Rheumatology Flashcards

1
Q

Name 4 systemic features of Marfan’s syndrome. (4)

Clue: MARFANS

A
Mitral prolapse
Aortic dissection
Regurg of aortic valve
Fingers long
Arm span>height
Nasal voice (high arched palate)
Sternal excavation
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2
Q

What is arthralgia? (1)

A

joint pains when the joint appears normal on examination

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3
Q

Describe the synovial membrane. (3)

A

Sterile, vascularised connective tissue without a basement membrane.
Contains type A cells (derived from monocytes) and type B cells (produce synovial fluid).
Synovial fluid contains hyaluronic acid.

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4
Q

What is the enthesis? 1)

A

Point on the bone at which ligaments and tendons insert.

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5
Q

What does hyaline cartilage consist of? (3)

A

Water, type 2 collagen and proteoglycans.

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6
Q

Name 3 differentials for polyarticular arthritis in a young adult? (3)

A

Males: reactive arthritis, ankylosing spondylitis
Both: Psoriatic arthropathy, Enteropathic arthropathy, Lyme disease, HIV
Females: SLE, RA, Sjogren’s

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7
Q

Name 3 differentials for polyarticular arthritis in a middle aged adult? (3)

A

Male: gout
Both: OA, Lyme disease, HIV
Female: RA

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8
Q

Name 3 differentials for polyarticular arthritis in an older adult? (3)

A

Both: OA, PM, Pseudogout

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9
Q

Name 3 differentials for a large joint monoarthritis. (3)

A

OA, gout, pseudogout, trauma, septic arthritis, disseminated gonnococcal infectino.
Less common: RA, spondyloarthopathies, TB, haemarthrosis.

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10
Q

What is arthroscopy? (1)

A

Direct means of visualising the inside of a joint, particularly knee and shoulder.

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11
Q

name 3 things that synovial fluid is analysed for? (3)

A

Colour, cell count, culture, glucose, viscosity, protein

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12
Q

How can golfer’s elbow and tennis elbow be distinguished? (2)

A

Tennis is lateral epicondyle tenderness caused by insertion of wrist extensor tendon.
Golfers is medial epicondyle tenderness caused by wrist flexor tendon.

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13
Q

Milo has back pain.

Name 3 red flags for back pain. (3)

A
Age 50 years
Thoracic pain
Constant pain without relief
History of TB, HIV, carcinoma or steroid use
Systemically unwell: ever, weight loss
Localised bone tenderness
Bilateral signs in the legs
Neurological deficit involving more than on e root level
Bladder, bowel or sexual dysfunction
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14
Q

Karl has lumbar back pain.
What are the differences in the history and examination that may distinguish between a mechanical and an inflammatory cause? (2)
Name 2 mechanical causes of lumbar pain? (2)
Name 2 inflammatory causes of lumbar pain. (2)

A

Mechanical: sudden onset, pain worse in evening, no morning stiffness, pain aggravated by exercise.
Causes: lumbar disc prolapse, spondylolisthesis, OA, fractures, spinal stenosis.

Inflammatory: gradual onset, morning stiffness, pain relieved by exercise, pain worst in the morning.
Ankylosing spondylitis, Infection.

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15
Q

Name 2 serious causes of lumbar back pain. (2)

A
Metastases
Multiple myeloma
TB osteomyelitis
Bacterial osteomyelitis
Spinal and root canal stenosis
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16
Q

Why is lumbar disc prolapse more a disease of younger people (20-40 years)? (1)

A

The inter vertebral discs degenerate with age and so in the elderly is no longer capable of prolapse

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17
Q

What are the most likely causes of sciatica in younger patients (1) and in older patients? (1)

A

Younger: Lumbar disc prolapse
Older: Osteophytes int he lateral recess of spinal canal causing compression.

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18
Q

Ralph has an S1 root lesion caused by a lumbar disc prolapse. Describe the symptoms he may be experiencing and signs on examination. (3)

A

Pain: from buttock, down back of thigh and leg to ankle and foot
Sensory: loss on sole of foot and posterior calf
Motor: loss of plantar flexion of ankle and toes
Reflex: loss of ankle jerk
Pain on straight leg raising test.

