Multi-system Autoimmune Disease Flashcards

1
Q

… are forms of arthritis that usually strike the bones in your spine and nearby joints.

A

Spondyloarthropathies are forms of arthritis that usually strike the bones in your spine and nearby joints.

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2
Q

Spondyloarthropathies types

  • … spondylitis
  • Psoriatic Arthropathy
  • … Arthropathy
  • Intestinal Arthropathy
  • … Ankylosing Spondylitis
    • Hallmark ocular involvement is UVEITIS
A
  • Ankylosing spondylitis
  • Psoriatic Arthropathy
  • Reactive Arthropathy
  • Intestinal Arthropathy
  • Juvenile Ankylosing Spondylitis
    • Hallmark ocular involvement is UVEITIS
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3
Q

Spondyloarthropathies types

  • Ankylosing spondylitis
  • … Arthropathy
  • Reactive Arthropathy
  • … Arthropathy
  • Juvenile Ankylosing Spondylitis
    • Hallmark ocular involvement is …
A
  • Ankylosing spondylitis
  • Psoriatic Arthropathy
  • Reactive Arthropathy
  • Intestinal Arthropathy
  • Juvenile Ankylosing Spondylitis
    • Hallmark ocular involvement is UVEITIS
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4
Q

Spondyloarthropathies types

  • … spondylitis
  • … Arthropathy
  • … Arthropathy
  • Intestinal Arthropathy
  • Juvenile Ankylosing Spondylitis
    • Hallmark … involvement is UVEITIS
A
  • Ankylosing spondylitis
  • Psoriatic Arthropathy
  • Reactive Arthropathy
  • Intestinal Arthropathy
  • Juvenile Ankylosing Spondylitis
    • Hallmark ocular involvement is UVEITIS
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5
Q

CLINICAL CHARACTERISTICS OF SPONDYLOARTHROPATHIES

  • Pattern of peripheral arthritis: predominantly … limb, asymmetric
  • E..
  • Tendency to radiographic sacroiliitis
  • Absence of rheumatoid factor / anti CCP
  • Extra-articular features characteristic of the group (such as anterior uveitis)
  • Significant … aggregation
  • Association with HLA-…
A
  • Pattern of peripheral arthritis: predominantly lower limb, asymmetric
  • Enthesitis
  • Tendency to radiographic sacroiliitis
  • Absence of rheumatoid factor / anti CCP
  • Extra-articular features characteristic of the group (such as anterior uveitis)
  • Significant familial aggregation
  • Association with HLA-B27
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6
Q

CLINICAL CHARACTERISTICS OF SPONDYLOARTHROPATHIES

  • Pattern of peripheral arthritis: predominantly lower limb, …
  • Enthesitis
  • Tendency to radiographic …
  • Absence of … factor / anti …
  • Extra-articular features characteristic of the group (such as anterior …)
  • Significant familial aggregation
  • Association with …-B27
A
  • Pattern of peripheral arthritis: predominantly lower limb, asymmetric
  • Enthesitis
  • Tendency to radiographic sacroiliitis
  • Absence of rheumatoid factor / anti CCP
  • Extra-articular features characteristic of the group (such as anterior uveitis)
  • Significant familial aggregation
  • Association with HLA-B27
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7
Q

… is inflammation of the …, the sites where tendons or ligaments insert into the bone

A

Enthesitis is inflammation of the entheses, the sites where tendons or ligaments insert into the bone

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8
Q

Ankylosing Spondylitis - Disease Probability

  • Chronic back pain - ‘inflammatory’ (requires the presence of at least four of the following)
    • Age at onset less than … years
    • … onset
    • Improvement with …
    • No improvement with …
    • … pain with improvement upon getting up
  • Enthesitis
    • family history
  • Uveitis
  • Asymmetic arthritis
    • Response to NSAIDs
A
  • Chronic back pain - ‘inflammatory’ (requires the presence of at least four of the following)
    • Age at onset less than 40 years
    • Insidious onset
    • Improvement with exercise
    • No improvement with rest
    • Night pain with improvement upon getting up
  • Enthesitis
    • family history
  • Uveitis
  • Asymmetic arthritis
    • Response to NSAIDs
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9
Q

Ankylosing Spondylitis - Disease Probability

  • Chronic back pain - ‘inflammatory’ (requires the presence of at least four of the following)
    • Age at onset less than 40 years
    • … onset
    • Improvement with exercise
    • No improvement with rest
    • Night pain with improvement upon getting up
  • E…
    • … history
  • Uveitis
  • … arthritis
    • Response to …
A
  • Chronic back pain - ‘inflammatory’ (requires the presence of at least four of the following)
    • Age at onset less than 40 years
    • Insidious onset
    • Improvement with exercise
    • No improvement with rest
    • Night pain with improvement upon getting up
  • Enthesitis
    • family history
  • Uveitis
  • Asymmetic arthritis
    • Response to NSAIDs
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10
Q

