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MSK 2022 > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

What is osteoarthritis?

A

The most common form of arthritis and is primarily a degenerative disorder.

It is characterised by localised loss of hyaline cartilage and accompanying periarticular change such as remodelling of adjacent bone with new bone formation at joint margins.

Over time the normal structure of every joint is subject to wear and deterioration. Alongside Oa there may also be a considerable amount of associated inflammation, which can lead to periodic flaring of the OA.

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2
Q

What is localised OA?

A

can affect hips, knees, finger interphalangeal joints, facet joints of lower cervical and lower lumbar spines

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3
Q

What is generalised OA?

A

defined as OA at either the spinal or hand joints and in at least 2 other joint regions.

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4
Q

What are the risk factors of OA?

A
  • genetic - mendelian inheritance (40-60%)
  • females
  • obesity
  • occupational usage
  • joint laxity
  • developmental or pathological abnormal alignment of joints.
  • previous injuries such as articular cartilage injuries, intra-articular fractures, extra-articular fractures with subsequent malalignment and meniscal injuries.
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5
Q

What is the cause of primary OA?

A

Primary OA has no identifiable cause

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6
Q

What is the cause of secondary OA?

A

Secondary OA can be caused by:

  • Congenital dislocation of the hip
  • Perthes
  • SUFE
  • Previous intra-articular fracture
  • Extra-articular fracture with malunion
  • Osteochondral/hyaline cartilage injury
  • Crystal arthropathy
  • Inflammatory arthritis
  • Meniscal tears
  • Genu Varum or Valgum
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7
Q

What is the clinical presentation of OA?

A
  • extremely variable
  • pain - worse with joint use
  • morning stiffness lasting less than an hour
  • instability
  • poor grip in thumb OA
  • joint line tenderness
  • crepitus
  • joint effusion
  • bony swelling - Heberden’s nodes and Bouchard’s nodes
  • deformity
  • limitation of motion
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8
Q

What condition is this seen in?

A

Osteoarthritis

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9
Q

How is OA diagnosed?

A

An x-ray of osteoarthritis shows:

L - Loss of joint space

O - osteophytes

S - sclerosis

S- subchondral cysts

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10
Q

What does this MRI scan indicate?

A

Osteoarthritis of the lumbar spine

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11
Q

What does these x-ray features indicate?

A

Osteoarthritis of the knee?

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12
Q

What condition does this x-ray indicate?

A

Osteoarthritis

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13
Q

What is the conservative management of osteoarthritis?

A

Consists largely of pain control - simple analgesia and mild opiates may be helpful.

Physiotherapy is useful in strengthening surrounding structures.

Weight loss and exercise are also important.

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14
Q

What are the surgical options for osteoarthritis?

A

In some situations, surgery may be an option e.g., hip and knee replacement, but this depends on the joint affected.
Today the gold standard for THR is the cemented metal stem and head/polyethylene cup.

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15
Q

What are the early local complications of joint replacement surgery in OA?

A

infection
dislocation
nerve injury
leg length discrepancy

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16
Q

What are the early general complications of joint replacement surgery in OA?

A

MI
Chest infection
UTI
Blood loss
hypovolaemia
DVT
PE

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17
Q

What are the late local complications of joint replacement surgery in OA?

A

Early loosening
Late infection
Late dislocation

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18
Q

Describe rheumatoid arthritis

A

Most prevalent seropositive inflammatory arthropathy.

It is an auto-immune inflammatory symmetric polyarthropathy which most commonly affects the small joints of the hands and feet. Larger joints such as the knees, shoulders and elbows can also be affected as the disease progresses.

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19
Q

What is the pathogenesis of rheumatoid arthritis?

A

In the disease process, an immune response is initiated against synovium which lines synovial joints and some tendons. Inflammatory pannus forms which then attacks and denudes articular cartilage leading to joint destruction. Tendon ruptures and soft tissue damage can occur leading to joint instability and subluxation.

Main structure involved is the synovium which lines the inside of the synovial joint capsules and tendon sheath. The C1/C2 joint, hand joints, wrists, elbows, shoulders, TMJs, knees, hips, ankles and feet are affected.

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20
Q

What are the risk factors of rheumatoid arthritis?

A

Women are more likely to be affected 3:1

Prevalence increases with age peaking around 35-50.

Genetic factors account for 50% of the risk for developing RA.

First degree relatives of individuals with RA are at 2- to 3-fold higher risk for the disease.

Triggers such as smoking, infection or trauma have been implicated.

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21
Q

What are the clinical features of rheumatoid arthritis?