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19
Q

Troy has an L5 nerve root lesion caused by a lumbar disc prolapse. Describe the symptoms he may be experiencing and signs on examination. (3)

A

Pain: from buttock, to lateral aspect of leg and dorsum of foot
Sensory: loss of dorsum of foot and anterolateral aspect of lower leg
Motor: loss of dorsiflexion of foot and toes
Reflex: none affected

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20
Q

Yves has an L4 nerve root lesion caused by a lumbar disc prolapse. Describe the symptoms he may be experiencing and signs on examination. (3)

A

Pain: on lateral aspect of thigh to medial calf
Sensory: loss of medial aspect of calf and shin
Motor: weakness of dorsiflexion and inversion of ankle, and extension of knee
Reflex: loss of knee jerk
Positive femoral stretch test

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21
Q

What is spondylolisthesis? (1)

Which level is most commonly affected? (1)

A

Characterised by slipping forward of one vertebra onto another, most commonly at L4/5.

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22
Q

What is spinal stenosis? (2)

Name 2 causes. (2)

A

Narrowing of the lower spinal canal compresses cauda equina resulting in back and buttock pain, particularly after exercise.

Disc prolapse, degenerative osteophyte formation, tumour, congenital narrowing.

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23
Q

What is cervical spondylosis? (2)

A

Chronic cervical disc disease (in association with OA) causes stiffness and pain of the neck, with or without radiation to the arm.

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24
Q

Name 3 risk factors for developing OA. (3)

A
Females
FH
Obesity
Fracture through a joint
Congenital joint dysplasias
Pre-existing joint damage of any cause
Occupation (famers and labourers)
Repetitive use (associated with some sports)
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25
Q

What is the difference between primary and secondary OA? (2)

A

Primary OA occurs with no obvious predisposing factor

Secondary OA occurs in a damaged joint or congenitally abnormal joint.

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26
Q

What are osteophytes? (1)

A

Calcified cartilaginous growths at the margins of joints caused by repair attempts.

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27
Q

Name the 4 changes on x-ray that suggest OA. (4)

A

Narrowing of joint space
Osteophyte formation
Subchondral sclerosis
Subchondral cysts

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28
Q

Name 3 joints most commonly affected by OA. (3)

A
Distal interphalangeal joints
First Carpometocarpal joint
First Metatarsophalangeal joint 
Vertebrae
Hips
Knees

(Elbows, wrists and ankles are rarely affected)

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29
Q

What are Heberden’s and Bouchard’s nodes? (2)

A

Heberden’s nodes: bony swellings at the DIP’s

Bouchard’s nodes: bony swellings at the PIP’s.

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30
Q

Florence has severe OA of the knee.

Name 4 management options for her. (4)

A
Conservative: 
 - weight loss
 - physiotherapy: local strengthening and aerobic exercise
 - Heat or ice packs
 - Contralateral sided walking stick
 - Joint brace or support
 - Acupuncture 
Medical:
 - Paracetamol with weak opioid if necessary
 - Topical NSAIDs
 - Oral NSAIDs or Coxibs for short term
 - Intra-articular steroid injections
Surgical:
 - Total knee replacement
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31
Q

How would an FBC and an ESR result differ in John who has OA and Jackie who has RA? (2)

A

OA: normal
RA: normocytic normochromic anaemia, raised ESR/CRP

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32
Q

Name 3 risk factors for developing RA. (3)

A

Women before menopause.
FH
HLA-DR4

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33
Q

define RA. (2)

A

Chronic systemic autoimmune disorder causing a symmetrical polyarthritis

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34
Q

Describe the pathology of RA. (3)

A

RA is characterised by synovitis, with thickening of the synovial lining and infiltration by inflammatory cells.
There is generation of new synovial blood vessels and activated endothelial cells produce adhesion molecules (e.g. VCAM-1) which expedite extravasation of leucocytes into synovium.
Synovium proliferates and grows out over surface of cartilage producing tumour-like mass called pannus.
pannus destroys articular cartilage and subchondral bone causing bony erosions.

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35
Q

Describe some of the clinical articular features seen by patients with RA. (4)

A

Gradual onset of pain
Early morning stiffness lasting over 30 mins
Swelling in small joints of hands and feet
Spindling of fingers (swollen PIPs, unaffected DIPs)
Joint instability
Subluxation
Deformity of joints
Joint effusions
Wasting of muscles around affected joints
Ulnar deviation
Boutonniere deformity
Swan-neck deformity
Z shaped thumb

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36
Q

Name 4 non-articular manifestations of RA. (4)