Ankylosing Spondylitis - Disease Probability

  • Chronic back pain - ‘inflammatory’ (requires the presence of at least … of the following)
    • Age at onset less than 40 years
    • Insidious onset
    • Improvement with exercise
    • No improvement with rest
    • Night pain with improvement upon getting up
  • E…
    • family history
  • U…
  • Asymmetic arthritis
    • Response to …
A
  • Chronic back pain - ‘inflammatory’ (requires the presence of at least four of the following)
    • Age at onset less than 40 years
    • Insidious onset
    • Improvement with exercise
    • No improvement with rest
    • Night pain with improvement upon getting up
  • Enthesitis
    • family history
  • Uveitis
  • Asymmetic arthritis
    • Response to NSAIDs
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11
Q

Ankylosing Spondylitis - Disease Probability

  • Chronic back pain - ‘inflammatory’ (requires the presence of at least four of the following)
    • Age at onset less than … years
    • Insidious onset
    • Improvement with …
    • No improvement with …
    • Night pain with … upon getting up
  • Enthesitis
    • family history
  • Uveitis
  • Asymmetic arthritis
    • Response to NSAIDs
A
  • Chronic back pain - ‘inflammatory’ (requires the presence of at least four of the following)
    • Age at onset less than 40 years
    • Insidious onset
    • Improvement with exercise
    • No improvement with rest
    • Night pain with improvement upon getting up
  • Enthesitis
    • family history
  • Uveitis
  • Asymmetic arthritis
    • Response to NSAIDs
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12
Q

Imaging for Ankylosing Spondylitis diagnosis

A

Sacroiliac joint X-ray, if normal or equivocal, then CT or MRI, or HLA-B27 test

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13
Q

In patients with over … months back pain, with/without peripheral manifestations and age at onset 1 SpA feature or HLA-… + plus >2 other SpA features

A

In patients with over 3 months back pain, with/without peripheral manifestations and age at onset <45 years - Sacroiliitis on imaging plus >1 SpA feature or HLA-B27 + plus >2 other SpA features

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14
Q

Ankylosing Spondylitis

  • Greek ‘ankylos‘- bent/crooked (fused) - ‘spondylos‘-a spinal vertebra
  • Characteristically affects … adults
  • Peak onset between …-… yrs
  • Male:female - approximately 3:1*
A
  • Greek ‘ankylos‘- bent/crooked (fused) - ‘spondylos‘-a spinal vertebra
  • Characteristically affects young adults
  • Peak onset between 20-30 yrs
  • Male:female - approximately 3:1*
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15
Q

Ankylosing Spondylitis

  • Greek ‘ankylos‘- bent/crooked (fused) - ‘spondylos‘-a spinal vertebra
  • Characteristically affects young adults
  • Peak onset between 20-30 yrs
  • Male:female - approximately ..-..*
A
  • Greek ‘ankylos‘- bent/crooked (fused) - ‘spondylos‘-a spinal vertebra
  • Characteristically affects young adults
  • Peak onset between 20-30 yrs
  • Male:female - approximately 3:1*
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16
Q

Examining tenderness over sacroiliac joint

A
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17
Q

… test is classically used to determine if there is a decrease in lumbar spine range of motion (flexion), most commonly as a result of ankylosing spondylitis.

A

Schober’s test is classically used to determine if there is a decrease in lumbar spine range of motion (flexion), most commonly as a result of ankylosing spondylitis.

For both versions of the test, an increase of less than 5cm is a positive test and may indicate ankylosing spondylitis (AS).

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18
Q

Positive Schober’s Test

A

Less than 5cm increase in length with forward flexion: Decreased lumbar spine range of motion, ankylosing spondylitis

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19
Q

The … to Wall Distance is a routine clinical test for thoracic kyphosis that has been in use for many years

A

The Occiput to Wall Distance is a routine clinical test for thoracic kyphosis that has been in use for many years

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20
Q

Ankylosis of spine

A

on right

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21
Q

Ankylosed sacroiliac joints

A
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22
Q

Ankylosing Spondylitis - Clinical features

  • Low back pain - Often the first symptom (in ~ …%).
  • … pain - indicative of SI involvement. Often poorly localised and may be referred to the posterior thigh.
  • Limited spinal …
  • Hip and Shoulder pain- … arthritis
  • Peripheral arthritis -Acute monoarthritis or oligoarthritis Mostly non-erosive and non-destructive.
  • Enthesitis.
  • Constitutional features- fatigue and malaise. Disturbed sleep, caused by back or joint pain at night, may contribute to fatigue
A
  • Low back pain - Often the first symptom (in ~ 75%).
  • Buttock pain - indicative of SI involvement. Often poorly localised and may be referred to the posterior thigh.
  • Limited spinal mobility
  • Hip and Shoulder pain- girdle arthritis
  • Peripheral arthritis -Acute monoarthritis or oligoarthritis Mostly non-erosive and non-destructive.
  • Enthesitis.
  • Constitutional features- fatigue and malaise. Disturbed sleep, caused by back or joint pain at night, may contribute to fatigue
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23
Q

