Rheumatology Flashcards
(69 cards)
1
Q
Erosive osteoarthritis
A
The gull-wing or inverted T pattern erosions on plain radiographs.
2
Q
Osteoarthritis affected joints
A
-DIP ( Heberden nodes)
-PIP ( Bouchards nodes)
-Base of thumb ( First carpometacarpal joint)
-Weight-bearing joints( Hips, Knees and spine)
3
Q
Rheumatoid Arthritis
A
Chronic symmetrical inflammatory polyarthritis.
Symmetrical MCP, PIP, wrist joint synovitis.
In arthroscopy, Marked vascular proliferation on the synovial membrane
4
Q
Rheumatoid Arthritis ( Characteristic Deformities)
A
- Ulnar deviation of MCP joints
- Boutnonniere deformities of fingers
- Swan neck deformities of fingers
- Z deformity of thumbs
5
Q
Rheumatoid Arthritis Investigations
A
- RF
-anti-CCP
-ESR/CRP, CRP can be used to track the severity of disease flare-ups
6
Q
Rheumatoid Arthritis Radiology
A
EARLY CHANGES
-soft tissue swelling
- juxta-articular osteoporosis
- Osteopenia
INTERMEDIATE CHANGES
-Joint space narrowing due to loss of cartilage
LATE CHANGES
-Bone and joint destruction
-Subluxation
7
Q
Feltys syndrome
A
-RA, Splenomegaly, and Neutropenia
8
Q
RA Treatment
A
DMARDS
-Methotrexate
-Infliximab
-Adalimumab
9
Q
Worse prognosis in RA
A
- Female sex
- Gradual onset over a few months
- Positive IgM Rheumatoid factor
- Anemia develops within 3 months of the onset
-Anti-CCP antibody positivity
10
Q
Systemic sclerosis
A
- Systemic sclerosis is a chronic autoimmune disease characterised by increased fibroblast activity and fibrosis in a number of different organ systems. 90-95% of patients have positive antinuclear antibodies. There are two major subtypes: limited cutaneous and diffuse cutaneous. CREST syndrome is an older term for the limited cutaneous form (calcinosis, Raynauds’ phenomenon, oeosophageal dysmotility, sclerodactyly, telangiectasia).
- Pulmonary hypertension is a typical late complication of systemic sclerosis.
- Anti-centromere antibodies are associated with an increased risk of pulmonary hypertension.
11
Q
RF
A
- Rheumatoid factor is an antibody with reactivity to the heavy chain of IgG.
- The conventional test, detects only IgM RF.
12
Q
Trochanteric bursitis
A
A global reduction in movement affecting the right hip, and point tenderness over the trochanter indicate trochanteric bursitis.
13
Q
Action of bisphosphonates
A
Inhibits osteoclast activity
14
Q
Systemic sclerosis
A
Anti- centromere antibody- Limited cutaneous, plum HTN, good prognosis
Anti- topoisomerase 1 antibody- Diffuse cutaneous, Pulm fibrosis, high mortality
15
Q
Methotrexate
A
It acts by inhibition of dihydrofolate reductase thus depleting folate concentrations.
16
Q
Psoriasis
A
The commonest form of psoriasis is plaque psoriasis, making up approximately 80% of cases (guttate -10%, erythrodermic - 3%, pustular - 3%).
Studies report:
- a 5-42% prevalence of psoriatic arthropathy in patients with cutaneous psoriasis
- arthropathy precedes cutaneous lesions in 20%
- cutaneous lesions precede joint disease in 60-70%, and
- They occur simultaneously in 10-20%.
17
Q
Ocular Manifestations of Rheumatoid Arthritis
A
Ocular manifestations of rheumatoid arthritis are common, with 25% of patients having eye problems
Ocular manifestations
keratoconjunctivitis sicca (most common)
episcleritis (erythema)
scleritis (erythema and pain)
corneal ulceration
keratitis
Iatrogenic
steroid-induced cataracts
chloroquine retinopathy
18
Q
Anti- Ro
A
SLE - antibodies associated with congenital heart block = anti-Ro
19
Q
Lofgren’s syndrome
A
A pattern of sarcoidosis symptoms encompassing fever, joint pain, erythema nodosum, lymphadenopathy and bilateral hilar lymphadenopathy. Lofgren’s syndrome has a good prognosis and does not usually require any treatment; it will self-resolve within a year or so.
