MSK Flashcards
(132 cards)
1
Q
what is osteoarthritis?
A
it is often described as wear and tear in joints.
NOT an inflammatory condition
occurs in the synovial joints and is a result of a combination of genetic factors, overuse and injury.
there is loss of cartilage
remodelling of adjacent bone
associated inflammation
2
Q
what are the risk factors for osteoarthritis?
A
age >50 female obesity genetic factors trauma fam history
3
Q
what are the four key changes seen on an X-ray of a patient with osteoarthritis?
A
LOSS
L- loss of joint space
O - osteophytes forming in joint margins
S - subarticular sclerosis - increased density of the bone along the joint line
S - subchondral cysts - fluid-filled holes in bones
X-ray changes do not necessarily correlate with symptoms. Significant changes might be found incidentally on someone without symptoms. Equally, someone with severe symptoms of osteoarthritis may only have mild changes on X-ray
4
Q
how does osteoarthritis present?
A
pain - the pain tends to be worsened by activity (pain at rest or at night is unusual)
reduced joint function
bony deformities - common in the hands and lead to enlargement of the proximal interphalangeal joints (Bouchard’s nodes) and distal interphalangeal (DIP) joints (Heberden’s nodes), as well as squaring at the base of the thumb.
limited range of movement
joint instability
they may have joint tenderness and crepitus
5
Q
what joints are commonly affected in osteoarthritis?
A
often large weight-bearing joints - hips, knees, sacroiliac joint DIPs MCP joint at the base of the thumb wrist cervical spine
6
Q
what investigations do you perform for suspected osteoarthritis?
A
if the patient is over 45, has typical activity-related pain and no morning stiffness NICE suggests that a diagnosis can be made without any investigations.
investigations that can be performed are
X-ray affected joints
serum CPR and ESR
7
Q
how is osteoarthritis managed?
A
All patients should be offered help with weight loss, given advice about local muscle strengthening exercises and general aerobic fitness
1st line - topical analgesia e.g capsaicin topical, methylsalicylate, diclofenac topical (topical analgesia is usually only offered for knee and hand
2nd line - paracetamol plus topical analgesia
3rd line - NSAIDs can be added and also consider prescribing PPI to protect their stomach (omeprazole)
4th line - opioid - consider opiates such as codeine and morphine - should be used cautiously
other options:
- Intra-articular steroid injections
- Joint replacement
8
Q
what is another name for joint replacement?
A
arthroplasty
9
Q
after hip replacement how long should LMWH be given for?
A
4 weeks as there is an increased risk of thromboembolism.
10
Q
what is a baker’s cyst?
A
aka popliteal cyst - not true cysts but rather distension of the gastrocnemius-semimembranosus bursa
primary: no underlying pathology - typically seen in children
secondary - underlying condition such as OA - typically seen in adults
they present as swellings in the popliteal fossa behind the knee. Rupture may occur resulting in simial symptoms to DVT i.e. pain, redness and swelling in the calf however the majority of ruptures are asymptomatic
11
Q
what is cervical spondylosis?
A
cervical spndylosis is and extremely common condition that results from OA
peresents as neck pain although referred pain may mimic headaches
complications include radiculopathy and myelopathy
12
Q
what is rheumatoid arthritis?
A
it is an autoimmune condition that causes chronic inflammation of the synovial lining of the joints, tendon sheaths and bursa.
It is an inflammatory arthritis - a symmetrical poly-arthritis
13
Q
what are the genetic associations of rheumatoid arthritis?
A
HLA DR4
HLA DR1
14
Q
What are the antibodies involved in rheumatoid arthritis?
A
Rheumatoid factor - a circulating antibody, usually, IgM, which reacts with the Fc portion of the patients own IgG.
RF is positive in 70-80% of patients with RA - high titre levels are associated with severe progressive disease (but not a marker of disease activity.
Anti-cyclic citrullinated peptide antibody - may be detectable up to 10 years before the development of RA - it may therefore play a key role in the future of RA, allowing early detection of patients suitable for aggressive anti-TNF therapy.
15
Q
how can RF be detected?
A
rose-waaler test: sheep red cell agglutination
latex agglutination test
16
Q
what conditions other that RA are associated with positive RF?
A
Sjogren's syndrome Felty's syndrome infective endocarditis SLE systemic sclerosis 30% of the general population will have positive RF
17
Q
how does RA present?
A
swollen, painful joints in the hands and feet (typically the MCP and PIP in the hands and MTP in the feet)
stiffness worse in the morning
gradually gets worse with larger joints becoming involved (knees, shoulders, elbows, ankles)
presentation will usually develop over a few months
positive squeeze test - discomfort on squeezing across the metacarpal or metatarsal joints
18
Q
what is the difference between pain on OA and RA?
A
RA - worse at rest but improves with activity
OA - worse with activity and improves with rest
19
Q
what the extra-articular manifestations of RA?
A
ocular manifestations - keratoconjunctivitis, episcleritis, scleritis, corneal ulcerations, keratitis
respiratory manifestations - pulmonary fibrosis, pulmonary effusion, pulmonary nodules, bronchiolitis obliterans
Felty’s syndrome - RA, neutropenia and splenomegaly)
secondary sjogren’s syndrome - aka sicca syndrome
CV disease as RA increases risk of atherosclerosis
20
Q
what are poor prognostic features of RA?
A
anti-ccp antibodies RF positive poor functional status at presentation HLA DR4 x-ray - early erosions extra-articular features insidious onset
21
Q
what deformities occur in RA?
A
ulnar deviation
Boutonniere’s deformity
swan neck deformity
bakers cysts in the back of the knee
22
Q
what investigations would you perform for rheumatoid arthritis?
A
NICE have stated that clinical diagnosis is more
RF and anti CCP antibody
X-ray of the hands and feet
inflammatory markers - CRP and ESR
USS of the joints can be used to evaluate and confirm synovitis - it is particularly useful where the findings of the clinical examination are unclear.
23
Q
what x-ray changes would you see in RA?