Ankylosing Spondylitis - Clinical features

  • … … pain - Often the first symptom (in ~ 75%).
  • Buttock pain - indicative of SI involvement. Often poorly localised and may be referred to the posterior …
  • Limited spinal mobility
  • … and … pain- girdle arthritis
  • Peripheral arthritis -Acute monoarthritis or oligoarthritis Mostly non-erosive and non-destructive.
  • Enthesitis.
  • Constitutional features- fatigue and malaise. Disturbed sleep, caused by back or joint pain at night, may contribute to fatigue
A
  • Low back pain - Often the first symptom (in ~ 75%).
  • Buttock pain - indicative of SI involvement. Often poorly localised and may be referred to the posterior thigh.
  • Limited spinal mobility
  • Hip and Shoulder pain- girdle arthritis
  • Peripheral arthritis -Acute monoarthritis or oligoarthritis Mostly non-erosive and non-destructive.
  • Enthesitis.
  • Constitutional features- fatigue and malaise. Disturbed sleep, caused by back or joint pain at night, may contribute to fatigue
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24
Q

Ankylosing Spondylitis - Clinical features

  • Low back pain - Often the first symptom (in ~ 75%).
  • Buttock pain - indicative of SI involvement. Often poorly localised and may be referred to the posterior thigh.
  • Limited … mobility
  • Hip and Shoulder pain- girdle arthritis
  • Peripheral arthritis -Acute monoarthritis or oligoarthritis Mostly non-erosive and non-destructive.
  • E…
  • Constitutional features- … and …. Disturbed …, caused by back or joint pain at …, may contribute to …
A
  • Low back pain - Often the first symptom (in ~ 75%).
  • Buttock pain - indicative of SI involvement. Often poorly localised and may be referred to the posterior thigh.
  • Limited spinal mobility
  • Hip and Shoulder pain- girdle arthritis
  • Peripheral arthritis -Acute monoarthritis or oligoarthritis Mostly non-erosive and non-destructive.
  • Enthesitis.
  • Constitutional features- fatigue and malaise. Disturbed sleep, caused by back or joint pain at night, may contribute to fatigue
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25
Q

Ankylosing Spondylitis - Clinical features

  • Low back pain - Often the first symptom (in ~ ….%).
  • Buttock pain - indicative of SI involvement. Often poorly localised and may be referred to the … …
  • Limited spinal mobility
  • Hip and Shoulder pain- girdle arthritis
  • Peripheral … -Acute … or … Mostly non-erosive and non-destructive.
  • Enthesitis.
  • Constitutional features- fatigue and malaise. Disturbed sleep, caused by back or joint pain at night, may contribute to fatigue
A
  • Low back pain - Often the first symptom (in ~ 75%).
  • Buttock pain - indicative of SI involvement. Often poorly localised and may be referred to the posterior thigh.
  • Limited spinal mobility
  • Hip and Shoulder pain- girdle arthritis
  • Peripheral arthritis -Acute monoarthritis or oligoarthritis Mostly non-erosive and non-destructive.
  • Enthesitis.
  • Constitutional features- fatigue and malaise. Disturbed sleep, caused by back or joint pain at night, may contribute to fatigue
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26
Q

Other organ involvement - Ankylosing Spondylitis

  • Anterior … (iritis) – 40%
  • … tendonitis (enthesitis)
  • Aortic … (4%)
  • Apical lung … (1.5%)
  • A-V conduction defects (rare)
  • Amyloidosis (rare)
A
  • Anterior uveitis (iritis) – 40%
  • Achillies tendonitis (enthesitis)
  • Aortic regurgitation (4%)
  • Apical lung fibrosis (1.5%)
  • A-V conduction defects (rare)
  • Amyloidosis (rare)
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27
Q

Other organ involvement - Ankylosing Spondylitis

  • … uveitis (iritis) – 40%
  • Achillies … (enthesitis)
  • … regurgitation (4%)
  • Apical lung fibrosis (1.5%)
  • A-V … defects (rare)
  • …dosis (rare)
A
  • Anterior uveitis (iritis) – 40%
  • Achillies tendonitis (enthesitis)
  • Aortic regurgitation (4%)
  • Apical lung fibrosis (1.5%)
  • A-V conduction defects (rare)
  • Amyloidosis (rare)
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28
Q