Indications for immunosuppressive treatment include splenic/hepatic/renal/cardiac involvement, lupus pernio, hypercalcemia, eye/CNS involvement or deteriorating pulmonary function tests or deteriorating chest x-ray changes.
20
Q
Sarcoidosis
A
Sarcoidosis is a multisystem disorder of unknown aetiology characterised by non-caseating granulomas. It is more common in young adults and in people of African descent. Sarcoidosis remits without treatment in approximately two-thirds of people
Factors associated with poor prognosis
- Insidious onset, symptoms > 6 months
- Absence of erythema nodosum
- extrapulmonary manifestations: e.g. lupus pernio, splenomegaly
- CXR: stage III-IV features
- black African or African-Caribbean ethnicity
21
Q
PAN
A
Polyarteritis nodosa (PAN) is a vasculitis affecting medium-sized arteries with necrotizing inflammation leading to aneurysm formation. PAN is more common in middle-aged men and is associated with hepatitis B infection.
Features
fever, malaise, arthralgia
weight loss
hypertension
mononeuritis multiplex, sensorimotor polyneuropathy
testicular pain
livedo reticularis
haematuria, renal failure
perinuclear-antineutrophil cytoplasmic antibodies (ANCA) are found in around 20% of patients with ‘classic’ PAN
hepatitis B serology positive in 30% of patients
22
Q
Rapidly progressive glomerulonephritis
A
Rapidly progressive glomerulonephritis is a term used to describe a rapid loss of renal function associated with the formation of epithelial crescents in the majority of glomeruli.
Causes
- Goodpasture’s syndrome
-Wegener’s granulomatosis ( C-ANCA)
- Others: SLE, microscopic polyarteritis
Features
- Nephritic syndrome: haematuria with red cell casts, proteinuria.
- hypertension, oliguria
features specific to underlying cause (e.g. haemoptysis with Goodpasture’s, vasculitic rash or sinusitis with Wegener’s)
23
Q
Reactive Arthritis
A
Reactive arthritis: develops after an infection where the organism cannot be recovered from the joint
24
Q
A
25
Ankylosing spondylitis
Ankylosing spondylitis is a HLA-B27 associated spondyloarthropathy. It typically presents in males (sex ratio 3:1) aged 20-30 years old.
Features
typically a young man who presents with lower back pain and stiffness of insidious onset
stiffness is usually worse in the morning and improves with exercise
the patient may experience pain at night which improves on getting up
Clinical examination
reduced lateral flexion
reduced forward flexion - Schober's test - a line is drawn 10 cm above and 5 cm below the back dimples (dimples of Venus). The distance between the two lines should increase by more than 5 cm when the patient bends as far forward as possible
reduced chest expansion
Other features - the 'A's
Apical fibrosis
Anterior uveitis
Aortic regurgitation
Achilles tendonitis
AV node block
Amyloidosis
and cauda equina syndrome
peripheral arthritis (25%, more common if female)
26
Adhesive capsulitis
External rotation (on both active and passive movement) is classically impaired in adhesive capsulitis
27
Supraspinatus tendonosis and torn rotator cuff
The most prominent restriction in movement would be impaired abduction.
28
Supraspinatus tendonosis and torn rotator cuff
The most prominent restriction in movement would be impaired abduction.