A
joint destruction and deformity
soft tissue swelling
periarticular osteopenia
boney erosions
24
Q
what are the NICE guidelines for referral for RA??
A
NICE recommend referral for any adult with persistent synovitis, even if they have negative rheumatoid factor, anti-ccp antibodies and inflammatory markers - the referral should be urgent if it involves the small joints of the hands or feet, multiple joints or symptoms that have be present for more than 3 months.
25
what are the different diagnostic criteria for RA?
diagnostic criteria come from the American college of rheumatology (ACR)/ European league against Rheumatism (ELAR)
patients are scored based on
1. the joints that are involved (more and smaller joints are scored higher)
2. serology (rheumatoid factor and anti-CCP)
3. inflammatory markers (ESR and CRP)
4. duration of symptoms (more or less than 6 weeks)
scores are added up and a score greater than or equal to 6 indicates diagnosis of rheumatoid arthritis
26
what score is useful for monitoring disease activity and response to treatment in RA?
```
the DAS28 score
the DAS28 score is the disease activity score - it is based on the assessment for 28 joints and points are given for
- swollen joints
- tender joints
- ESR/CRP result
```
27
what is first and second line management of RA?
first line is mono-therapy with a DMARD
methotrexate, leflunomide or sulfasalazine
hydroxychloroquine can be considered in mild disease as it is considered the mildest anti-rheumatic drug
often a short course of prednisolone is started in combination
second line is 2 of these used in combination
28
what is third and fourth line management of RA?
third line - methotrexate plus a biological therapy - usually a TNF inhibitor
forth line is methotrexate plus rituximab
**remember TNF inhibitors all lead to immunosuppression so patients are prone to serious infection - they can also lead to reactivation of dormant infections such as TB and hep B.
29
how does methotrexate work and the side effects?
methotrexate works by interfering with the metabolism of folate and suppressing certain components of the immune system. It is taken by injection or tablet once a week. Folic acid 5mg is also prescribed once a week to be taken on a different day to methotrexate.
side effects:
- mouth ulcers and mucositis
- liver toxicity
- pulmonary fibrosis
- bone marrow suppression and leukopenia (low white blood cells)
- it is teratogenic - need to be avoided prior to conception in mothers and fathersm
30
how do anti TNF drugs work?
what are some side effects of anti TNF drugs?
what are some examples of anti TNF drugs?
tumour necrosis factor is a cytokine involved in stimulating inflammation. Blocking TNF reduces inflammation.
side effects - vulnerability to severe infections and sepsis, reactivation of TB and hep B
examples
- adalimumab
- infliximab
- golimumab
- certolizumab pegol
- etanercept
adalimumab, infliximab, golimumab and certolizumab pegol are monoclonal antibodies to TNF. Etanercept is a protein that binds TNF to the Fc portion of IgG and thereby reduces its activity
31
what is Rituximab? and what are the notable side effects?
Rituximab is monoclonal antibody that targets CD20 protein on the surface of B cells. this causes destruction of B cells. It is used for immunosuppression for autoimmune conditions such as rheumatoid arthritis and cancers relating to B cells
side effects
- vulnerability to severe infection and sepsis
- night sweats
- thrombocytopenia
- peripheral neuropathy
- liver and lung toxicity
32
what are the side effects of sulfasalazine ?
rashes
oligospermia
Heinz body anaemia
bone marrow suppression
33
what are the side effects of leflunomide?
liver impairment
interstitial lung disease
hypertension
peripheral neuropathy
34
what are the side effects of hydroxychloroquine?
reduced visual acuity
| nightmares
35
what is osteoporosis?
a complex skeletal diseases characterised by low bone density and micro-architectural defects in bone tissue resulting in increased bone fragility and susceptibility to fracture.
It is defined as bone mineral density of less than -2.5 standard deviations from the mean of a young adult
36
what are major risk factors for osteoporosis?
```
corticosteroid use
smoking
alcohol
low body mass index
family history
```
others:
age, female, post-menopausal, diabetes, vitamin D deficiency, low physical activity, hypothyroidism, CKD
37
What investigations would you perform for osteoporosis?
Dual-energy X-ray absorptiometry (DEXA) - work out a T score by measuring bone density at the hip
* t score is the number of standard deviations from the mean bone density of a 30 year old adult. Z score represents the the number of standard deviations the patients bone density fall below the mean for their age.
* DEXA scan be performed about every 2 years after diagnosis of osteoporosis
FRAX - fracture risk assessment tool - this gives a prediction of the risk of a fragility fracture over the net 10 years.
38
how is BMD classified depending on T score
More than -1 SD- BMD Normal
-1 to -2.5 SD- BMD = Osteopenia
Less than -2.5 S- BMD = Osteoporosis
Less than -2.5 plus a fracture - Severe Osteoporosis
39
how is osteoporosis managed?
lifestyle changes: activity and exercise, maintain adequate health weight, adequate vitamin D, avoid falls, stop smoking, reduce alcohol
Bisphosphonates are first line (alendronate, Risedronate, zolendronic)
If at risk of fractures - calcium with vitamin D (colecalciferol)
40
what are the side effects of bisphosphonates?
Reflux and oesophageal erosions. Oral bisphosphonates are taken on an empty stomach sitting upright for 30 minutes before moving or eating to prevent this.
Atypical fractures (e.g. atypical femoral fractures)
Osteonecrosis of the jaw
Osteonecrosis of the external auditory canal
41
if bisphosphonates are contraindicated in someone with osteoporosis what other options do you have?
Denoxumab is a monoclonal antibody that works by blocking the activity of osteoclasts.
Strontium ranelate is a similar element to calcium that stimulates osteoblasts and blocks osteoclasts but increases the risk of DVT, PE and myocardial infarction.
Raloxifene is used as secondary prevention only. It is a selective oestrogen receptor modulator that stimulates oestrogen receptors on bone but blocks them in the breasts and uterus.
Hormone replacement therapy should be considered in women that go through the menopause early.
42
what medications other than glucocorticoids may worsen osteoporosis?