Association of HLA-B27 and Spondyloarthropathies

A

general healthy can be positive for HLA-B27 - note

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29
Q

How is it diagnosed? - Ankylosing Spondylitis

  • History
  • Clinical examination
  • Blood test (not diagnostic): ESR, …, Anaemia, HLA …
  • Radiological:
    • …- pelvis (SI joints and hips) and spine
    • …- more sensitive and picks up changes early
  • GOALS OF THERAPY:
    • Symptomatic relief -Eliminate/reduce pain and stiffness.
    • Restore/preserve function
    • Prevent … damage
    • Prevent … … - to maintain spinal mobility, and prevent development of spinal deformities
    • Minimise extra-spinal and extra-articular manifestation
A
  • History
  • Clinical examination
  • Blood test (not diagnostic): ESR, CRP, Anaemia, HLA B27
  • Radiological:
    • X-rays- pelvis (SI joints and hips) and spine
    • MRI- more sensitive and picks up changes early
  • GOALS OF THERAPY:
    • Symptomatic relief -Eliminate/reduce pain and stiffness.
    • Restore/preserve function
    • Prevent joint damage
    • Prevent spinal fusion - to maintain spinal mobility, and prevent development of spinal deformities
    • Minimise extra-spinal and extra-articular manifestation
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30
Q

How is it diagnosed? - Ankylosing Spondylitis

  • Clinical …
  • Blood test (not diagnostic): …, CRP, …, HLA B27
  • Radiological:
    • X-rays- pelvis (SI joints and hips) and spine
    • MRI- more sensitive and picks up changes early
  • GOALS OF THERAPY:
    • Symptomatic relief -Eliminate/reduce pain and …
    • Restore/preserve …
    • Prevent joint damage
    • Prevent spinal fusion - to maintain spinal …, and prevent development of spinal …
    • Minimise extra-spinal and extra-articular manifestation
A
  • History
  • Clinical examination
  • Blood test (not diagnostic): ESR, CRP, Anaemia, HLA B27
  • Radiological:
    • X-rays- pelvis (SI joints and hips) and spine
    • MRI- more sensitive and picks up changes early
  • GOALS OF THERAPY:
    • Symptomatic relief -Eliminate/reduce pain and stiffness.
    • Restore/preserve function
    • Prevent joint damage
    • Prevent spinal fusion - to maintain spinal mobility, and prevent development of spinal deformities
    • Minimise extra-spinal and extra-articular manifestation
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31
Q

Treatment - Ankylosing Spondylitis

  • … & …
  • Pain relief: simple analgesics & Anti-inflammatory (NSAIDs)
  • … (if peripheral joints affected)
  • Steroids-oral/intra-muscular/intra-venous
  • Anti-… agents
  • Surgery e.g. hip replacement
  • …-Osteoporosis prophylaxis
  • Osteopathy, Chiropractic (any form of spinal manipulation) is not recommended.
A
  • Physiotherapy & exercise
  • Pain relief: simple analgesics & Anti-inflammatory (NSAIDs)
  • DMARDs (if peripheral joints affected)
  • Steroids-oral/intra-muscular/intra-venous
  • Anti-TNF agents
  • Surgery e.g. hip replacement
  • DEXA-Osteoporosis prophylaxis
  • Osteopathy, Chiropractic (any form of spinal manipulation) is not recommended.
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32
Q

Treatment - Ankylosing Spondylitis

  • Physiotherapy & exercise
  • Pain relief: simple analgesics & Anti-inflammatory (…)
  • DMARDs (if peripheral joints affected)
  • …-oral/intra-muscular/intra-venous
  • Anti-TNF agents
  • Surgery e.g. … replacement
  • DEXA-Osteoporosis …
  • Osteopathy, Chiropractic (any form of spinal manipulation) is not recommended.
A
  • Physiotherapy & exercise
  • Pain relief: simple analgesics & Anti-inflammatory (NSAIDs)
  • DMARDs (if peripheral joints affected)
  • Steroids-oral/intra-muscular/intra-venous
  • Anti-TNF agents
  • Surgery e.g. hip replacement
  • DEXA-Osteoporosis prophylaxis
  • Osteopathy, Chiropractic (any form of spinal manipulation) is not recommended.
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33
Q

NICE Guidance on use of Anti-TNF alpha agents in AS

  • The patient’s disease satisfies the modified New York criteria for diagnosis of Ankylosing Spondylitis.
    • A score of ≥… units on the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI)
      • and
    • ≥… cm on the 0 to 10 cm spinal pain visual analogue scale (VAS).
  • Conventional treatment with two or more non-steroidal anti-inflammatory drugs taken sequentially at maximum tolerated or recommended dosage for 4 weeks has failed to control symptoms.
A
  • The patient’s disease satisfies the modified New York criteria for diagnosis of Ankylosing Spondylitis.•
    • A score of ≥4 units on the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI)
      • and
    • 4 cm on the 0 to 10 cm spinal pain visual analogue scale (VAS).
  • Conventional treatment with two or more non-steroidal anti-inflammatory drugs taken sequentially at maximum tolerated or recommended dosage for 4 weeks has failed to control symptoms.
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34
Q