29
Gout
Gout is a form of microcrystal synovitis caused by the deposition of monosodium urate monohydrate in the synovium. It is caused by chronic hyperuricaemia (uric acid > 450 µmol/l)
Acute management
NSAIDs or colchicine are first-line
the maximum dose of NSAID should be prescribed until 1-2 days after the symptoms have settled
gastroprotection (e.g. a proton pump inhibitor) may also be indicated
colchicine
inhibits microtubule polymerization by binding to tubulin, interfering with mitosis. Also inhibits neutrophil motility and activity
has a slower onset of action
may be used with caution in renal impairment: the BNF advises to reduce the dose if eGFR is 10-50 ml/min and to avoid if eGFR < 10 ml/min BNF
the main side-effect is diarrhoea
oral steroids may be considered if NSAIDs and colchicine are contraindicated.
a dose of prednisolone 15mg/day is usually used
another option is intra-articular steroid injection
if the patient is already taking allopurinol it should be continued
Indications for urate-lowering therapy (ULT)
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
Urate-lowering therapy
it has traditionally been taught that urate-lowering therapy should not be started until 2 weeks after an acute attack, as starting too early may precipitate a further attack. The evidence base to support this however looks weak
in 2017 the BSR updated their guidelines. They still support a delay in starting urate-lowering therapy because it is better for a patient to make long-term drug decisions whilst not in pain
the key passage is: 'Commencement of ULT is best delayed until inflammation has settled as ULT is better discussed when the patient is not in pain'
allopurinol is first-line
initial dose of 100 mg od, with the dose titrated every few weeks to aim for a serum uric acid of < 360 µmol/l
a lower target uric acid level below 300 µmol/L may be considered for patients who have tophi, chronic gouty arthritis or continue to have ongoing frequent flares despite having a uric acid below 360 µmol/L
a lower initial dose of allopurinol should be given if the patient has a reduced eGFR
colchicine cover should be considered when starting allopurinol. NSAIDs can be used if colchicine cannot be tolerated. The BSR guidelines suggest this may need to be continued for 6 months
the second-line agent when allopurinol is not tolerated or ineffective is febuxostat (also a xanthine oxidase inhibitor)
in refractory cases other agents may be tried:
uricase (urate oxidase) is an enzyme that catalyzes the conversion of urate to the degradation product allantoin. It is present in certain mammals but not humans
in patients who have persistent symptomatic and severe gout despite the adequate use of urate-lowering therapy, pegloticase (polyethylene glycol modified mammalian uricase) can achieve rapid control of hyperuricemia. It is given as an infusion once every two weeks
Lifestyle modifications
reduce alcohol intake and avoid during an acute attack
lose weight if obese
avoid food high in purines e.g. Liver, kidneys, seafood, oily fish (mackerel, sardines) and yeast products
Other points
consideration should be given to stopping precipitating drugs (such as thiazides)
losartan has a specific uricosuric action and may be particularly suitable for the many patients who have coexistent hypertension
- increased vitamin C intake (either supplements or through normal diet) may also decrease serum uric acid levels.
30
Role of Pilocarpine in Sjogren syndrome
Pilocarpine is a cholinergic agonist that stimulates muscarinic receptors to increase saliva production. This patient’s presentation of xerostomia, difficulty swallowing, and increased dental caries suggests Sjögren’s syndrome, where the immune-mediated destruction of exocrine glands impairs salivary flow. Pilocarpine is commonly used to treat dry mouth in such patients, as it enhances saliva secretion and provides relief from the symptoms of oral dryness and difficulty swallowing, improving both the patient’s ability to eat and speak. Therefore, pilocarpine is the most appropriate treatment for this patient’s dry mouth and associated difficulties.
31
Sjogren's Syndrome
Sjogren's syndrome is an autoimmune disorder affecting exocrine glands resulting in dry mucosal surfaces. It may be primary (PSS) or secondary to rheumatoid arthritis or other connective tissue disorders, where it usually develops around 10 years after the initial onset. Sjogren's syndrome is much more common in females (ratio 9:1). There is a marked increased risk of lymphoid malignancy (40-60 fold).