A
  • Systemic: fever, fatigue, weight loss, greater risk of infection
  • Eyes: Sjogren’s, scleritis
  • Neuro: carpal tunnel, atlanto-axial subluxation, cord compression, polyneuropathy, mononuritis multiplex
  • Haem: Lymphadenopathy, Felty’s syndrome, anaemia, thrombocytosis
  • Pulm: pleural effusion, lung fibrosis, rheumatoid nodules in pleura or lung, rheumatoid pneumoconiosis, obliterative bronchiolitis
  • CV: pericarditis, pericardial effusion, pericardial rheumatoid nodules, Raynaud’s syndrome
  • Kidneys: amyloidosis, analgesic nephropathy
  • Vasculitis: leg ulcers, nail fold infarcts, gangrene of fingers and toes.
  • Periarticular: osteoporosis, bursitis, tenosynovitis, muscle wasting, subcut nodules over pressure points.
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37
Q

What is Felty’s syndrome? (1)

A

Triad of RA, Splenomegaly and neutropenia.

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38
Q

How is a diagnosis of RA made? (3)

A

Clinically: symmetrical peripheral polyarthritis with morning stiffness.
Bloods:
-FBC and ESR: normocytic, normochromic anaemia and thrombocytosis; ESR and CRP raised.
-Autoantibodies: anti-cyclic citrullinated peptide, Rf
Radiology:
-X-ray: soft tissue swelling in early disease and later joint narrowing, erosions at joint margins and porosis of periarticular bone and cysts.

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39
Q

What result would you expect in a analysis of synovial fluid? (1)

A

Uncomplicated disease: Synovial fluid is sterile with high neutrophil count.

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40
Q

Louise is a 27 year old female who has a symmetrical polyarthritis.
You suspect RA due to morning stiffness over 30 minutes.
Name 2 other differentials of a symmetrical polyarthritis. (2)

A

Symmetrical seronegative spondyloarthropathies
Form of Psoriatic arthritis (arthritis mutilans)
SLE (joints normal on examination)
Acute viral polyarthritis (usually recovered by 6 weeks)

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41
Q

Julie is a 54 year old smoker who has RA.
Name 3 healthcare professionals who should be involved in her care. (3)
Describe management options for her. (3)

A

Rheumatologists, Occupational health, Physiotherapists, Smoking cessation team, Orthopaedic surgeons.

  • Analgesia: NSAIDs, Coxibs, Paracetamol/dihydrocodeine
  • Steroids: suppress disease activity but large doses required, and long term is not advised.
  • DMARDs: inhibit inflammatory cytokines and used early to reduce disease inflammation. Also slows joint erosion, irreversible damage and reduces CV risk.
    Sulfasalazine (in mild-moderate disease & desire for family), Methotrexate (more active disease, teratogenic, do not use 3 months before conception), Leflunomide (blocks T cell proliferation)
  • Biologicals: TNF-a inhibitors (infliximab, etanercept); lysis of B cells (rituximab); IL-6 receptor antibody (tocilizumab)
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42
Q

When can biological agents be tried in a patient with RA? (2)

A

Active disease despite adequate treatment with at least 2 DAMRDs, including methotrexate.
TNF-a inhibitors are the first line and slow or halt erosion formation in up to 70% of patients.

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43
Q

Why is regular monitoring of FBC undertaken in DMARD therapy? (1)

A

Myelosuppression (anaemia, thrombocytopenia and neutropenia)

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44
Q

Roger has been told he has a seronegative spondyloarthropy and would like to know what clinical features are common within this group of diseases.
Name 3. (3)

A
  • Predilection for axial (sacra and sacroiliac) inflammation.
  • Asymmetrical peripheral arthritis
  • Absence of Rf
  • Inflammation of the enthesis
  • Strong associated with HLA-B27
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45
Q

Tom is a 24 year old man who attends GP surgery with increasing pain and morning stiffness in his back and buttocks, which improves with exercise but not with rest.
He has also noticed progressive restriction in the movement of his spine.
What do you suspect? (1)
What do you expect to find on inspection of the spine? (2)

A

Ankylosing spondylitis

  • Loss of lumbar lordosis and increased kyphosis
  • Limitation of lumbar spine mobility in both sagittal and frontal planes. (Spinal flexion measured by Schober test)
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46
Q

What is Schober test? (2)

A

Assessment of spinal flexion. Mark is made at L5 spinous process and 10cm above with patient erect. Normal result is >15cm on bending forward.