NICE Guidance on use of Anti-TNF alpha agents in AS

  • The patient’s disease satisfies the modified New York criteria for diagnosis of Ankylosing Spondylitis.•
    • A score of ≥4 units on the … Ankylosing Spondylitis Disease Activity Index (BASDAI)
      • and
    • ≥4 cm on the 0 to 10 cm spinal pain … … scale (VAS).
  • Conventional treatment with … or more non-steroidal anti-inflammatory drugs taken sequentially at maximum tolerated or recommended dosage for … weeks has failed to control symptoms.
A
  • The patient’s disease satisfies the modified New York criteria for diagnosis of Ankylosing Spondylitis.•
    • A score of ≥4 units on the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI)
      • and
    • ≥4 cm on the 0 to 10 cm spinal pain visual analogue scale (VAS).
  • Conventional treatment with two or more non-steroidal anti-inflammatory drugs taken sequentially at maximum tolerated or recommended dosage for 4 weeks has failed to control symptoms.
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35
Q

Reactive Arthritis Triad

  • Common organisms:
  • Chlamydia / Gonorrhoea, Shigella / Salmonella / Yersinia / Campylobacter / C.Difficile, Flu
  • Cant see, cant pee, cant climb a tree…
A
  • Common organisms:
  • Chlamydia / Gonorrhoea, Shigella / Salmonella / Yersinia / Campylobacter / C.Difficile, Flu
  • Conjunctivitis: discharge, erythema, burning, photophobia
  • Urethritis: dysuria, urgency, frequency, discharge
  • Arthritis: Knees, ankles, feet
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36
Q

Reactive Arthritis Triad

  • Common organisms:
  • … / Gonorrhoea, Shigella / Salmonella / Yersinia / Campylobacter / C.Difficile, Flu
  • : discharge, erythema, burning, photophobia
  • : dysuria, urgency, frequency, discharge
  • : Knees, ankles, feet
A
  • Common organisms:
  • Chlamydia / Gonorrhoea, Shigella / Salmonella / Yersinia / Campylobacter / C.Difficile, Flu
  • Conjunctivitis: discharge, erythema, burning, photophobia
  • Urethritis: dysuria, urgency, frequency, discharge
  • Arthritis: Knees, ankles, feetul
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37
Q

Most common organism causing reactive arthritis

A

Chlamydia (but can be Gonorrhoea, Shigella / Salmonella / Yersinia / Campylobacter / C.Difficile, Flu)

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38
Q

triad for…

A
  • reactive arthritis:
    • Conjunctivitis: discharge, erythema, burning, photophobia
    • Urethritis: dysuria, urgency, frequency, discharge
    • Arthritis: Knees, ankles, feet
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39
Q

Reactive arthritis images

A
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40
Q

Investigations for Reactive Arthritis

  • ESR, …-… protein, FBC, Liver and Renal function, RF
  • … analysis, … of affected joints
  • … examination if … symptoms present
  • Joint fluid Aspiration-Cell count, crystals, Gram stain & culture
  • Bacterial culture of:
    • Feces, Urine or urethral swab, Cervical sample, Throat
    • … culture (not always necessary)
  • Treatment
    • Rest
    • … if infection is still present
    • NSAIDs
    • Intra-articular/Systemic corticosteroids
    • Disease modifying anti-rheumatic drugs in some cases
A
  • ESR, C-Reactive protein, FBC, Liver and Renal function, RF
  • Urine analysis, Radiograph of affected joints
  • Ophthalmologic examination if eye symptoms present
  • Joint fluid Aspiration-Cell count, crystals, Gram stain & culture
  • Bacterial culture of:
    • Feces, Urine or urethral swab, Cervical sample, Throat
    • Blood culture (not always necessary)
  • Treatment
    • Rest
    • Antibiotics if infection is still present
    • NSAIDs
    • Intra-articular/Systemic corticosteroids
    • Disease modifying anti-rheumatic drugs in some cases
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41
Q

Investigations for Reactive Arthritis

  • …, C-Reactive protein, FBC, Liver and Renal function, RF
  • Urine analysis, Radiograph of affected joints
  • Ophthalmologic examination if eye symptoms present
  • Joint fluid …-Cell count, crystals, Gram stain & culture
  • Bacterial culture of:
    • …, Urine or … swab, Cervical sample, Throat
    • Blood culture (not always necessary)
  • Treatment
    • R…
    • Antibiotics if infection is still present
    • N..
    • Intra-articular/Systemic …
    • Disease modifying .. … drugs in some cases
A
  • ESR, C-Reactive protein, FBC, Liver and Renal function, RF
  • Urine analysis, Radiograph of affected joints
  • Ophthalmologic examination if eye symptoms present
  • Joint fluid Aspiration-Cell count, crystals, Gram stain & culture
  • Bacterial culture of:
    • Feces, Urine or urethral swab, Cervical sample, Throat
    • Blood culture (not always necessary)
  • Treatment
    • Rest
    • Antibiotics if infection is still present
    • NSAIDs
    • Intra-articular/Systemic corticosteroids
    • Disease modifying anti-rheumatic drugs in some cases
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42
Q