A
  • symmetrical synovitis (doughy swelling)
  • pain
  • morning stiffness
  • hands and feet tend to be involved early - MCP and PIPs joints are affected as well as wrists, but DIP joints are not.
  • late features in aggressive or untreated disease include deformities
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22
Q

What are the systemic features of advanced rheumatoid arthritis?

A
  • over time larger joints can become affected. Including the cervical spine such as atlanto-axial subluxation which can result in cervical cord compression.
  • rheumatoid nodules occur in approximately 25% of patients with RA. These lesions are most commonly found on extensor surfaces or sites of frequent mechanical irritation.
  • lung involvement includes pleural effusions, interstitial fibrosis and pulmonary nodules.
  • cardiovascular morbidity and mortality are increased in patients with RA
  • ocular involvement is common in individuals with RA and includes keratoconjunctivitis sicca, episcleritis, uveitis, and nodular scleritis that may lead to scleromalacia.
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23
Q

When is this seen in a patient?

A

Rheumatoid arthritis

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24
Q

When is this seen in a patient?

A

Rheumatoid arthritis

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25
Q

What is seen in this patient?

A

Deformities due to advanced rheumatoid arthritis

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26
Q

What is seen in this X-ray?

A

Atlanto-axial subluxation in rheumatoid arthritis

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27
Q

What is seen in this image?

A

Rheumatoid nodules seen in rheumatoid arthritis

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28
Q

What are the investigations for rheumatoid arthritis?

A

Identified auto-antibodies which can be measured, and aid diagnosis include Rheumatoid Factor and Anti-CCP antibody.

Anti-CCP is far more specific and is therefore the preferred test. Around 15-20% of patients with RA are seronegative. These patients remain positive despite treatment. The absence of Anti CCP antibody does not exclude disease.

CRP, ESR and plasma viscosity are usually raised. X-rays at the onset of disease will often show no joint abnormality.

Early features can include peri-articular osteopenia (bone thinning) and soft tissue swelling.

Periarticular erosions can occur later in the disease.

Ultrasound may be useful in detecting synovial inflammation if there is clinical uncertainty.

Metacarpal and Metatarsal squeeze test may induce symptoms.

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29
Q

What is seen in this x-ray?

A

Rheumatoid arthritis of the feet (metatarsals)

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30
Q

What does this image show?

A

An MRI of rheumatoid arthritis in the hand

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31
Q

How is rheumatoid arthritis assessed?

A

Calculate DAS28 score

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32
Q

What does the DAS28 score indicate?

A

DAS28 < 2.6 Remission

DAS28 2.7-3.2 Low disease activity

DAS28 3.3-5.1 Moderate disease activity

DAS28 >5.1 High disease activity

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33
Q

What is the treatment of rheumatoid arthritis?

A

Aimed at relieving symptoms and preventing disease progression. The goal is to commence DMARD therapy within 3 months of symptom onset.

Short term treatments include simple analgesia, NSAIDs and intramuscular/intra-articular and oral steroids.

DMARD therapy can include various drugs, but methotrexate is usually used first line. Other DMARDs include sulphasalazine, hydroxychloroquine and leflunomide. Most DMARDs are immunosuppressive so may increase the risk of infection and can cause bone marrow suppression and liver function derangement. Regular blood monitoring is therefore required.

If the disease does not respond to standard DMARD therapy, then the patient may be eligible for biologic therapy. These are newer agents which can be more effective in treating active disease. The most common biologics are anti-TNF alpha drugs, all of which are given by injection. There are other available biologics including tocilizumab (IL-6 Blocker), rituximab (B cell depletor) and abatacept (T cell receptor blocker). Again, there is an increased risk of infection, especially tuberculosis.

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34
Q

What operative management is available for rheumatoid arthritis?

A
  • Surgery can be used for resistant disease, to control pain from a particular joint or to improve or maintain function but increasingly this is less often the case. Operations include:
    • Synovectomy
    • Joint replacement
    • Joint excision
    • Tendon transfers
    • Arthrodesis (fusion)
    • Cervical spine stabilisation
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35
Q

What is ankylosing spondylitis?

A

A chronic inflammatory disease of the spine and sacro-iliac joints which can lead to eventual fusion of the intervertebral joints and SI joints.

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36
Q

What are the typically patients with ankylosing spondylitis?

A
  • males are more affected 3:1
  • age of onset typically 20-40 yrs
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37
Q

What are the clinical features of ankylosing spondylitis?