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47
Q

Name 3 seronegative spondyloarthropathies. (3)

A
Ankylosing spondylitis
Psoriatic arthritis
Reactive arthritis
Post-dysenteric reactive arthritis
Enteropathic arthritis (UC or Crohn's)
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48
Q

Name 2 non auricular features sometimes seen in patients with ankylosing spondylitis. (2)

A

Anterior uveitis
Aortic incompetence
Cardiac conduction defects
Apical lung fibrosis

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49
Q

What changes may you seen in an x-ray of ankylosing spondylitis? (2)

A
  • Normal
  • Erosion and sclerosis of margins of the sacroiliac joints
  • Blurring of upper or lower vertebral rims at the thoracolumbar junction caused by enthesitis at insertion of the intervertebral ligaments.
  • Syndesmophytes: enthesitis heals with new bone formation resulting in bony spurs (syndesmophytes)
  • Bamboo spine: progressive calcification of interspinous ligaments and syndesmophytes.
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50
Q

What is the management of ankylosing spondylitis? (1)

A

Morning exercises to maintain posture and mobility.
Slow release NSAIDs at night for morning pain.
Methotrexate helps peripheral arthritis but not spinal disease
TNF-a blocking drugs are effective in active disease and help both spinal and peripheral arthritis.

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51
Q

What proportion of patients with psoriasis will develop psoriatic arthritis? (1)

A

20% (especially if nail disease)

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52
Q

Name 2 types of psoriatic arthritis. (2)

A
  • Asymmetrical involvement of the small joints of hand
  • Symmetrical seronegative polyarthritis resembling RA
  • Arthritis mutilans: severe with destruction of small bones in hands and feet
  • Sacroilitis: uni or bilateral
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53
Q

How is psoriatic arthritis treated? (2)

A

Analgesia and NSAIDs.
Local synovitis: intra-articular corticosteroid injections
Sever: methotrexate or TNF-a blockers

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54
Q

Name 2 causes of reactive arthritis. (2)

A

Reactive arthritis is a sterile synovitis caused by;

  • GI: shigella, campylobacter, yersinia or salmonella
  • STI: non-specific urethritis or cervicitis due to chlamydia
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55
Q

What is Reiter’s syndrome? (2)

A

Urethritis, reactive arthritis and conjunctivitis

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56
Q

What is enteropathic arthritis? (2)

A

Large joint mono or asymmetrical oligoarthritis occurring in 10-15% of UC/Crohn’s patients. It usually mirrors disease activity.

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57
Q

What are the two types of crystals that can cause arthritis? (2)

A
Sodium urate (gout)
Calcium pyrophosphate (pseudogout)
58
Q

Name 3 causes of gout. (3)

A
Impaired excretion of uric acid:
 -CKD
 -Thiazides, aspirin
 -Hypertension
 -Lead toxicity
 -Primary hyperparathyroidism
 -Hypothyroidism
 -Increased lactic acid production from alcohol, exercise
Increased production of uric acid:
 -Increased turnover of purines
 -Myeloproliferative disorder (eg polycythaemia rubra vera)
 -Lymphoproliferative disorders (eg leukaemia)
 -Severe psoriasis
59
Q

Name 3 of the 4 clinical syndrome associated with hyperuricaemia and crystal deposition. (3)

A

Acute sodium urate synovitis (acute gout)
Chronic polyarticular gout
Chronic tophaceous gout
Urate renal stone formation

60
Q

Which joint is most commonly affected by acute gout? (1)

A

Metatarsophalangeal joint of the big toe (75%)

61
Q

How does chronic tophaceous gout present? (2)

A

Large smooth white deposits (tophi) in the skin and around he joints, particularly the ear, fingers and Achilles tendon.

62
Q

What would you expect to find on joint aspiration of a joint affected by gout? (2)

A

Long needle shaped, negatively birefringent under polarised light.

63
Q

Name 2 medications used in the treatment of acute gout. (2)

A

NSAIDs
Colchicine
Intra-articular methylprednisolone

64
Q

When is prevention given for acute gout? (1)

A
  • If more than 2 attacks per year, despite dietary changes
  • Gouty tophi
  • Renal impairment
65
Q

Allopurinol may exacerbate an acute attack of gout, when should it be initiated? (1)

A

At least one month after an acute attack.

66
Q

Name one drug that can be used as an alternative to allopurinol. (1)

A

Febuxostat

67
Q

Name 2 bits of dietary advice for a patient with repeat attacks of gout. (2)

A
Lose weight
Reduce alcohol consumption
Stop thiazides and aspirin
Reduce total cholesterol intake
Avoid purine rich foods: spinach, offal, some fish and shellfish
68
Q

What is chondrocalcinosis? (1)

A

Seen on x-ray in pseudo gout as linear calcification parallel to articular surfaces, it is caused by deposition of calcium pyrophosphate in the articular cartilage and periarticular tissue.