Psoriatic Arthritis

  • Arthritis may precede the onset of psoriasis / can also be … degree relative with …
  • Prevalence 0.04%-0.1%
  • … … is equal
  • … changes have the strongest association with arthritis
  • Spondyloarthropathy seen in about 5%
  • Sacroiliac joint involvement can be symmetric or asymmetric
A
  • Arthritis may precede the onset of psoriasis / can also be first degree relative with psoriasis
  • Prevalence 0.04%-0.1%
  • Sex distribution is equal
  • Nail changes have the strongest association with arthritis
  • Spondyloarthropathy seen in about 5%
  • Sacroiliac joint involvement can be symmetric or asymmetric
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43
Q

Psoriatic Arthritis

  • Arthritis may … the onset of psoriasis / can also be first degree relative with psoriasis
  • Prevalence …%
  • Sex distribution is equal
  • Nail changes have the strongest association with arthritis
  • Spondyloarthropathy seen in about …%
  • … joint involvement can be symmetric or asymmetric
A
  • Arthritis may precede the onset of psoriasis / can also be first degree relative with psoriasis
  • Prevalence 0.04%-0.1%
  • Sex distribution is equal
  • Nail changes have the strongest association with arthritis
  • Spondyloarthropathy seen in about 5%
  • Sacroiliac joint involvement can be symmetric or asymmetric
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44
Q

THE MOLL AND WRIGHT CLASSIFICATION OF PSORIATIC ARTHRITIS

  • Arthritis with … joint involvement predominant
  • Arthritis M…
  • Symmetric polyarthritis – indistinguishable from RA
  • Asymmetric oligoarticular arthritis
  • … spondylitis
A
  • Arthritis with DIP joint involvement predominant
  • Arthritis mutilans
  • Symmetric polyarthritis – indistinguishable from RA
  • Asymmetric oligoarticular arthritis
  • Predominant spondylitis
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45
Q

THE MOLL AND WRIGHT CLASSIFICATION OF PSORIATIC ARTHRITIS

  • Arthritis with DIP joint involvement predominant
  • Arthritis mutilans
  • … polyarthritis – indistinguishable from RA
  • … oligoarticular arthritis
  • Predominant spondylitis
A
  • Arthritis with DIP joint involvement predominant
  • Arthritis mutilans
  • Symmetric polyarthritis – indistinguishable from RA
  • Asymmetric oligoarticular arthritis
  • Predominant spondylitis
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46
Q

Symmetric polyarthritis – indistinguishable from …

A

Symmetric polyarthritis – indistinguishable from RA

47
Q

X-ray shows what?

A

Classic ‘pencil in cup’ erosive changes - Psoriatic Arthritis

48
Q

Anti-TNF in Psoriatic Arthritis

A
49
Q

Seronegative Spondyloarthropathy Summary

A
50
Q

Which joints are diseased?

A

PIPs / DIPs

51
Q

Name the radiological appearance seen on the x-ray

A

Pencil in cup - psoriatic arthritis

52
Q

Name the pathology

A

Dactylitis

53
Q

What is vasculitis?

A

Vasculitis means inflammation of the blood vessels.

54
Q

Vasculitis Presentations

A
55
Q

Vasculitis Classification

A
56
Q

Vasculitis - Classification tree

A
57
Q

Vasculitis Case 1:

  • A 64 year old man complains of general malaise and has lost 6kg in weight over six months
  • Haemoptysis 9 days ago
  • Purpuric rash on his legs
  • BP is 165/90.
  • Dipstick urinalysis showed +++blood and ++protein.
  • Creatinine is 320, Hb 95 and platelets 210.
  • Serology: ANCA, Anti GBM abs, Anti phospholipid screen, ANA/ENA, dsDNA / Complement, Anti streptococcal abs, RF / Anti CCP
  • If the ANCA is +, what could he have?
A
  • A 64 year old man complains of general malaise and has lost 6kg in weight over six months
  • Haemoptysis 9 days ago
  • Purpuric rash on his legs
  • BP is 165/90.
  • Dipstick urinalysis showed +++blood and ++protein.
  • Creatinine is 320, Hb 95 and platelets 210.
  • Serology: ANCA, Anti GBM abs, Anti phospholipid screen, ANA/ENA, dsDNA / Complement, Anti streptococcal abs, RF / Anti CCP
  • If the ANCA is +, what could he have?
    • Most likely to be GPA

BUT screen for -

  • Infections: Hep B/C, N. Meningitidis
  • Drug: Cocaine, Amphetamines
  • Medications: NSAIDs, Abx, Thiazide Diuretics, Warfarin
  • Malignancy: Lymphomas, Myeloma
  • Inflammatory: RA / SLE
58
Q

Vasculitis is classified into 3 groups, which are…

A

Small, medium and large

59
Q

Types of Vasculitis (by size of arteries affected)