A
  • spinal pain and stiffness
  • may develop knee or hip arthritis
  • spinal morning stiffness and improves with exercise
  • development of a question mark spine - loss of lumbar lordosis and increased thoracic kyphosis
  • associated include anterior uveitis, aortitis, pulmonary fibrosis and amyloidosis
  • enthesitis
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38
Q

What are the investigations for ankylosing spondylitis?

A
  • lumbar spine flexion measured with Schober’s test.
  • x-rays may show sclerosis and fusion of the sacroiliac joints and bony spurs from the vertebral bodies which can bridge the intervertebral disc resulting in fusion, producing a bamboo spine.
  • X-rays can be normal at time of presentation.
  • MRI can detect earlier features such as bone marrow oedema and enthesitis of the spinal ligaments.
  • 90% of sufferers are HLA-B27 positive
  • inflammatory markers may be raised
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39
Q

Explain the New York Criteria for diagnosing ankylosing spondylitis?

A
  1. Limited lumbar motion
  2. Lower back pain for 3 months
    1. Improved with exercise
    2. Not relieved by rest
  3. Reduced chest expansion
  4. Bilateral, Grade 2 to 4, sacroillitis on x-ray
  5. Unilateral, Grade 3 to 4, sacroillitis on x-ray

Definite AS if criteria 4 or 5 plus 1,2 or 3.

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40
Q

What is seen in this x-ray?

A

Shows a “bamboo spine” as seen in ankylosing spondylitis

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41
Q

What is seen in this image?

A

MRI showing signs of ankylosing spondylitis

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42
Q

What is the treatment for ankylosing spondylitis?

A
  • physiotherapy
  • exercise
  • NSAIDs
  • Anti-TNF inhibitors for more aggressive disease.
  • DMARDs are only used if there is peripheral joint inflammation.
  • Surgery is mainly reserved for hip and knee arthritis and kyphoplasty to straighten out the spine is controversial and carries considerable risk.
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43
Q

What is psoriatic arthritis?

A

Occurs in 30% of people affected by skin psoriasis.

10-15% of patients can have PsA without psoriasis.

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44
Q

What are the clinical features of psoriatic arthritis?

A
  • an asymmetrical oligoarthritis but may also affect the hands in a pattern similar to RA.
  • Spondylitis, dactylitis and enthesitis are common.
  • nail changes including nail pitting and onycholysis
  • enthesitis - achilles tendinitis and plantar fasciitis
  • extra-articular features in eye disease
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45
Q

What is seen in this image?

A

Deformities associated with psoriatic arthritis.

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46
Q

What rheumatological condition is associated with these changes?

A

Psoriatic arthritis - shows nail changes and psoriatic plaques. Swelling also associated with DIP joints

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47
Q

What are the investigations for psoriatic arthritis?

A

History

Examination

Bloods - Inflammatory parameters raised and negative RF

X-rays - marginal erosions and whiskering, pencil in cup deformity, osteolysis and enthesitis

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48
Q

What is the treatment of psoriatic arthritis?

A
  • DMARDs usually methotrexate.
  • Anti-TNF therapy is available for those who do not respond to standard treatment.
  • Joint replacement can be considered in larger joints which are severely affected, and DIP joint fusion can occasionally help.
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49
Q

What is enteropathic arthritis?

A

Refers to an inflammatory arthritis involving the peripheral joints and sometimes spine, occurring in patients with inflammatory bowel disease (Crohn’s and Ulcerative Collitis).

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50
Q

How does enteropathic arthritis present?

A
  • tends to be a large joint asymmetrical oligoarthritis.
  • 10-20% of IBD sufferers will experience spine or joint problems
  • worsening of symptoms during flare-ups of inflammatory bowel disease.
  • GI - loose, watery stool with mucous and blood
  • weight loss, low grade fever
  • eye involvement - uveitis
  • skin involvement - pyoderma gangrenosum
  • enthesitis - achilles tendonitis, plantar fasciitis, lateral epicondylitis
  • Oral - apthous ulcers
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51
Q

What are the investigations of enteropathic arthritis?

A
  • upper and lower GI endoscopy with biopsy showing ulceration/colitis
  • Joint aspirate - no organisms or crystals
  • raised inflammatory markers - CRP, PV
  • X-ray/MRI showing sacroiliitis
  • USS showing synovitis/tenposynovitis
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52
Q

How is enteropathic arthritis treated?

A

involves finding medication to manage both the underlying condition and the arthritis.

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53
Q

What is reactive arthritis?

A

Occurs in response to an infection in another part of the body, most commonly genitourinary infections (Chlamydia, Neisseria) or GI infections (Salmonella, Campylobacter).