69
Q

How does pseudo gout present? (2)

A

Shedding of crystals causes an acute synovitis resembling gout, except that it most commonly affects the knees and wrists most frequently in elderly women.

70
Q

What would yo expect to see after joint aspiration of a joint affected by pseudo gout? (2)

A

Small, cuboidal pyrophosphate crystals that are positively birefringent under polarised light.

71
Q

What is the treatment of pseudo gout? (1)

A

Joint aspiration
NSAIDs or colchicine
(injection of local steroids can also help)

72
Q

Name 3 risk factors for septic arthritis. (3)

A
Prosthetic joints
Pre-existing joint disease
Recent intra-articular joint infection
Diabetes mellitus
Direct injury to a joint
73
Q

What clinical features would suggest septic arthritis? (3)

A

Hot, painful, swollen, red joint (80% monoarticular)

Fever or other evidence of infection.

74
Q

Name 3 bacteria that can can lead to joint involvement after blood-borne spread? (3)

A

Gonococcal
Meningococcal (polyarthritis from deposition of immune complexes containing meningococcal antigens)
Tuberculosis (hip, knee and spine most common)

75
Q

Define osteomyelitis. (2)

A

Infection of bone, usually caused by haematogenous spread (e.g. from boil) or due to local infection.
Staphylococcus is most common cause, also haemophilus influenza and Salmonella (in SCD).

76
Q

What are the clinical features of osteomyelitis? (2)

A

Fever, local pain, erythema and sinus formation in chronic osteomyelitis.

77
Q

How is osteomyelitis diagnosed? (1)

A

CT, MRI or bone scan

78
Q

Fred has an acute mono arthritis of the knee. You expect Fred has septic arthritis.
Name 3 tests that may help to diagnose it. (3)

A

Joint aspiration and synovial fluid analysis
FBC, ESR, CRP, blood cultures
Swab urethra, and anorectic if gonococcal infection suspected.
(xray do not help in diagnosis)

79
Q

What treatment would you give for acute non-gonnococcal bacterial arthritis? (2)

A

Empirical: flucloxacillin 1-2g qds (erythromycin if allergic)
oral fusidic acid 500mg tis.
Add gentamicin if immunosuppressed to cover gram negative.

80
Q

What is the difference between autoimmune rheumatic diseases and autoimmune organ specific diseases? (2)

A

Rheumatic autoimmune disease autoantibodies are not organ specific causing diverse, systemic features.

81
Q

name 2 rheumatic autoimmune diseases. (2)

A

SLE
Anti-phospholipid syndrome
Systemic sclerosis
Polymyositis and dermatomyositis

82
Q

Who is the typical patient presenting with SLE? (2)

A

Young (20-40y)
Female
African descent more common

83
Q

Name 2 drugs that may cause a lupus like syndrome. (2)

A

Hydralazine
Isoniazid
Penicillamine
Procainamide

84
Q

How is SLE diagnosed? (5)

A

4/11 diagnostic criteria from American College of Rheumatology

  • Serositis
  • Oral ulcers
  • Arthritis (non-erosive)
  • Photosensitivity
  • Blood disorders (thrombocytopenia, haemolytic anaemia, leukopenia)
  • Renal (proteinuria/cellular casts)
  • ANA (anti-ANA abs)
  • Immunologic (anti-dsDNA, anti-Sm, anti-phospholipid)
  • Neuropsych (seizures or psychosis)
  • Malar rash
  • Discoid rash
85
Q

What is the management for SLE? (3)

A

Mild: avoid sunlight and topical steroids. Simple analgesia. hydroxychloroquine.
Mod: oral steroids or immunosuppressants (azathioprine, cyclophosphamide) especially for renal or cerebral disease
Resistant: Rituximab

86
Q

Which other disease is anti-phospholipid syndrome most linked to? (1)
How is anti-phospholipid syndrome characterised? (2)

A

SLE

Characterised by thrombosis or recurrent miscarriages, and persistently positive anti-phospholipid antibodies.

87
Q

Name 3 clinical features/complications of anti-phospholipid syndrome. (3)

A

Artery: TIAs, strokes, MI
Veins: DVT, Budd-Chiari syndrome
Placenta: recurrent miscarriages
Others: valvular heart disease, migraine, epilepsy, thrombocytopenia, renal impairment and accelerated atheroma.