```
SSRIs
antiepileptics
PPI
glitazones
long term herparin therapy
aromatase inhibitors e.g. anastrozole
```
43
who should be assessed for osteoporosis?
They advise that all women aged >= 65 years and all men aged >= 75 years should be assessed. Younger patients should be assessed in the presence of risk factors, such as:
previous fragility fracture
current use or frequent recent use of oral or systemic glucocorticoid
history of falls
family history of hip fracture
other causes of secondary osteoporosis
low body mass index (BMI) (less than 18.5 kg/m²)
smoking
alcohol intake of more than 14 units per week for women and more than 14 units per week for men.
44
what methods are used to assess risk of osteoporosis?
NICE recommend using a clinical prediction tool such as FRAX or QFracture to assess a patients 10 year risk of developing a fracture. This is analogous to the cardiovascular risk tools such as QRISK.
FRAX
estimates the 10-year risk of fragility fracture
valid for patients aged 40-90 years
based on international data so use not limited to UK patients
assesses the following factors: age, sex, weight, height, previous fracture, parental fracture, current smoking, glucocorticoids, rheumatoid arthritis, secondary osteoporosis, alcohol intake
bone mineral density (BMD) is optional, but clearly improves the accuracy of the results. NICE recommend arranging a DEXA scan if FRAX (without BMD) shows an intermediate result
QFracture
estimates the 10-year risk of fragility fracture
developed in 2009 based on UK primary care dataset
can be used for patients aged 30-99 years (this is stated on the QFracture website, but other sources give a figure of 30-85 years)
includes a larger group of risk factors e.g. cardiovascular disease, history of falls, chronic liver disease, rheumatoid arthritis, type 2 diabetes and tricyclic antidepressants
45
when would you use a DEXA rather than one of the clinical prediction tools for osteoporosis?
before starting treatments that may have a rapid adverse effect on bone density (for example, sex hormone deprivation for treatment for breast or prostate cancer).
in people aged under 40 years who have a major risk factor, such as history of multiple fragility fracture, major osteoporotic fracture, or current or recent use of high-dose oral or high-dose systemic glucocorticoids (more than 7.5 mg prednisolone or equivalent per day for 3 months or longer).
46
what are signs of osteoporotic vertebral fractures?
Loss of height: vertebral osteoporotic fractures of lead to compression of the spinal vertebrae hence a reduction in overall length of the spine and thus the patient becomes shorter
Kyphosis (curvature of the spine)
Localised tenderness on palpation of spinous processes at the fracture site
47
what are bisphosphonates used for?
prevention and treatment of osteoporosis
hypercalcaemia
Paget's disease
pain from bone metatases
48
what is SLE?
it is a chronic multi-system disorder that most commonly affects woman during their reproductive years. It is more common in asians and black africans.
It is an inflammatory autoimmune connective tissue disease.
It is “systemic” because it affects multiple organs and systems and “erythematosus” refers to the typical red malar rash that occurs across the face.
49
what is the basic pathophysiology in SLE?
SLE is characterised by anti-nuclear antibodies. These are antibodies to proteins within the persons own cell nucleus. This causes the immune system to target theses proteins. When the immune system is activated by these antibodies targeting proteins in the cell nucleus it generates an inflammatory response.
The immune system dysregulation leads to immune complex formation
Immune complex deposition can affect any organ including the skin, joints, kidneys and brain.
It is a type 3 hypersensitivity reaction
associated with HLA B8, DR2 and DR£
It often takes a relapsing-remitting course, with flares and periods where symptoms are improved. The result of chronic inflammation means patients with lupus often have shortened life expectancy. Cardiovascular disease and infection are leading causes of death.
50
what causes SLE?
Not really known but the interaction of an environmental agent in a genetically susceptible host is thought to be fundamental. The strong female preponderance also suggests a role for hormonal factors.
It is thought that drugs can be causative agents - procainamide, minocycline, terbinafine, sulfasalazine, isoniazid, phenytoin, carbamazepine.
51
how does SLE present?
General Features - fatigue, features, mouth ulcers, lymphadenopathy
skin - malar rash (most commonly erythema over the cheeks and bridge of nose), photosensitive rash (rash occurring after sun exposure - it can be painful and pruritic and usually lasts a few days), discoid rash (scaly, erythematous, well demarcated rash in sun-exposed areas), raynaud's phenomenon, livedo reticularis, non-scarring alopecia.
MSK - arthralgia, non erosive arthritis (usually symmetrical, smaller joints, pol
CV - pericarditis, myocarditis
Resp - pleurisy, fibrosing alveolitis
Renal - proteinuria, glomerulonephritis
Neuropsychiatric - anxiety and depression, psychosis, seizures
52
what investigations would you perform for SLE?
> Autoantibodies - anti-nuclear antibodies (ANA) (however other things can cause this to be positive e.g. autoimmune hepatitis), anti-double stranded DNA (anti-dsDNA) is also specific to SLE. Anti-smith, anti Ro and anti La may also be positive. 20% of patients will also be RF positive.
> FBC - normocytic anaemia of chronic disease
> ESR and CRP - raised with active inflammation
> complement levels - C3 and C4 are low during active disease.
> Immunoglobulins - may be raised due to activation of B cells with inflammation
> Urinalysis - if there is renal involvement there may be haematuria, casts (red cell, granular, tubular or mixed) or proteinuria
> renal biopsy to look for lupus nephritis
> U&E's - elevated urea and creatinine if there is renal manifestations
> CXR - if there are cardiopulmonary manifestations there may be evidence of pleural effusion, infiltrates, cardiomegaly.
> ECG - may exclude other causes of chest pain
** you can use the SLICC criteria or the ACR criteria for establishing diagnosis
53
what are some differentials for SLE ?