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54
Q

What are the risk factors for reactive arthritis?

A

Affects young adults (20-40) with equal sex distribution

HLA B27 positive

55
Q

What are the clinical features of reactive arthritis?

A
  • large joints e.g., the knee become inflamed around 1-3 weeks following the infection.
  • general symptoms - fever, fatigue, malaise
  • asymmetrical monoarthritis
  • enthesitis
  • Some patients have Reiter’s Syndrome - urethritis, uveitis or conjunctivitis and arthritis
56
Q

What are the investigations for reactive arthritis?

A

History

Examination

Bloods - inflammatory parameters, FBC, U&Es, HLA B27

Cultures - blood, urine, stool

Joint fluid analysis

X-ray of affected joints

Ophthalmology opinion

57
Q

What are the treatment options for reactive arthritis?

A
  • IA or IM steroid injections
  • occasionally DMARDs are required in chronic cases.
58
Q

What is systemic lupus erythematosis?

A

A chronic autoimmune disease whose presentation and disease course can be highly variable. It mainly involves the skin, joints, kidneys, blood cells and nervous system but can affect almost any organ system.

59
Q

What is the pathogenesis of systemic lupus erythematosis?

A

One longstanding proposed mechanism for the development of autoantibodies involves a defect in apoptosis that causes increased cell death and a disturbance in immune tolerance. The defective clearance of the apoptotic cell debris allows for the persistence of antigen and immune complex production.

Many clinical manifestations of SLE are mediated by circulating immune complexes that form with antigens in various tissues. Immune complexes form in the small blood vessels, leading to complement activation and inflammation. Moreover, antibody-antigen complexes are deposited on the basement membranes of skin and kidneys.

60
Q

What are the risk factors for SLE?

A
  • 90% cases occur in women
  • frequently starting at childbearing age typically 20-30s
  • female to male ratio is 9:1
  • genetic component.
  • Commoner and more severe in those of Afro-Caribbean, Hispanic American, Asian and Chinese ethnicity
61
Q

What is the clinical presentation of SLE?

A
  • fever
  • fatigue
  • weight loss
  • arthralgia
  • myalgia
  • inflammatory arthritis
  • lupus nephritis
  • pleurisy
  • pleural effusion
  • pneumonitis
  • pulmonary embolism
  • interstitial lung disease
  • pericarditis
  • pericardial effusion
  • autoimmune hepatitis
  • pancreatitis
  • increased prevalence of avascular necrosis
  • malar rash
  • photosensitivity
  • discoid lupus
  • subacute cutaneous lupus
  • oral/nasal ulceration
  • Raynaud’s phenomenon
  • leukopenia
  • lymphopenia
  • anaemia
  • seizures
  • psychosis
  • headache
62
Q

What are the investigation for SLE?

A

There is no diagnostic test for lupus. The following may be useful:

  • FBC may show anaemia, leucopenia and thrombocytopenia
  • ANA - positive in >95% of patients not specific
  • Anti-dsDNA - specific and varies with disease activity
  • Anti-Sm - specific but low sensitivity
  • Anti-Ro, anti-La and anti-RNP - may eb seen in SLE but also in other conditions
  • C3/4 - low when disease activity especially renal disease.
  • Urinalysis - look for evidence of glomerulonephritis
  • Imaging studies - CT chest for interstitial lung disease, MRI brain for cerebral vasculitis, echo for pericardial effusion.
63
Q

What is the treatment for SLE?

A

For skin disease and arthralgia hydroxychloroquine, topical steroids and NSAIDs are commonly used.

If there is inflammatory arthritis or evidence of organ involvement then immunosuppression with medication like azathioprine or mycophenolate mofetil may be required. Corticosteroids at moderate doses may be used, ideally for short periods.

In severe organ disease e.g., lupus nephritis or CNS lupus then treatment tend to involve IV steroids and cyclophosphamide.

In unresponsive cases other therapies such as IV immunoglobulin and rituximab may be necessary.

Monitoring anti-dsDNA antibodies and complement levels regularly as these vary with disease activity and may give some warning of a disease flare.

64
Q

What is sjogren’s syndrome?

A

An autoimmune condition characterised by lymphocytic infiltrates in exocrine organs. It can be a primary condition or can occur secondary to other autoimmune conditions such as RA and SLE.

65
Q

What is the clinical presentation of SLE?

A
  • dryness of the eyes and mouth
  • arthralgia
  • fatigue
  • vaginal dryness
  • parotid gland swelling
  • peripheral neuropathy
  • interstitial lung disease
  • increased risk of lymphoma
  • unexplained increase in dental carries
66
Q

What are the investigations for sjogren’s syndrome?