88
Q

What is the management of a patient with anti-phospholipid syndrome? (2)

A

Previous thrombosis: warfarin
Pregnancy: aspirin and LMWH
No history of thrombosis: aspirin and clopidogrel

Accelerated atheroma formation: secondary prevention

89
Q

What is systemic sclerosis (scleroderma)? (2)

A

Rare connective tissue disease characterised by widespread small vessel damage and fibrosis in skin and internal organs.
Aetiology is unknown.

90
Q

What are the main types of systemic sclerosis? (2)

A

Limited cutaneous systemic scleroderma (~70%) (formerly CREST syndrome)
Diffuse cutaneous systemic sclerosis (~30%)

91
Q

What is CREST syndrome? (1)

A

Previous term for limited cutaneous systemic scleroderma.
Calcinosis (palpable subcut calcium nodules in fingers)
Raynaud’s phenomenon
Esophageal dysmotility
Sclerodactyly
Telangiectasia

92
Q

What features may be seen in limited cutaneous systemic scleroderma? (4)

A
Raynaud's (early sign)
Microstomia
Beaked appearance of nose
Thickened skin
Sclerodactyly
Telangiectasia
Calcium depositions in the fingers (palpable sub cut nodules)
Oesophageal dysmotility
93
Q

What are the features of diffuse systemic cutaneous scleroderma? (2)

A

DcSSc is less common, but skin changes occur more rapidly and are more widespread.
Early organ involvement includes;
-GI: dilatation and atony in the oesophagus, small intestine and colon
-Renal: AKI and CKD. Acute hypertensive crisis is complication.
-Lung: fibrosis and pulmonary hypertension
-Myocardial fibrosis: arrhythmias and conduction disturbances.

94
Q

Name 3 investigations that may help to diagnose scleroderma. (3)

A
FBC: normocytic anaemia
ESR: may be raised
U+E: renal disease
Serum antibodies: 
 - Anti-nuclear antibodies
 - Anti-topoisomerase 1 (Scl 70) (DcSSc)
 - Anti-centromere antibodies (LcSSc)
X-ray of hands: calcinosis
HRCT: lung fibrosis
Barium swallow: oesophageal dysmotility
Oesophageal manometry: failure of peristalsis in distal oesophagus.
95
Q

What is the management of scleroderma? (1)

A

Symptomatic management

ACEi first line for hypertension and renal protection.

96
Q

What is polymyositis? (2)

A

Rare muscle disorder of unknown aetiology, characterised by inflammation and necrosis of skeletal muscle fibres.
Skin involvement occurs in dematomyositis.

97
Q

Describe the clinical features of polymyositis. (3)

A

Symmetrical progressive muscle weakness and wasting, affecting proximal muscles of pelvic and shoulder girdle. (Difficulty in rising from chair, raising arms above head, using stairs)
Involvement of pharyngeal, laryngeal and respiratory muscles: dysphagia, dysphonia, respiratory failure.

98
Q

What is the clinical features of dermatomyositis? (2)

A

Symmetrical progressive muscle weakness and wasting of proximal muscles of pelvic and shoulder girdle.
Involvement of pharyngeal, laryngeal and respiratory muscles.
SKIN: heliotrope (purple) discolouration of eyelids, scaly erythematous plaques over knuckles.

99
Q

Name 2 tests that may help in the diagnosis of polymyositis and dermatomyositis? (2)

A
Muscle biopsy (definitive test)
Raised serum muscle enzymes (CK)
Anti-JO antibodies
EMG (electromyography)
MRI: areas of muscle inflammation
100
Q

What is the management of dermatomyositis and polymyositis? (2)

A

Oral prednisolone and immunosuppressives if disease relapse on tapering of steroids.

101
Q

What is Sjogren’s syndrome? (2)

A

Autoimmune rheumatic disease characterised by destruction of epithelial exocrine glands, a specially lacrimal and salivary glands.
Predominantly in middle aged females.

102
Q

What is the difference between primary and secondary Sjogren’s syndrome? (2)

A

Primary: occurs alone
Secondary: occurs with another autoimmune disease (most commonly RA or SLE.

103
Q

What are the 2 main clinical features of Sjogren’s? (2)

A
Xerostomia (dry mouth)
Keratoconjunctivitis sicca (dry eyes)
104
Q

What antibodies may be present in Sjogren’s? (2)

A

Anti-nuclear antibodies
Anti-Ro
Rf

105
Q

What is Schirmer test? (2)

A

Filter paper on lower eye lid, positive test confirms defective tear production. (wetting of <10mm in 5 mins is positive)

106
Q

What is systemic vasculitides? (2)

A

Group of multi system disorders in which vasculitis in the principal feature and classification is based on size of vessels affected.
All are associated with anaemia, raised ESR and rare (except GCA).