> RA- may be difficult to differentiate clinically, SLE usually less symmetrical joint involvement that RA
>anti-phospholipid syndrome
> systemic sclerosis - often both have Raynaud's phenomenon however in SLE they tend not to ulcerate compared with patients with systemic sclerosis
> mixed connective tissue disease - difficult to differentiate clinically
> adult still's disease
> lyme disease
54
what are the complications of SLE?
complications re related to chronic inflammations
- CV disease - is the leading cause of death - chronic inflammation in the blood vessels leads to hypertension and CAD
- infection is more common due to the disease process and also secondary to immunosuppressants
- anaemia of chronic disease
- patients get leucopenia, neutropenia, and thrombocytopenia
- pericarditis
- pleuritis
- interstitial lung disease - caused by inflammation in the lungs which leads to pulmonary fibrosis
- lupus nephritis due to inflammation in the kidney
- neuropsychiatric SLe
- recurrent miscarriage
- VTE
55
how is SLE managed?
immunosuppression is the mainstay of treatment
first line treatments are:
- NSAIDs
- steroids - prednisolone
- hydroxychloroquine - first line for mild SLE
- suncream and sun avoidance for the photosensitive rash
```
Methotrexate can be added (f so folic acid should also be added)
other immunosuppressants that can be used in more severe or resistant SLE:
Methotrexate
Mycophenolate mofetil
Azathioprine
Tacrolimus
Leflunomide
Ciclosporin
```
if there is severe disease biological therapies can be added:
Rituximab is a monoclonal antibody that targets the CD20 protein on the surface of B cells
Belimumab is a monoclonal antibody that targets B-cell activating factor
56
what is antiphospholipid syndrome?
it is an acquired disorder characterised by a predisposition to both venous and arterial thromboses, recurrent fetal loss and thrombocytopenia.
It may occur as a primary disorder or secondary to other condition, most commonly SLE.
**A key point for the exam is to appreciate that antiphospholipid syndrome causes a paradoxical rise in the APTT. This is due to an ex-vivo reaction of the lupus anticoagulant autoantibodies with phospholipids involved in the coagulation cascade
57
what antibodies are associated with antiphospholipid syndrome?
Lupus anticoagulant
Anticardiolipin antibodies
Anti-beta-2 glycoprotein I antibodies
These antibodies interfere with coagulation and create a hyper coagulable state where the blood is more prone to clotting.
58
how does antiphospholipid syndrome present?
recurrent venous/arterial thrombosis
- venous - DVT, PE
- arterial - stoke, MI, renal thrombosis
Pregnancy complications
- recurrent miscarriage
- still birth
- pre- eclampsia
livedo reticularis - a purple lace like rash that gives a mottled appearance to the skin
Libmann-Sacks endocarditis - a type of non bacterial endocarditis where there are growth (vegetations) on the valves of the heart. it is also associated with SLE
59
what investigations would you perform for antiphospholipid syndrome?
Diagnosis is made when there is a history of thrombosis or pregnancy complication plus persistent antibodies: lupus anticoagulant, Anticardiolipin, anti-beta2-glycoprotein I antibodies - needs to be positive on two occasions, 12 weeks apart
FBC - may show thrombocytopenia
Creatine and urea may be raised if nephropathy is present
> ANA, double stranded DNA - if positive may suggest underlying SLE
60
how is antiphospholipid syndrome managed?
Patients are usually managed jointly between rheumatology, haematology and obstetrics (if pregnant)
primary prophylaxis - low dose aspirin
secondary - long term warfarin with a target INR of 2-3
61
what is Sjogren's syndrome
It is a systemic auto-immune disorder that affects the exocrine glands resulting in dry mucosal surface characterised (dry eyes, dry mouth as a consequence of lymphocytic infiltration into the lacrimal and salivary glands. There may also be dry skin, nose, throat, vagina)
Primary - when is occurs in isolation
Secondary - when it occurs secondary to SLE or RA
62
what are the features of Sjogren's syndrome?
```
dry eyes - keratoconjunctivitis sicca
dry mouth
vaginal dryness
arthralgia
raynaud's
sensory polyneuropathy
recurrent episodes of parotitis
renal tubular acidosis - usually subclinical
```
63
what investigations would you perform for Sjogren's syndrome?
Schirmer's test - involves inserting a folded piece of filter paper under the lower eyelid with a strip hanging out over the eyelid. This is left in for 5 minutes and the distance along the strip hanging out that becomes moist is measured. The tears should travel 15mm in a healthy young adult. A result of less than 10mm is significant.
Anti Ro (SSA) and and Anti La (SSB)
ANA - positive in 70%
RF positive in nearly 100% of patients
64
how s Sjogren's managed?
- artificial tears
- artificial saliva
- vaginal lubricants
- hydroxychloroquine is used to halt the progression of the disease
pilocarpine may stimulate saliva production
65
what are some complications of sjogren's?
Eye infections such as conjunctivitis and corneal ulcers
Oral problems such as dental cavities and candida infections
Vaginal problems such as candidiasis and sexual dysfunction
```
Sjogren's can rarely affect other organs causing complications such as:
Pneumonia and bronciectasis
Non-Hodgkins lymphoma
Peripheral neuropathy
Vasculitis
Renal impairment
```
66
what is systemic sclerosis?
```
Systemic sclerosis (SSc), also known as scleroderma, is a multi-system, autoimmune disease, characterised by functional and structural abnormalities of small blood vessels, fibrosis of skin and internal organs, and production of auto-antibodies.
It is 4x more common in women
```
67
what are the two main types of systemic sclerosis ?
```
Limited Cutaneous Systemic Sclerosis
Limited cutaneous systemic sclerosis is the more limited version of systemic sclerosis. It used to be called CREST syndrome. This forms a helpful mnemonic for remembering the features of limited cutaneous systemic sclerosis:
C – Calcinosis
R – Raynaud’s phenomenon
E – oEsophageal dysmotility
S – Sclerodactyly
T – Telangiectasia
```
Diffuse Cutaneous Systemic Sclerosis
Diffuse cutaneous systemic sclerosis includes the features of CREST syndrome plus many internal organs causing:
Cardiovascular problems, particularly hypertension and coronary artery disease.
Lung problems, particularly pulmonary hypertension and pulmonary fibrosis.
Kidney problems, particularly glomerulonephritis and a condition called scleroderma renal crisis.
68
what are the features of systemic sclerosis?