A
  • Diagnosis requires four of the criteria below:
    • Ocular symptoms - dry eyes for more than 3 months, foreign-body sensation, use of tear substitutes more than 3 times daily
    • Oral symptoms - feeling of dry mouth, recurrently swollen salivary glands, frequent use of liquids to aid swallowing
    • Ocular signs - Schirmer test performed without anesthesia, positive vital dye staining results
    • Oral signs - abnormal salivary scintigraphy findings, abnormal parotid sialography findings, abnormal sialometry findings (unstimulated salivary flow)
    • Positive minor salivary gland biopsy findings
    • Positive anti-Ro and anti-La test
67
Q

What is the management of sjogren’s syndrome?

A

Largely symptomatic.

Lubricating eyedrops are used and saliva replacement products may be tried.

Regular dental care is important due to high risk of caries,

Pilocarpine can stimulate saliva production but often causes side effects such as flushing.

Hydroxychloroquine can sometimes help with arthralgia and fatigue.

Other immunosuppression would usually only be used in the context of organ involvement e.g., interstitial lung disease.

At increased risk of lymphoma.

68
Q

What is systemic sclerosis?

A

A systemic connective tissue disease which can cause excessive collagen deposition creating skin and internal organ changes. Characterised by vasculopathy, autoimmunity and fibrosis.

69
Q

What is the clinical presentation of systemic sclerosis?

A
  • Raynaud’s phenomenon - unusual to have condition without this
  • skin becomes thickened and tight
  • major features include centrally located skin sclerosis that affects the arms, face and/or neck.
  • Minor features include sclerodactyly and atrophy of the fingertips and bilateral lung fibrosis.
  • can have beaking or pinching of the nose
  • tightening of the skin around the mouth
  • telangiectasia
  • calcinosis in the digits
70
Q

What condition is this a characteristic presentation of?

A

Systemic Sclerosis

71
Q

What does this image show?

A

Raynaud’s Phenomenon

72
Q

What is limited sclerosis?

A

skin involved tends to be confined to face, hands and forearms and feet. Organ involvement tends to occur later. Anti-centromere antibody association

73
Q

What is diffuse sclerosis?

A

skin changes develop more rapidly and can involve the trunk. Early significant organ involvement. Anti-Scl-70 antibody association.

74
Q

What is the management of systemic sclerosis?

A

There is no one overall treatment.

Raynaud’s/digital ulcers - calcium channel blockers, iloprost, bosentan

Renal involvement - ACE inhibitors

GI involvement - proton pump inhibitors for reflux

Interstitial lung disease - immunosuppression usually with cyclophosphamide.

75
Q

What is mixed connective tissue disease?

A

A defined condition which features symptoms also seen in other connective tissue diseases.

76
Q

What is the clinical presentation of mixed connective tissue disease?

A
  • Raynaud’s phenomenon
  • Arthralgia/arthritis
  • Myositis
  • Sclerodactyly
  • Pulmonary Hypertension
  • Interstitial lung disease
77
Q

What are the investigations for mixed connective tissue disease?

A
  • Associated with anti-RNP antibodies.
  • Because of the risk of pulmonary hypertension, regular echocardiograms are suggested. Screening for ILD with pulmonary function tests should also take place.
78
Q

What is the treatment of mixed connective tissue disease?

A

Varies according to presentation.

Calcium channel blockers may be helpful for Raynaud’s

If there is significant muscle or lung disease, then immunosuppression may be required.

79
Q

What is anti-phospholipid syndrome?

A

A disorder that manifests clinically as recurrent venous or arterial thrombosis and/or foetal loss.

Some patients with APS have no evidence of any definable associated disease, while, in other patients, APS occurs in association with SLE or another rheumatic or autoimmune disorder.

80
Q

What is the clinical presentation of anti-phospholipid syndrome/

A
  • increased frequency of stroke or MI especially in younger individuals.
  • recurrent pulmonary emboli or thrombosis can lead to life-threatening pulmonary hypertension.
  • Late spontaneous foetal loss is common however it can occur at any time during pregnancy. Recurrent early foetal loss is also possible.
  • many patients have migraines
  • livedo reticularis
81
Q

What is catastrophic APS?

A

a rare, serious and often fatal manifestation characterised by multiorgan infarctions over a period of days to weeks.

82
Q

How is APS diagnosed?

A

Lupus anticoagulant, anti-cardiolipin antibodies and anti-beta 2 glycoproteins may be positive.