107
Q

What is the lower age limit for diagnosis of polymyalgia rheumatica or giant cell arteritis? (1)

A

Over 50 years old.

108
Q

What are the clinical features of PMR/GCA? (2)

A

PMR: abrupt onset of stiffness and pain in the muscles of neck, shoulder, hips and lumbar spine. May have malaise, fever, weight loss and anorexia.
GCA: headache, tenderness over scalp or temple and claudication of the jaw.

109
Q

Name 2 complications of GCA. (2)

A

Stroe if vertebrobasilar or carotid arteries affected.

Sudden loss of vision caused by opthalmic artery involvement (may be permanent)

110
Q

GCA should be treated once suspected due to risk of permanent blindness.
When should a temporal artery biopsy be taken to confirm suspicion? (1)

A

Before treatment or within one week of starting steroids.

111
Q

How is GCA or PMR managed? (2)

A

Prenisolone
PMR: 10-15mg OD
GCA: 60mg OD, reduced by weekly increments of 5mg, until 10mg OD then reduce by 1mg every 2-4 weeks.

112
Q

How can the response to treatment be assessed in PMR/GCA? (1)

A

Symptomatically or by ESR.

113
Q

What infection is associated with polyarteritis nodosa? (1)

A

Hepatitis B antigenaemia

114
Q

What is Churg-Strauss syndrome? (3)

A

Triad of asthma, eosinophilia and a systemic vasculitis affecting peripheral nerves and skin (nodules, petechiae and purpura).

Kidney involvement is uncommon.

treat with corticosteroids in combination with immunosuppressives.

115
Q

What is Henoch-Schonlein purpura? (3)

A

Precipitated by URTI.
Most commonly seen in children with purpuric rash n legs and buttocks.
Abdominal pain, arthritis, haematuria and nephritis also occur.
Characterised by vascular deposition of IgA-dominant immune complexes.
recovery is spontaneous.

116
Q

What is a cryoglobulin? (1)

A

Immunoglobulin or complement components that precipitate out at lower temperatures causing purpura, glomerulonephritis and polyneuropathy.

117
Q

What is Behcet’s disease? (2)

A

rare multisystem chronic disease of unknown cause.
Characterised by recurrent oral ulcers, and any 2 of; genital ulcers, eye lesions, skin lesions or a positive skin pathergy test (skin injury leads to pustule formation in 48 hours)

118
Q

What are the two major cell types involved in bone remodelling? (2)

A

Osteoblasts: produce type 1 collagen and regulate it’s mineralisation
Osteoclasts: produce hydrogen ions and lysosomal enzyme which remove and resorb the mineral phase and collagen matrix.

119
Q

Name 3 substances that can have an effects of bone metabolism. (3)

A
Vitamin D
PTH
Calcitonin
Glucorticoids
GH
Sex hormones
Thyroid hormones
120
Q

PTH aims to increase serum calcium levels and decrease serum phosphate levels.
Name 2 methods in which it does this. (2)

A

Increased osteoclastic resorption of bone
Increased intestinal absorption of calcium
Increased synthesis of 1, 25-dihydroxyvitamin D3
Increased renal tubule reabsorption of calcium

121
Q

Define osteoporosis. (2)

A

Reductino in bone mass and micro-architectural deterioration of bone tissue leading to bone fragility an increased risk of fracture.
Bone mineral density of more than 2 standard deviations below the young adult mean value (T score <-2.5)

122
Q

If a DXA scan gave a T score of -2.5, what is the condition? (1)

A

Osteoporosis

123
Q

If a dual-energy x-ray absorpiometry scan gave a T score of -1.5, what is the condition? (1)

A

Osteopenia (T-score between -1 and -2.5)

124
Q

Name 4 risk factors for osteoporosis and fragility fractures. (4)

A

BMD-dependent

  • female
  • caucasian/asian
  • immobilisation
  • chronic liver disease
  • chronic renal disease
  • COPD
  • Low dietary calcium intake
  • vitamin D insufficiency
  • drugs (steroids, heparin, anticonvulsants, ciclosporin)
  • Endocrine disease (Cushing’s, hyperthyroid, hyperparathyroid)
  • Other: DM, multiple myeloma, osteogenesis imperfecta