Scleroderma refers to hardening of the skin - most notable on the hands and face.
Sclerodactyly - skin changes in the hands. As the skin tightens around joints it restricts the range of motion in the joint and reduces the function of the joints. As the skin hardens and tightens further the fat pads on the fingers are lost. The skin can break and ulcerate.
Telangiectasia are dilated small blood vessels in the skin.
Calcinosis is where calcium deposits build up under the skin. This is most commonly found on the fingertips.
Raynaud’s phenomenon
Oesophageal dysmotility is caused by connective tissue dysfunction in the oesophagus. - reflux and dysphagia
Systemic and pulmonary hypertension is caused by connective tissue dysfunction in the systemic and pulmonary arterial systems. Systemic hypertension can be worsened by renal impairment.
Pulmonary fibrosis can occur in severe systemic sclerosis. This presents with gradual onset dry cough and shortness of breath.
Scleroderma renal crisis is an acute condition where there is a combination of severe hypertension and renal failure.
69
what autoantibodies are associated with systemic sclerosis?
Antinuclear antibodies (ANA) are positive in most patients with systemic sclerosis. They are not specific to systemic sclerosis.
Anti-centromere antibodies are most associated with limited cutaneous systemic sclerosis.
Anti-Scl-70 antibodies are most associated with diffuse cutaneous systemic sclerosis. They are associated with more severe disease.
70
what investigations would you perform for systemic sclerosis?
```
systemic auto-antibodies
nailfold capillaroscopy
barium swallow
CXR
echo and ECG
```
71
how is systemic sclerosis managed?
if there is diffuse disease then steroids and immunosuppressants are started (methotrexate)
Non-medical management involves:
Avoid smoking
Gentle skin stretching to maintain the range of motion
Regular emollients
Avoiding cold triggers for Raynaud’s
Physiotherapy to maintain healthy joints
Occupational therapy for adaptations to daily living to cope with limitations
Medical management focuses on treating symptoms and complications:
Nifedipine can be used to treat symptoms of Raynaud’s phenomenon
Anti acid medications (e.g. PPIs) and pro-motility medications (e.g. metoclopramide) for gastrointestinal symptoms
Analgesia for joint pain
Antibiotics for skin infections
Antihypertensives can be used to treat hypertension (usually ACE inhibitors)
Treatment of pulmonary artery hypertension
Supportive management of pulmonary fibrosis
72
what is polymyositis?
Polymyositis is an autoimmune disorders where there is inflammation in the muscles (myositis).
Polymyositis is a condition of chronic inflammation of muscles - causing symmetrical, proximal muscle weakness
It is thought to be a T-cell mediate cytotoxic process directed against muscle fibres
it may be idiopathic or associated with connective tissue disorders
73
what is dermatomyostis?
dermatomyositis is an autoimmune disorders where there is inflammation in the muscles (myositis). It is a connective tissue disorder where there is chronic inflammation of the skin and muscles.
it will cause symmetrical, proximal muscle weakness and characteristic skin lesions
It may be idiopathic or associated with connective tissue disorders or underlying malignancies (typically ovarian, breast, and lung cancer)
74
what cancers are associated with polymyositis and dermatomyositis?
```
Polymyositis or dermatomyositis can be caused by an underlying malignancy. This makes them paraneoplastic syndromes. The most common associated cancers are:
Lung
Breast
Ovarian
Gastric
```
75
what are the features of polymyositis?
proximal muscle weakness +/- tenderness - often bilateral and mostly affects shoulder and pelvic girdle - develops over weeks
Raynaud's
respiratory muscle weakness
interstitial lung disease e.g. fibrosing alveolitis or organising pneumonia
dysphagia
dysphonia
76
what are the features of dermatomyositis?
Skin features
photosensitive
macular rash over back and shoulder
heliotrope rash in the periorbital region
Gottron's papules - roughened red papules over extensor surfaces of fingers
'mechanic's hands': extremely dry and scaly hands with linear 'cracks' on the palmar and lateral aspects of the fingers
nail fold capillary dilatation
Other features
proximal muscle weakness +/- tenderness - often bilateral and mostly affects shoulder and pelvic girdle - develops over weeks
Raynaud's
respiratory muscle weakness
interstitial lung disease: e.g. Fibrosing alveolitis or organising pneumonia
dysphagia, dysphonia
77
what investigations would you perform for polymyositis?
- creatine kinase - elevated
other muscle enzymes - lactate dehydrogenase, aldolase, AST and ALT may also be elevated in 85-95% of patients
EMG
muscle biopsy
anti-synthetase antibodies
anti-Jo-1 antibodies are seen in pattern of disease associated with lung involvement, Raynaud's and fever
78
what is the investigation for dermatomyositis?
ANA positive (80%)
anti-synthetase antibodies positive in 30% including:
> antibodies against histidine-tRNA ligase (also called Jo-1)
> antibodies to signal recognition particle (SRP)
> anti-Mi-2 antibodies
EMG
CK
muscle biopsy
serum aldolase - high levels
skin biopsy
79
what autoantibodies are involved in dermatomyositis and polymyositis?
Anti-Jo-1 antibodies: polymyositis (but often present in dermatomyositis)
Anti-Mi-2 antibodies: dermatomyositis.
Anti-nuclear antibodies: dermatomyositis.
80
what is the key investigation for myositis?
The key investigation for diagnosing myositis is a creatine kinase blood test. Creatine kinase is an enzyme found inside muscle cells. Inflammation in the muscle cells (myositis) leads to the release of creatine kinase. Creatine kinase is usually less than 300 U/L. In polymyositis and dermatomyositis the result is usually over 1000, often in the multiples of thousands.
81
other than myositis what can cause raised CK?
Other causes of a raised creatine kinase include:
```
Rhabdomyolysis
Acute kidney injury
Myocardial infarction
Statins
Strenuous exercise
```
82
how is dermatomyositis and polymyositis managed?
* new cases should be assessed for possible underlying cancer
- physio and occy health to help with fuscle strength and function
- corticosteroid are first line
- 2nd line- immunosuppressants (azathioprine), IV immunoglobulins, biological therapies (infliximab or etanercept)
83
what is Raynaud's?