  • The vasculitic diseases are usually classified according to the predominant size of vessel involved.
  • Vasculitis mainly affecting … arteries:
    • Giant Cell Arteritis/Temporal Arteritis (GCA)
    • Takayasu Arteritis (TA)
  • Vasculitis mainly affecting … … arteries:
    • Polyarteritis Nodosa (PAN)
    • Kawasaki Disease (KD
  • Diseases affecting the … blood vessels - These are divided into … associated vasculitis and non-… vasculitis.
    • … associated vasculitis
      • Eosinophillic Granulomatosis with Polyangiitis
      • Microscopic Polyangiitis (MPA)
      • Drug induced
      • Wegener’s Granulomatosis (WG)
    • Non-… vasculitis
      • Henoch-Schönlein Purpura (HSP)
      • Behcet’s
      • CTD related vasculitis
      • Cyroglobulinemic vasculitis
      • Hypersensitivity vasculitis
A
  • The vasculitic diseases are usually classified according to the predominant size of vessel involved.
  • Vasculitis mainly affecting large arteries:
    • Giant Cell Arteritis/Temporal Arteritis (GCA)
    • Takayasu Arteritis (TA)
  • Vasculitis mainly affecting middle sized arteries:
    • Polyarteritis Nodosa (PAN)
    • Kawasaki Disease (KD
  • Diseases affecting the small blood vessels - These are divided into ANCA associated vasculitis and non-ANCA vasculitis.
    • ANCA associated vasculitis
      • Eosinophillic Granulomatosis with Polyangiitis
      • Microscopic Polyangiitis (MPA)
      • Drug induced
      • Wegener’s Granulomatosis (WG)
    • Non-ANCA vasculitis
      • Henoch-Schönlein Purpura (HSP)
      • Behcet’s
      • CTD related vasculitis
      • Cyroglobulinemic vasculitis
      • Hypersensitivity vasculitis
60
Q

Examination Features - GCA

  • Abnormal … Temporal Artery
    • Tender
    • Thickened
    • Reduced/ Absent …
  • … Tenderness
  • Cranial Nerve …
  • Vascular …
  • … Pulses
A
  • Abnormal Superficial Temporal Artery
    • Tender
    • Thickened
    • Reduced/ Absent Pulsation
  • Scalp Tenderness
  • Cranial Nerve Palsies
  • Vascular Bruits
  • Asymmetric Pulses
61
Q

Vasculitis Mimickers:

  • Infections: Hep B/C, N. Meningitidis
  • Drug: …, …
  • Medications: NSAIDs, Abx, Thiazide Diuretics, Warfarin
  • Malignancy: Lymphomas, …
  • Inflammatory: … / SLE
A
  • Infections: Hep B/C, N. Meningitidis
  • Drug: Cocaine, Amphetamines
  • Medications: NSAIDs, Abx, Thiazide Diuretics, Warfarin
  • Malignancy: Lymphomas, Myeloma
  • Inflammatory: RA / SLE
62
Q

Vasculitis Mimickers:

  • Infections: Hep B/C, N. …
  • Drug: Cocaine, Amphetamines
  • Medications: …, Abx, … Diuretics, …
  • Malignancy: …, Myeloma
  • Inflammatory: RA / …
A
  • Infections: Hep B/C, N. Meningitidis
  • Drug: Cocaine, Amphetamines
  • Medications: NSAIDs, Abx, Thiazide Diuretics, Warfarin
  • Malignancy: Lymphomas, Myeloma
  • Inflammatory: RA / SLE
63
Q

ANCA positive Vasculitis Treatment

A
64
Q

Vasculitis Systems Review

  • Systemic symptoms
  • Cutaneous (… / ulcers)
  • Mucus membranes (ulcers / eyes)
  • ENT (nasal … / … loss / …)
  • Bowels (PR bleeding / abdo pain / perforation)
  • Respiratory (wheeze / haemoptysis / fibrosis)
  • Cardiovascular (pulses / percarditis / CP)
  • Renal (… BP / renal … / …uria)
  • Neuro (… / seizures / …)
A
  • Systemic symptoms
  • Cutaneous (purpura / ulcers)
  • Mucus membranes (ulcers / eyes)
  • ENT (nasal crusting / hearing loss / sinusitis)
  • Bowels (PR bleeding / abdo pain / perforation)
  • Respiratory (wheeze / haemoptysis / fibrosis)
  • Cardiovascular (pulses / percarditis / CP)
  • Renal (High BP / renal failure / proteinuria)
  • Neuro (stroke / seizures / neuropathy)
65
Q