BMD-independent

  • elderly
  • previous fragility fracture
  • FH of hip fracture
  • low BMI
  • smoking
  • alcohol abuse
  • RA
  • increased risk of falls
125
Q

Name 3 of the 4 commonest sites for fractures associated with osteoporosis. (3)

A

Thoracic spine
Lumbar spine
Proximal femur
Distal radius (Colles’ fracture)

126
Q

Which 2 areas does a dual-energy x-ray absorptiometry scan measure bone density? (2)

A

Proximal femur

Lumbar spine

127
Q

Name 2 causes of secondary osteoporosis. (2)

A
Thyrotoxicosis
Hyperparathyroidism
Coeliac
Hypogonadism
Myeloma
128
Q

What can be used to assess the fracture risk of a patient? (1)

A

FRAX: WHO fracture risk assessment tool works out the 10 year fracture risk.

129
Q

What is the difference between the T and Z-score in DXA scans? (2)

A

T-score is number of SD’s away from the BMD of young normal mean BMD. (Defines osteoporosis)
Z-score is the number of SD’s away from the BMD of age-matched mean BMD. (Determines if investigations for secondary causes is warranted)

130
Q

What is the management of osteoporosis? (2)

A

Prevention: Smoking cessation, reduce alcohol intake, adequate intake of calcium and vitamin D, regular weight bearing exercise.
Medical:
-bisphosphonates first line
-strontium ranelate if unable to comply with dosage regimen of bisphosphonates
-raloxifene (SERM) stimulates ER on bone but not endometrium
-teriparatide (synthetic PTH)
-HRT is second line sue to risks of breast cancer and CVD
-Testosterone for men with evidence of hypogonadism

131
Q

What is Paget’s disease of the bone? (2)

A

Focal disorder of bone remodelling in which there is increased osteoclastic bone absorption, followed by formation of weaker new bone, increased local bone blood flow and fibrous tissue.

132
Q

Where are the commonest sites affected by Paget’s disease of the bone? (2)

A
Pelvis
Lumbar spine
Thoracic spine
Femur
Skull
Tibia
133
Q

What are the clinical features of Paget’s disease of the bone? (2)

A

Asymptomatic
Pain in the bone or nearby joint
Deformities: bowing of tibia, enlargement of skull
Complications: nerve compression (deafness, paraparesis), pathological fractures, rarely high output cardiac failure (due to increased bone blood flow) and osteogenic sarcoma.

134
Q

Name 2 investigations in the diagnosis of Paget’s disease. (2)

A

Serum ALP: reflects level of bone formation (Ca and PO4 are normal)
Urinary hydroxyproline excretion can be used as a marker for disease activity.
X-ray: characteristic changes; localised bony enlargement and distortion, sclerotic changes (increased density) and osteolytic areas.
Radionuclide bone scan showing increased uptake of bone-seeking radionuclides.

135
Q

What is the management of Paget’s disease of the bone? (2)

A

Bisphosphonates inhibit bone resorption by decreasing osteoclastic activity and form the mainstay of treatment.
Monitor disease by symptoms, ALP or urinary hydroxyproline.

136
Q

What are the diseases caused by profound vitamin D deficiency? (2)

A

Rickets in children
Osteomalacia in adults

Both are inadequate mineralisation of the osteoid framework leading to soft bones.

137
Q

Name 3 groups at risk of vitamin D deficiency. (3)

A
Pigmented skin
Use of sunscreen
Concealing clothing
Elderly
Institutionalised
Malabsorption
Short bowel
Renal disease
Cholestatic liver disease
Anti-convulsants, rifampicin, HAART
138
Q

What would you expect biochemistry results to be in a patient with osteomalacia? (2)

A

ALP high

CA and PO4 normal or low

139
Q

What characteristic feature would you expect to see on x-ray of a patient with osteomalacia? (1)

A

Defective mineralisation and Looser’s zones (low density bands extending from cortex inwards in the shafts of long bones).

140
Q

What is Marfan’s syndrome? (2)

A

Autosomal dominant disorder associated with mutations in the fibrillin 1 gene on chromosome 15.
Abnormalities of the collagen cause fragility of the skin and bruising.

141
Q

Name 2 complications of Marfan’s syndrome. (2)

A

Upward dislocation of the lens due to weakness of the suspensory ligament.
CV: ascending aortic aneurysm formation, aortic dissection and aortic valve incompetence.
Spontaneous pneumothorax