Raynaud's phenomena may be primary (Raynaud's disease) or secondary (Raynaud's phenomenon)
Raynaud's phenomenon is characterised by vasospasm that causes digits to change colour to white (pallor) from lack of blood flow, usually brought on by cold temperatures. Affected areas subsequently turn blue due to de-oxygenation and/or red due to reperfusion. It can be a painful condition and can lead to complications.
84
what are the features of Raynaud's?
- digit pain/discomfort
- digital paraesthesia - when fingers are re-warmng
- pallor of digits
- red and/or blue discolouration of digits
- dilated capillaries at nailbeds - occurs in secondary RP - if present the secondary should be assumed until proven otherwise
85
what factors suggest underlying connective tissue disease causing Raynaud's?
```
onset after 40 years
unilateral symptoms
rashes
presence of autoantibodies
features which may suggest rheumatoid arthritis or SLE, for example arthritis or recurrent miscarriages
digital ulcers, calcinosis
very rarely: chilblains
```
86
what are secondary causes of Raynaud's?
connective tissue disorders: scleroderma (most common), rheumatoid arthritis, SLE
leukaemia
type I cryoglobulinaemia, cold agglutinins
use of vibrating tools
drugs: oral contraceptive pill, ergot
cervical rib
87
how is Raynaud's diagnosed?
usually a clinical diagnosis
| investigations for secondary causes (autoantibodies, FBC, ESR, urinalysis)
88
how s Raynaud's managed?
calcium channel blocker - nifedipine
analgesia
If severe IV prostacyclin (epoprostenol) infusions - the effects may last several weeks/months
89
what are the complications of Raynaud's?
necrosis with gangrene
ischaemic digital ulcers
traumatic digital ulcers
cellulitis/osteomyelitis
90
what are seronegative spondyloarthropathies?
Ankylosing spondylitis
psoriatic arthritis
reactive arthritis
* seronegative for RF
91
what is psoriatic arthritis?
Psoriatic arthritis is an inflammatory arthritis associated with psoriasis. This can vary in severity. Patients may have a mild stiffening and soreness in the joint or the joint can be completely destroyed in a condition called arthritis mutilans.
Psoriatic arthropathy correlates poorly with cutaneous psoriasis and often precedes the development of skin lesions
92
what are the different patterns of psoriatic arthritis?
distal interphalangeal joint (DIP) arthritis
asymmetric oligoarthritis - affects mainly digits and feet.
symmetric polyarthritis - similar to RA, more common in woman
arthritis mutilans
psoriatic spondylitis with sacroiliac and spinal involvement - back stiffness, sacroilitis,
93
what are the diagnostic features of psoriatic arthritis?
- personal or family history of psoriasis
- joint pain and stiffness (inflammatory joint pain is characterised by prolonged morning stiffness, improvement with use, and recurrence with prolonged rest
- peripheral arthritis - usually indicated by swelling and tenderness of individual joints
- Dactylitis - uniform swelling of an entire finger
- plaques of psoriasis on the skin
- pitting of the nails
- onycholysis - separation of the nail from the nail bed
- Enthesitis (inflammation of the entheses, which are the points of insertion of tendons into bone)
other associations include:
Eye disease (conjunctivitis and anterior uveitis)
Aortitis (inflammation of the aorta)
Amyloidosis
94
What is PEST?
Psoriasis Epidemiological Screening Tool
NICE recommend patients with psoriasis complete the PEST tool to screen for psoriatic arthritis. This involves several questions asking about joint pain, swelling, a history of arthritis and nail pitting. A high score triggers a referral to a rheumatologist.
95
what investigations would you perform for psoriatic arthritis?
```
X-rays of hands and feet
ESR and CRP - normal or elevated
RF
Anticyclic citrullinated peptide antibody - highly specific for RA so will be negative
synovial biopsy
```
* there is an increased risk of metabolic syndrome in psoriatic disease so patients should have appropriate metabolic screening - lipid profile, fasting blood glucose, uric acid level
96
what would you see on an X-ray in someone with psoriatic arthritis?
Periostitis is inflammation of the periosteum causing a thickened and irregular outline of the bone
Ankylosis is where bones joining together causing joint stiffening
Osteolysis is destruction of bone
Dactylitis is inflammation of the whole digit and appears on the xray as soft tissue swelling
Pencil-in-cup appearance
The classic xray change to the digits is the “pencil-in-cup appearance”. This is where there are central erosions of the bone beside the joints and this causes the appearance of one bone in the joint being hollow and looking like a cup whilst the other is narrow and sits in the cup.
97
how is psoriatic arthritis managed?
Management is similar to rheumatoid arthritis. There is a crossover between the systemic treatments of psoriasis and treatment of psoriatic arthritis. Treatment is often coordinated between dermatologists and rheumatologists.
Depending on the severity the patient might require:
NSAIDs for pain
DMARDS (methotrexate, leflunomide or sulfasalazine)
Anti-TNF medications (etanercept, infliximab or adalimumab)
Ustekinumab is last line (after anti-TNF medications) and is a monoclonal antibody that targets interleukin 12 and 23
98
what is ankylosing spondylitis?
```
Ankylosing spondylitis (AS) is a chronic progressive inflammatory arthropathy
it mainly affecting the spine that causes progressive stiffness and pain. It is part of the seronegative spondyloarthropathy group of conditions relating to the HLA B27 gene. Other conditions in this group are reactive arthritis and psoriatic arthritis.
```
The key joints that are affected in AS are the sacroiliac joints and the joints of the vertebral column. The inflammation causes pain and stiffness in these joints. It can progress to fusion of the spine and sacroiliac joints. Fusion of the spine leads to the classical “bamboo spine” finding on spinal xray that often appears in medical exams.
99
how does ankylosing spondylitis present?