Features
- dry eyes: keratoconjunctivitis sicca
- dry mouth
- vaginal dryness
- arthralgia
- Raynaud's, myalgia
- sensory polyneuropathy
- recurrent episodes of parotitis
- Renal tubular acidosis (usually subclinical)
Investigation
- Rheumatoid factor (RF) positive in nearly 50% of patients
- ANA positive in 70%
- anti-Ro (SSA) antibodies in 70% of patients with PSS
- anti-La (SSB) antibodies in 30% of patients with PSS
- Schirmer's test: filter paper near conjunctival sac to measure tear formation
Histology: focal lymphocytic infiltration
also: hypergammaglobulinaemia, low C4
Management
- artificial saliva and tears
- Pilocarpine may be helpful to stimulate saliva production
32
Management of patients at risk of corticosteroid-induced osteoporosis
The RCP guidelines essentially divide patients into two groups.
1. Patients over the age of 65 years or those who've previously had a fragility fracture should be offered bone protection.
2. Patients under the age of 65 years should be offered a bone density scan, with further management dependent:
T score Management
Greater than 0 Reassure
Between 0 and -1.5 Repeat bone density scan in 1-3 years
Less than -1.5 Offer bone protection
The first-line treatment is alendronate. Patients should also be calcium and vitamin D replete.
33
Rickets
- Aching bones and joints
lower limb abnormalities:
- widening of the wrist joints due to an excess of non-mineralized osteoid at the growth plate
- In toddlers genu varum (bow legs)
in older children - genu valgum (knock knees)
- 'rickety rosary' - swelling at the costochondral junction
- kyphoscoliosis
- craniotabes - soft skull bones in early life
- Harrison's sulcus
34
Granulomatosis with polyangiitis
Consider granulomatosis with polyangiitis when a patient presents with ENT, respiratory and kidney involvement
35
Secondary causes of Raynaud's Phenomenon
- connective tissue disorders
scleroderma (most common)
rheumatoid arthritis
systemic lupus erythematosus
- leukaemia
- type I cryoglobulinaemia, cold agglutinins
- Use of vibrating tools
- drugs: oral contraceptive pill, ergot
- Cervical rib
36
Anakinra
Anakinra is an interleukin-1 (IL-1) receptor antagonist. Interleukin-1 refers to a 'superfamily' of 11 cytokines that are responsible for acute inflammation and inducing fever. Blocking IL-1 with anakinra is beneficial in pro-inflammatory conditions such as rheumatoid arthritis. Il-1 is predominantly secreted by macrophages
37
Ankylosing Spondylitis
Syndesmophytes (ossification of outer fibres of annulus fibrosus) are a feature of ankylosing spondylitis
38
Ankylosing Spondylitis
- Ankylosing spondylitis is a HLA-B27 associated spondyloarthropathy. It typically presents in males (sex ratio 3:1) aged 20-30 years old.
- Plain x-ray of the sacroiliac joints is the most useful investigation in establishing the diagnosis. - Radiographs may be normal early in disease, later changes include:
- sacroiliitis: subchondral erosions, sclerosis
- squaring of lumbar vertebrae
- 'bamboo spine' (late & uncommon)
- syndesmophytes: due to ossification of outer fibers of annulus fibrosus
- chest x-ray: apical fibrosis
39
HLA associations
HLA antigens are encoded for by genes on chromosome 6. HLA A, B and C are class I antigens whilst DP, DQ, DR are class II antigens. Questions are often based around which diseases have strong HLA associations. The most important associations are listed below:
HLA-A3
haemochromatosis
HLA-B51
Behcet's disease
HLA-B27
ankylosing spondylitis
reactive arthritis
acute anterior uveitis
psoriatic arthritis
HLA-DQ2/DQ8
coeliac disease
HLA-DR2
narcolepsy
Goodpasture's
HLA-DR3
dermatitis herpetiformis
Sjogren's syndrome
primary biliary cirrhosis
HLA-DR4
type 1 diabetes mellitus*
rheumatoid arthritis - in particular the DRB1 gene (DRB1*04:01 and DRB1*04:04 hence the association with DR4)
40
RA