83
Q

What is the treatment of APS?

A

The mainstay of treatment is anti-coagulation for those with an episode of thrombosis.

In patients with recurrent pregnancy loss low molecular weight heparin is used during pregnancy (warfarin is teratogenic)

Patients who are found to have positive antibodies but who have never had had an episode of thrombosis do not require anti-coagulation.

84
Q

What is gout?

A

A crystal arthropathy caused by deposition of monosodium urate crystals within a joint. It is usually due to high serum uric acid levels. Uric acid is the final compound in the breakdown of purines in DNA metabolism.

85
Q

What are the causes of gout?

A

Increased Urate Production:

  • inherited enzyme defects
  • myeloproliferative/lymphoproliferative disorders
  • psoriasis
  • alcohol
  • high dietary purine intake e.g., red meat, seafood, corn syrup

Reduced Urate Excretion:

  • chronic renal impairment
  • volume depletion e.g., heart failure
  • hypothyroidism
  • diuretics
  • cytotoxics e.g., ciclosporin
86
Q

What are the risk factors of gout?

A
  • Genetic predisposition
  • dehydration
  • trauma
  • surgery
  • diuretics
  • alcohol
  • red meat

Rare under the age of 20.

Prevalence decreases after age 80

More common in men than women

Very rare in women before menopause

87
Q

What is the clinical presentation of gout?

A
  • painful
  • usually monoarthropathy 1st MTP > ankle > knee
  • settles in 10 days without treatment
  • settles in 3 days with treatment.
  • abrupt onset, often overnight
  • red
  • hot and swollen joint
  • symptoms may last 7-10 days if untreated then resolve
  • gouty tophi are painless white accumulations or uric acid which can occur in the soft tissues and occasionally erupt through the skin.
88
Q

What condition does this image show?

A

Gout

89
Q

What is seen in this image?

A

Gouty Tophi

90
Q

What is this?

A

Uric acid crystals seen in gout

91
Q

What are the investigations for gout?

A

A definitive diagnosis can be made by analysing a sample of synovial fluid with polarised microscopy (the fluid is also analysed with Gram stain and culture to exclude infection). uric acid crystals are needle shaped and display negative birefringence.

Serum uric acid raised (may be normal during acute attack)

X-rays

92
Q

What is the treatment for acute attacks of gout?

A

includes NSAIDs, corticosteroids, opioid analgesics and colchicine for patients who cannot tolerate NSAIDs.

93
Q

What is the treatment for recurrent attacks of gout?

A

allopurinol or other urate lowering therapies can prevent attacks but they should not be started until an acute attack has settled as they can potentiate a further flare (wait 1 week)

94
Q

What are the indications for prophylactic therapy in gout?

A
  1. One or more attacks of gout in a year inspite of lifestyle modification
  2. Presence of gouty tophi or signs of chronic gouty arthritis
  3. Uric acid calculi
  4. Chronic renal impairment
  5. Heart failure where unable to stop diuretics
  6. Chemotherapy patients who develop gout
95
Q

What is pseudogout?

A

Another crystal arthropathy causing acute arthritis but, in contrast to gout, it is caused by calcium pyrophosphate crystals.

96
Q

What is seen in this image?

A

Calcium pyrophosphate crystals

97
Q

What is chondrocalcinosis?

A

used when calcium pyrophosphate deposition occurs in cartilage and other soft tissues in the absence of acute inflammation. This increases with age

98
Q

What are the risk factors for pseudogout?

A
  • hyperparathyroidism
  • hypothyroidism
  • renal osteodystrophy
  • haemochromatosis
  • Wilson’s disease
  • Osteoarthritis
  • commoner in elderly
99
Q

How is pseudogout diagnosed?

A

Calcium pyrophosphate crystals are envelope shaped, mildly positive birefringent.

Marked rise in inflammatory markers

100
Q

What is the treatment for pseudogout?

A

Acute attacks - NSAIDs, corticosteroids and occasionally colchicine. Rehydration is important

There are no medications used as prophylaxis to prevent recurrence.

101
Q

What is polymyalgia rheumatica?

A

This is a relatively common chronic inflammatory condition of unknown aetiology that affects elderly individuals (very rare under 50 years).

Incidence higher in northern regions

Associated with temporal arteritis/ giant cell arteritis

102
Q

What is the clinical presentation of polymyalgia rheumatica?

A
  • proximal myalgia of the hip and shoulder girdles
  • morning stiffness that lasts for more than 1 hour
  • symptoms tend to improve as day goes on.
  • usually symmetrical
  • fatigue, anorexia, weight loss and fever may occur
  • reduced movement of shoulders, neck and hips
  • muscle strength is normal
103
Q

What are the investigations for polymyalgia rheumatica?