> inflammatory back pain (stiffness that occurs early morning, improvement of stiffness with exercise)
> lower back pain and stiffness and sacroiliac pain in the buttock region
> usually a young adult male in their late teens or 20s
> symptoms develop over around 3 months
> symptoms can fluctuate with flares of worsening symptoms
> vertebral fractures are a key complication of AS
* it does not just affect the spine:
- systemic symptoms such as weight loss and fatigue
- chest pain related to costovertebral and costosternal joints
- Enthesitis - can causes plantar fascitits and achilles tendonitis
- dactylitis - inflammation of fingers and toes
- anaemia
- anterior uveitis
- aoritis - inflammation of the aorta
- heart block - caused by fibrosis of the heart's conducting system
- restrictive lung disease - caused by restrictive chest wall movement
- pulmonary fibrosis at the upper lobes occurs in 1% of patients
- inflammatory bowel disease is associated with AS
100
how would you diagnose ankylosing spondylitis?
examination - reduced lateral flexion, reduced forward flexion (Schober's test), reduced chest expansion
Pelvic X-ray - will show sacroiliitis
HLAB27 - present in 90-95% of patients with AS
MRI - bone marrow oedema early in the disease before X-ray changes
ESR and CRP are typically raised
101
what is Schober's test?
This is a test used as part of a general examination of the spine to assess how much mobility there is in the spine.
Have the patient stand straight. Find the L5 vertebrae. Mark a point 10cm above and 5cm below this point (15cm apart from each other). Then ask the patient to bend forward as far as they can and measure the distance between the points.
If the distance with them bending forwards is less than 20cm, this indicates a restriction in lumbar movement and will help support a diagnosis of ankylosing spondylitis.
102
what are differentials for ankylosing spondylitis?
```
osteoarthritis
diffuse idiopathic skeletal hyperostosis
psoriatic arthritis
reactive arthritis
infection e.g. discitis
vertebral fracture
bony mets
myeloma
mechanical back pain
```
103
what X-ray changes would you see in ankylosing spondylitis?
“Bamboo spine” is the typical exam description of the xray appearance of the spine in later stage ankylosing spondylitis. This is worth remembering for your exams.
Xray images in ankylosing spondylitis can show:
- Squaring of the vertebral bodies
- Subchondral sclerosis and erosions
- Syndesmophytes are areas of bone growth where the ligaments insert into the bone. They occur related to the ligaments supporting the intervertebral joints.
- Ossification of the ligaments, discs and joints. This is where these structures turn to bone.
- Fusion of the facet, sacroiliac and costovertebral joints
104
how is ankylosing spodnylitis managed?
Medication:
>NSAIDs
> Steroids can be use during flares to control symptoms. (This could oral, intramuscular slow release injections or joint injections.)
> Anti-TNF medications such as etanercept (in severe disease)
> monoclonal antibody against TNF such as infliximab, adalimumab or certolizumab pegol (in severe disease)
> Secukinumab is a monoclonal antibody against interleukin-17. It is recommended by NICE if the response to NSAIDS and TNF inhibitors is inadequate.
```
Additional management:
Physiotherapy
Exercise and mobilisation
Avoid smoking
Bisphosphonates to treat osteoporosis
Treatment of complications
Surgery is occasionally required for deformities to the spine or other joints
```
105
how is ankylosing spondylitis managed?
Medication:
>NSAIDs
> Steroids can be use during flares to control symptoms. (This could oral, intramuscular slow release injections or joint injections.)
> Anti-TNF medications such as etanercept (in severe disease)
> monoclonal antibody against TNF such as infliximab, adalimumab or certolizumab pegol (in severe disease)
> Secukinumab is a monoclonal antibody against interleukin-17. It is recommended by NICE if the response to NSAIDS and TNF inhibitors is inadequate.
```
Additional management:
Physiotherapy
Exercise and mobilisation
Avoid smoking
Bisphosphonates to treat osteoporosis
Treatment of complications
Surgery is occasionally required for deformities to the spine or other joints
```
106
what is reactive arthritis?
Reactive arthritis is where synovitis occurs in the joints as a reaction to a recent infective trigger (GI or GU infections)
It used to be known as Reiter Syndrome.
Typically it causes an acute monoarthritis, affecting a single joint in the lower limb (most often the knee) presenting with a warm, swollen and painful joint.
There is a link with the HLA B27 gene
107
how does reactive arthritis present and what is it associated with?
- acute, asymmetrical polyarthritis, tends to be larger joints in the lower extremity (ankle and knee)
- dactylitis
- axial arthritis - spinal inflammation is a common finding
- symptoms usually begin within 1 to 4 weeks after the onset of infection, symptoms can vary from a mild arthritis to a serious multi system infection.
associations:
> Bilateral conjunctivitis (non-infective)
> Anterior uveitis
> Circinate balanitis is dermatitis of the head of the penis
** These features of reactive arthritis (eye problems, balanitis and arthritis) lead to the saying “can’t see, pee or climb a tree”.
there may sometimes be mouth ulcers, fever, fatigue, eight loss, CNS and CV involvement.
108
what are the most common causes of reactive arthritis?
GI infections - Shigella, salmonella, campylobacter, yersinia
GU infectons - chlamydia
109
how is reactive arthritis diagnosed?
ESR and CRP - raised
Urogenital and stool cultures
X-ray - typically shows sacroiliitis or enthesopathy
HLAB27
Synovial aspirate to exclude septic arthritis, gout and pseudogout.
rule out other causes of arthritis - RF, ANA,
110
how is reactive arthritis managed?
NSAIDs
Steroid injections of affected joints
systemic steroids if there are multiple joints affected
recurrent cases may require DMARDs or anti-TNF medications
111
what are the differential of reactive arthritis?
```
Ankylosing spondylitis
psoriatic arthritis
RA
adult onset stills disease
disseminated gonococcal disease
arthritis associated with IBD
gout
septic arthritis
post-viral arthritis
```
112
what types of vasculitis affect the small vessles?
- Henoch-Schonlein purpura
- Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
- Microscopic polyangiitis
- Granulomatosis with polyangiitis (Wegener’s granulomatosis)
113
what types of vasculitis affect the medium sized vessels?