The HLA-DRB1 gene is the major genetic susceptibility locus for rheumatoid arthritis (DRB1*04:01 and DRB1*04:04 hence the association with DR4
41
Ankylosing spondylitis
Plain x-ray of the sacroiliac joints is the most useful investigation in establishing the diagnosis. Radiographs may be normal early in disease, later changes include:
sacroiliitis: subchondral erosions, sclerosis
squaring of lumbar vertebrae
'bamboo spine' (late & uncommon)
syndesmophytes: due to ossification of outer fibers of annulus fibrosus
chest x-ray: apical fibrosis
42
Relapsing polychondritis
Ears: auricular chondritis, hearing loss, vertigo
Nasal: nasal chondritis → saddle-nose deformity
Respiratory tract: e.g. hoarseness, aphonia, wheezing, inspiratory stridor
Ocular: episcleritis, scleritis, iritis, and keratoconjunctivitis sicca
Joints: arthralgia
Less commonly: cardiac valcular regurgitation, cranial nerve palsies, peripheral neuropathies, renal dysfunction
43
Psoriatic Arthritis
Inflammatory arthritis involving DIP swelling and dactylitis points to a diagnosis of psoriatic arthritis
44
RA- Ocular manifestations
Ocular manifestations of rheumatoid arthritis are common, with 25% of patients having eye problems
Ocular manifestations
keratoconjunctivitis sicca (most common)
episcleritis (erythema)
scleritis (erythema and pain)
corneal ulceration
keratitis
Iatrogenic
steroid-induced cataracts
chloroquine retinopathy
45
Lambert-Eaton myasthenic syndrome
Lambert-Eaton myasthenic syndrome is seen in association with small cell lung cancer and to a lesser extent breast and ovarian cancer. It may also occur independently as an autoimmune disorder. Lambert-Eaton myasthenic syndrome is caused by an antibody directed against presynaptic voltage-gated calcium channel in the peripheral nervous system.
Features
repeated muscle contractions lead to increased muscle strength
46
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48
Psoriasis
The following factors may exacerbate psoriasis:
trauma
alcohol
drugs: beta blockers, lithium, antimalarials (chloroquine and hydroxychloroquine), NSAIDs and ACE inhibitors, infliximab
withdrawal of systemic steroids
49
Osteoarthritis of the Hands
Usually bilateral: Usually one joint at a time is affected over a period of several years. The carpometacarpal joints (CMCs), distal interphalangeal joints (DIPJs) are affected more than the proximal interphalangeal joints (PIPJs).
Episodic joint pain: An intermittent ache. Provoked by movement and relieved by resting the joint.
Stiffness
worse after long periods of inactivity e.g. waking up in the morning
stiffness lasts only a few minutes compared to the morning joint stiffness seen in rheumatoid arthritis.
Painless nodes (bony swellings)
Heberden's nodes at the DIP joints
Bouchard's Nodes at the PIP joints
these nodes are the result of osteophyte formation.
Squaring of the thumbs: Deformity of the carpometacarpal joint of the thumb resulting in fixed adduction of the thumb.
50
Osteoarthritis
NICE recommend co-prescribing a PPI with NSAIDs in all patients with osteoarthritis
51
Bisphosphonates
'Tablets should be swallowed whole with plenty of water while sitting or standing; to be given on an empty stomach at least 30 minutes before breakfast (or another oral medication); patient should stand or sit upright for at least 30 minutes after taking tablet'
52
Osteoporosis: DEXA scan
Osteoporosis: DEXA scan
Basics
T score: based on bone mass of young reference population
T score of -1.0 means bone mass of one standard deviation below that of young reference population
Z score is adjusted for age, gender and ethnic factors
T score
> -1.0 = normal
-1.0 to -2.5 = osteopaenia
< -2.5 = osteoporosis
53
pseudogout
Pseudogout is a form of microcrystal synovitis caused by the deposition of calcium pyrophosphate dihydrate crystals in the synovium. For this reason, it is now more correctly termed acute calcium pyrophosphate crystal deposition disease.