A

No specific diagnostic test for PMR but it is almost always associated with raised CRP and PV/ESR.

Symptoms respond very dramatically to low dose steroids (prednisolone 15mg) and this is sometimes used as a diagnostic tool.

104
Q

What is the treatment for polymyalgia rheumatica?

A

Prednisolone 15 mg with dose reducing over the course of around 18 months. By the end of this period the condition will have resolved in the majority of cases.

105
Q

What are the complications of polymyalgia rheumatica?

A

Approx. 15% of patients develop giant cell arthritis (GCA)

40-50% of patients with GCA have associated PMR.

106
Q

What is giant cell arteritis?

A

The most common form of systemic vasculitis in adults.

It is of unknown aetiology and occurs in older patients.

Typically occurs over the age of 50.

107
Q

What is the presentation of giant cell arteritis?

A
  • visual disturbances
  • headache
  • jaw claudication
  • scalp tenderness
  • fatigue
  • malaise
  • fever
  • visual loss is a co-morbidity in GCA
  • tender, enlarged, non-pulsatile temporal arteries.
108
Q

When is this seen?

A

In Giant cell arteritis

109
Q

How is giant cell arteritis diagnosed?

A

Histologically marked by transmural inflammation of the intima, media and adventitia of affected arteries as well as patchy infiltration by lymphocytes, macrophages and multinucleated giant cells.

Vessel wall thickening can result in arterial luminal narrowing resulting in subsequent distal ischaemia.

Inflammatory markers are almost always raised.

Most definitive test is a temporal artery biopsy. This should be done as soon as possible. A positive temporal artery biopsy has 100% specificity but relatively low sensitivity for the diagnosis of GCA. Due to patchy involvement of the artery. Typical biopsy findings include mononuclear infiltration or granulomatous inflammation, usually with multinucleated giant cells.

110
Q

What is the treatment of giant cell arteritis?

A

Corticosteroids, usually prednisolone 40mg if no visual impairment and 60mg if visual symptoms. This treatment should not be delayed for the biopsy.

Prednisolone treatment is tapered over around 2 years.

111
Q

What is polymyositis?

A

An idiopathic inflammatory myopathy that causes symmetrical, proximal muscle weakness.

112
Q

What are the risk factors of Polymyositis?

A

More common in women 2:1

affects adults older than 20 (esp. 45-60 years)

113
Q

What is the pathogenesis of polymyositis?

A

Evidence points towards a T-cell mediated cytotoxic process directed against unidentified muscle antigens. CD8 T cells long with macrophages, initially surround healthy non-necrotic muscle fibres and eventually invade and destroy them.

An autoimmune response to nuclear and cytoplasmic autoantigens is detected in about 60-80% of patients with polymyositis and dermatomyositis. Some serum auto-antibodies are shared with other auto-immune diseases, and some are unique to myositis.

114
Q

What is the clinical presentation of polymyositis?

A
  • symmetrical proximal muscle weakness
  • affects upper and lower extremities
  • insidious onset
  • difficulty with particular activities e.g., climbing stairs.
  • myalgia
  • dysphagia secondary to oropharyngeal and oesophageal involvement occurs in about one third of patients and is a poor prognostic sign.
  • interstitial lung disease occurs in 5-30% of patients.
115
Q

What is the investigations of polymyositis?

A

Inflammatory markers are often raised

Serum creatine kinase level is usually raised often more than 10 times normal.

Autoantibodies include ANA, anti-Jo-1 and anti-SRP

MRI may be used to localise the extent of muscle involvement including inflammation, oedema or scarring.

Electromyographic (EMG) findings are abnormal in almost all patients.

Muscle biopsy shows muscle fibres in varying stages of inflammation, necrosis and regeneration.

116
Q

What is the treatment of polymyositis?

A

Prednisolone initially around 40mg combined with immunosuppressive drugs such as methotrexate or azathioprine.

117
Q

What is the clinical presentation of dermatomyositis?

A
  • V-shaped rash over chest
  • heliotrope rash
  • Gottron’s sign
  • Shawl sign
118
Q

What are the investigations of dermatomyositis?

A

Inflammatory markers are often raised

Serum creatine kinase level is usually raised often more than 10 times normal.

Autoantibodies include ANA, anti-Jo-1 and anti-SRP

MRI may be used to localise the extent of muscle involvement including inflammation, oedema or scarring.

