extra-articular manifestations e.g. uveitis, pulmonary fibrosis (upper zone), amyloidosis, aortic regurg
492
What are the seronegative spondyloarthropathies?
ankylosing spondylitis psoriatic arthritis Reiter's syndrome (including reactive arthritis) enteropathic arthritis (associated with IBD)
493
What kind of hearing loss do you get in Paget's?
Sensorineural
494
After someone is on bisphosphonates for how many years can you consider stopping them?
5 years or 3 for IV zolendronate - reassess need for ongoing treatment, if T score >-2.5 can stop and review in 2y
495
What things would make you keep someone on bisphosphonates indefinitely?
``` Age >75 Glucocorticoid therapy Previous hip/vertebral fractures Further fractures on treatment High risk on FRAX scoring T score ```
496
What criteria can be used to diagnose RA?
2010 American College of Rheumatology criteria
497
Who should you consider a diagnosis of RA in?
Those with: 1. At least 1 joint with definite clinical synovitis 2. synovitis is not better explained by another disease
498
What score is needed on the 2010 American College of Rheumatology criteria for a diagnosis of RA?
6/10
499
What is the 2010 American College of Rheumatology criteria for a diagnosis of RA?
``` A) JOINT INVOLVEMENT 1 large joint = 0 2-10 large joints = 1 1-3 small joints = 2 4-10 small joints = 3 10 joints (at least 1 small) = 5 ``` B. SEROLOGY -ve RF + ACPA = 0 low +ve RF or ACPA = 2 high positive RF or ACPA = 3 C. ACUTE PHASE REACTANTS Normal CRP + ESR = 0 Abnormal CRP or ESR = 1 D. DURATION OF SYMPTOMS <6w = 0 >6w = 1
500
What investigation should you do before starting someone on TNF inhibitors?
CXR - TNF inhibitors can cause reactivation of TB

MSK (27 decks)

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