Pseudogout is strongly associated with increasing age. Patients who develop pseudogout at a younger age (e.g. < 60 years) usually have some underlying risk factor, such as:
haemochromatosis
hyperparathyroidism
low magnesium, low phosphate
acromegaly, Wilson's disease
54
Pseudogout
Features
knee, wrist and shoulders most commonly affected
joint aspiration: weakly-positively birefringent rhomboid-shaped crystals
x-ray: chondrocalcinosis
in the knee this can be seen as linear calcifications of the meniscus and articular cartilage
Management
aspiration of joint fluid, to exclude septic arthritis
NSAIDs or intra-articular, intra-muscular or oral steroids as for gout
55
Lofgren's syndrome
Lofgren's syndrome is an acute form sarcoidosis characterised by bilateral hilar lymphadenopathy (BHL), erythema nodosum, fever and polyarthralgia.
56
RA
Early X-ray findings
Loss of joint space
juxta-articular osteoporosis
soft-tissue swelling
periarticular erosions
subluxation.
>> Periarticular erosions are the most supportive of the diagnosis of RA.
57
Bisphosphonates
Clinical uses
prevention and treatment of osteoporosis
hypercalcaemia
Paget's disease
pain from bone metatases
58
59
HLA-B27
HLA-B27
- Ankylosing spondylitis
- reactive arthritis
- acute anterior uveitis
- psoriatic arthritis
60
Supraspinatus tendonitis
A 55-year-old woman presents with a four week history of shoulder pain. There has been no obvious precipitating injury and no previous experience. The pain is worse on movement and there is a grating sensation if she moves the arm too quickly. She also gets pain at night, particularly when she lies on the affected shoulder. On examination there is no obvious erythema or swelling. Passive abduction is painful between between 60 and 120 degrees. She is unable to abduct the arm herself past 70-80 degrees. Flexion and extension are preserved.
-This patient has a classic 'painful arc' which is a sign of shoulder impingement, most commonly secondary to supraspinatus tendonitis.
61
Rotator Cuff Muscles
( SITS)
>>Supraspinatus
aBDucts arm before deltoid
Most commonly injured
>>Infraspinatus
Rotates arm laterally
>>teres minor
aDDucts & rotates arm laterally
>>Subscapularis
aDDucts & rotates arm medially
62
Vibrating Tools
Use of vibrating tools may result in Raynaud's phenomenon
63
Myotonic Dystrophy
Genetics
autosomal dominant
a trinucleotide repeat disorder
DM1 is caused by a CTG repeat at the end of the DMPK (Dystrophia Myotonica-Protein Kinase) gene on chromosome 19.
DM2 is caused by a repeat expansion of the ZNF9 gene on chromosome 3
64
Myotonic Dystrophy
General features
myotonic facies (long, 'haggard' appearance)
frontal balding
bilateral ptosis
cataracts
dysarthria
Other features
myotonia (tonic spasm of muscle)
weakness of arms and legs (distal initially)
mild mental impairment
diabetes mellitus
testicular atrophy
cardiac involvement: heart block, cardiomyopathy
dysphagia
65
Extractable Nuclear Antigens
Examples
anti-Ro: Sjogren's syndrome, SLE, congenital heart block
anti-La: Sjogren's syndrome
anti-Jo 1: polymyositis
anti-scl-70: diffuse cutaneous systemic sclerosis
anti-centromere: limited cutaneous systemic sclerosis
66
Polyarteritis nodosa (PAN)
Polyarteritis nodosa (PAN) is a vasculitis affecting medium-sized arteries with necrotizing inflammation leading to aneurysm formation. PAN is more common in middle-aged men and is associated with hepatitis B infection.
Features
fever, malaise, arthralgia
weight loss
hypertension
mononeuritis multiplex, sensorimotor polyneuropathy
testicular pain
livedo reticularis
haematuria, renal failure
perinuclear-antineutrophil cytoplasmic antibodies (ANCA) are found in around 20% of patients with 'classic' PAN
hepatitis B serology positive in 30% of patients
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