Electromyographic (EMG) findings are abnormal in almost all patients. Increased fibrillations, abnormal motor potentials, complex repetitive diagnosis

Muscle biopsy shows muscle fibres in varying stages of inflammation, necrosis and regeneration.

Examination - confrontational testing - direct testing of power and isotonic testing - 30 second sit to stand test.

119
Q

What is the management of dermatomyositis?

A

Prednisolone initially around 40mg combined with immunosuppressive drugs such as methotrexate or azathioprine.

120
Q

What is the risk of malignancy of dermatomyositis?

A

There is an associated risk of malignancy. This is found in around 25% in patients and is greatest in the 5 years following diagnosis. Common cancers include breast, ovarian, lung, colon, oesophagus and bladder.

Malignancy should be screened for at the time of diagnosis.

121
Q

What is fibromyalgia?

A

An unexplained condition causing widespread muscle pain and fatigue. Not associated with inflammation.

122
Q

What are the risk factors of fibromyalgia?

A
  • Commonest in young and middle aged patients
  • women more affected in 22-50 years
  • commoner in women 6:1
  • may begin after emotional or physical trauma
123
Q

What is the pathogenesis of fibromyalgia?

A

It is thought to be a disorder of central pain processing or a syndrome of central sensitivity. Patients tend to have a lower threshold of pain and of other stimuli, such as heat, noise and strong odours.

Although the pathogenesis of fibromyalgia is not completely understood, research shows biochemical, metabolic and immunoregulatory abnormalities.

May occur as a primary condition but is also seen in approx 25% of patients with RA and approx 50% of patients with SLE.

124
Q

What is the presentation of fibromyalgia?

A
  • Persistent >3 months widespread pain (pain/tenderness on both sides of the body, above and below the waist and includes the axial spine.
  • Fatigue - disrupted and unrefreshing sleep
  • cognitive difficulties
  • multiple other unexplained symptoms, anxiety and/or depression and functional impairment of activities of daily living
125
Q

How is fibromyalgia diagnosed?

A

No specific investigations and diagnosis is made on clinical basis.

Rule out other conditions such as hypothyroidism, rheumatoid arthritis, SLE, polymyalgia rheumatica etc

ACR Proposed Diagnostic Criteria:

  • Patient experiences widespread pain and associated symptoms
  • Symptoms have been present at the same level for 3 or more months
  • No other condition otherwise explains the pain.
126
Q

What is the management of fibromyalgia?

A

Self-management techniques

Validate patient illness

normal investigations which may be frustrating

Advice regarding graded exercise and activity pacing

Atypical analgesia including tricyclics (e.g., amitriptyline), gabapentin and pregabalin

Psychological input with techniques such as cognitive behavioural therapy

127
Q

What is Takayasu arteritis?

A

A form of large vessel vasculitis occurring in patients under the age of 50.

128
Q

What are the risk factors of Takayasu arteritis?

A
  • 100 times higher incidence in East Asian countries compared to Western population.
  • Most often affects young women in their second and third decades of life.
129
Q

What is the presentation of Takayasu arteritis?

A
  • low-grade fever
  • malaise
  • night sweats
  • weight loss
  • arthralgia
  • fatigue
  • claudicant symptoms in both upper and lower limbs
  • vascular stenosis
  • aneurysms
  • bruit with the most common location being the carotid artery
  • blood pressure difference of extremities - pulseless disease
130
Q

What are the investigations of Takayasu Arteritis?

A
  • ESR, PV and CRP are elevated
  • Imaging such as MR angiography can detect thickened vessel walls and stenosis
  • PET CT shows increased metabolic activity in the large vessels
131
Q

What is the treatment of Takayasu Arteritis?

A

Corticosteroids (starting at 40-60mg prednisolone) and gradually reducing.

Steroid sparing agents such as methotrexate and azathioprine may be added.

132
Q

What is granulomatosis with polyangiitis?

A

Granulomatous inflammation involving the respiratory tract and necrotising vasculitis affecting small- to medium-sized vessels e.g., capillaries, venules, arterioles and arteries.

133
Q

What is the presentation of granulomatosis with polyangiitis?

A
  • ENT symptoms including nosebleeds, deafness, recurrent sinusitis and nasal crusting
  • Respiratory symptoms such as haemoptysis and cavitating lesions on x-ray are frequently seen.
134
Q

What are the investigations for granulomatosis with polyangiitis?

A

Associated with cANCA and PR3.

X-rays may show lesions in chest.

ESR, PV and CRP is raised

U&E - to look for renal involvement.

MSK 2022 (11 decks)

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