- polyarteritits nodosa
- Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss syndrome)
- Kawasaki Disease
114
what types of vasculitis affect the large vessels?
Giant cell arteritis
| Takayasu’s arteritis
115
what clinical features are associated with most types of vasculitis?
- Purpura - purple coloured non-blanching spots caused by blood leaking from the vessels under the skin
- joint and muscle pain
- peripheral neuropathy
- renal impairment
- GI disturbance (diarrhoea, abdominal pain and bleeding)
- anterior uveitis and scleritis
systemic manifestations
- fatigue
- weight loss
- fever
- anorexia (loss of appetite)
- anaemia
116
what general tests would you do for vasculitis?
- ESR and CRP - usually raised
- ANCA - p- ANCA and c-ANCA
* P-ANCA aka anti-MPO - microscopic polyangitis and churg-stauss syndrome
* C-ANCA - anti-PR3 - wegener's granulomatosis
117
In general how is vasculitis managed?
treatment usually involves a combination of steroids and immunosupressants
Steroids can be administered to target the affected area:
Oral (i.e. prednisolone)
Intravenous (i.e. hydrocortisone)
Nasal sprays for nasal symptoms
Inhaled for lung involves (e.g. Churg-Strauss syndrome)
Immunosuppressants that are used include:
Cyclophosphamide
Methotrexate
Azathioprine
Rituximab and other monoclonal antibodies
The management of HSP and Kawasaki disease (the types mainly affecting children) is different.
118
what is HSP?
Henoch-Schonlein Purpura (HSP) is an IgA vasculitis that commonly presents with a purpuric rash affecting the lower limbs or buttocks in children
HSP usually triggered by an upper airway infection or a gastroenteritis
most common in children under age of 10
4 classic features:
- purpura
- joint pain
- abdominal pain
- renal involvement
** management is supportive - simple analgesia an proper hydration
119
what is Churg-stauss syndrome?
Eosinophilic granulomatosis with polyangiitis
It is a small and medium vessle vasculitis
most associated with lung and skin but can affect other organs such as kidney
120
what is wegener's?
Granulomatosis with polyangiitis
Wegener’s granulomatosis is a small vessel vasculitis. It affects the respiratory tract and kidneys.
In the upper respiratory tract it commonly affects the nose causing nose bleeds (epistaxis) and crusty nasal secretions, ears causing hearing loss and sinuses causing sinusitis. A classic sign in exams is the saddle shaped nose due to a perforated nasal septum. This causes a dip halfway down the nose.
In the lungs it causes a cough, wheeze and haemoptysis. A chest xray may show consolidation and it may be misdiagnosed as pneumonia.
In the kidneys it can cause a rapidly progressing glomerulonephritis.
121
what is Takayasu's arteritis?
Takayasu’s arteritis is a form of large vessel vasculitis. It mainly affects the aorta and it’s branches. It also affect the pulmonary arteries. These large vessels and their branches can swell and form aneurysms or become narrowed and blocked. This leads to it’s other name of “pulseless disease”.
It usually presents before the age of 40 years with non-specific systemic symptoms, such as fever, malaise and muscle aches, or with more specific symptoms of arm claudication or syncope. It is diagnosed using CT or MRI angiography. Doppler ultrasound of the carotids can be useful in detecting carotid disease.
122
what is polymyalgia rheumatica?
- an inflammatory rheumatological syndrome
- characterised by muscle stiffness involving the neck, shoulder girdle, and/or pelvic girdle in individuals older than 50
- it is either and isolated condition or associated with GCA (temporal arteritis)
123
how does polymyalgia rheumatica present?
- shoulder/hip girdle stiffness and pain
- they will have rapid response to corticosteroids
- acute onset
- lower grade fever
- loss of appetite
- weight loss
- malaise
- mild polyarthralgia
- lethargy
* they may find it particularly difficult to stand from seated position, get up out of bed, raise arms to brush hair
* examination findings are often normal
* the morning stiffness if caused by synovitis
124
how is polymyalgia rheumatica investigated?
ESR and CRP - raised
| EMG and CK will be normal
125
how is polymyalgia rheumatica managed?
- prednisolone
- alendronic acid, colecalciferol and calcium carbonate
2nd line - methotrexate
126
what criteria is used for polymyalgia rheumatica?
Criteria: must have any 3 factors, or just 1 and a temporal artery biopsy positive for giant cell arteritis
```
Age over 65 years
Bilateral shoulder girdle pain
More than 1 hour morning stiffness
Symptom onset <2 weeks
ESR >40 mm/hour
Depression/weight loss
Upper arm tenderness, bilateral.
```
127
what is polyarteritis nodosa?
- it is a rare form of a systemic vasculitis which only affects medium-sized vessels
- there is necrotising inflammation of the vessels which leads to aneurysm formation
- it is more common in middle age men and is associated with hepatitis B infection.
128
what are the features of polyarteritis nodosa?
```
fever
weight loss
myalgi or arthralgia
mononeuritis multiple
paraesthesia
muscle tenderness
abdominal pain - caused by ischaemic bowel
skin manifestations - Livedo reticularis (purple lace like rash) , skin ulcers, bullous or vesicular eruptions, purpura, or skin infarction may occur in PAN
high diastolic blood pressure
```
129
what investigations would you perform for polyarteritis nodosa?
CRP and ESR will be raised
FBC - normocytic anaemia, mildly elevated WBC, raised platelet
liver function tests - elevated liver enzymes
HBV serology
blood cultures to exclude endovascular infection
ANCA, ANA, anti-dsDNA, RF, anti CCP - negaive
130
how would you manage polyarteritis nodosa?
non-HBV - oral prednisolone and DMARD (azathioprine or methotrexate)
if HBV related - oral pred +/- IV methylpred pus plasma exchange and lamivudine
131
what are differentials for polyarteritis nodosa?
Granulomatosis with polyangiitis (wegner's), Churg-stauss syndrome -all ANCA associated and only affects small vessels
Infection
RA
SLE
132
what is gout?
gout is a type of crystal arthropathy associated with chronically high blood uric acid levels.