Vasculitis Systems Review

  • Systemic symptoms
  • Cutaneous (purpura / …)
  • Mucus membranes (… / eyes)
  • ENT (nasal crusting / hearing loss / sinusitis)
  • Bowels (… bleeding / … pain / perforation)
  • Respiratory (… / haemoptysis / …)
  • Cardiovascular (pulses / … / CP)
  • Renal (High BP / renal failure / proteinuria)
  • Neuro (stroke / … / neuropathy)
A
  • Systemic symptoms
  • Cutaneous (purpura / ulcers)
  • Mucus membranes (ulcers / eyes)
  • ENT (nasal crusting / hearing loss / sinusitis)
  • Bowels (PR bleeding / abdo pain / perforation)
  • Respiratory (wheeze / haemoptysis / fibrosis)
  • Cardiovascular (pulses / percarditis / CP)
  • Renal (High BP / renal failure / proteinuria)
  • Neuro (stroke / seizures / neuropathy)
66
Q

In general, flares of systemic vasculitides require escalation of immunosuppressive treatment:

  • …. (3 days IV Methylpred 1g)
  • Cyclophosphamide / R…
  • Azathioprine / M…
A
  • Corticosteroids (3 days IV Methylpred 1g)
  • Cyclophosphamide / Rituximab
  • Azathioprine / Methotrexate
67
Q

In general, flares of systemic vasculitides require escalation of immunosuppressive treatment:

  • Corticosteroids (… days IV Methylpred 1g)
  • C… / Rituximab
  • A… / Methotrexate
A
  • Corticosteroids (3 days IV Methylpred 1g)
  • Cyclophosphamide / Rituximab
  • Azathioprine / Methotrexate
68
Q

Case 2 - Vasculitis

  • 55 year old lady
  • Proximal myalgia
  • Complaining of pain and difficulty turning over in bed and combing her hair
  • ESR 60
  • CRP 45
  • Diagnosis?
  • What other diagnosis is associated with this?
    • … cell …
A
  • 55 year old lady
  • Proximal myalgia
  • Complaining of pain and difficulty turning over in bed and combing her hair
  • ESR 60
  • CRP 45
  • What other diagnosis is associated with Polymyalgia Rheumatica?
    • Giant cell arteritis
69
Q

Polymyalgia Rheumatica (PMR) & Giant Cell Arteritis (GCA)

  • Age > …
  • Scalp tenderness and headache (temporal and unilateral)
  • Jaw or limb …
  • Blindness or …
  • Weight loss, anorexia, fever, night sweats, malaise
  • Raised … and …
  • Biopsy or …
  • … muscle pain and stiffness
A
  • Age > 50
  • Scalp tenderness and headache (temporal and unilateral)
  • Jaw or limb claudication
  • Blindness or diplopia
  • Weight loss, anorexia, fever, night sweats, malaise
  • Raised ESR and CRP
  • Biopsy or USS
  • Proximal muscle pain and stiffness
70
Q

Polymyalgia Rheumatica (PMR) & Giant Cell Arteritis (GCA)

  • Age > 50
  • … tenderness and … (temporal and unilateral)
  • Jaw or limb claudication
  • … or diplopia
  • Weight …, …, fever, … sweats, …
  • Raised ESR and CRP
  • … or USS
  • Proximal … pain and …
A
  • Age > 50
  • Scalp tenderness and headache (temporal and unilateral)
  • Jaw or limb claudication
  • Blindness or diplopia
  • Weight loss, anorexia, fever, night sweats, malaise
  • Raised ESR and CRP
  • Biopsy or USS
  • Proximal muscle pain and stiffness
71
Q

Examination Features - GCA

  • Abnormal Superficial Temporal Artery
    • …/ … Pulsation
  • Scalp …
  • … Nerve Palsies
  • … Bruits
  • Asymmetric …
A
  • Abnormal Superficial Temporal Artery
    • Tender
    • Thickened
    • Reduced/ Absent Pulsation
  • Scalp Tenderness
  • Cranial Nerve Palsies
  • Vascular Bruits
  • Asymmetric Pulses
72
Q

Visual Loss - GCA

  • … Defect
  • Afferent … Defect
  • Anterior Ischaemic Optic Neuritis
  • Central Retinal Artery …

    • Early … Review
A
  • Field Defect
  • Afferent Pupillary Defect
  • Anterior Ischaemic Optic Neuritis
  • Central Retinal Artery Occlusion
  • Diplopia
    • Early Opthalmology Review
73
Q

Giant cell arteritis - May cause abnormal findings on fundoscopy:

  • Central retinal artery occlusion may cause a …-spot
  • Anterior ischaemic optic neuropathy may cause a … disc … (caused by inflammatory occlusion of posterior ciliary arteries and subsequent ischaemia of optic nerve)
A
  • Central retinal artery occlusion may cause a cherry-spot
  • Anterior ischaemic optic neuropathy may cause a Pallid disc oedema (caused by inflammatory occlusion of posterior ciliary arteries and subsequent ischaemia of optic nerve)
74
Q

Giant cell arteritis - May cause abnormal findings on fundoscopy:

  • …. … artery occlusion may cause a cherry-spot
  • … … optic neuropathy may cause a Pallid disc oedema (caused by inflammatory occlusion of posterior ciliary arteries and subsequent ischaemia of optic nerve)
A
  • Central retinal artery occlusion may cause a cherry-spot
  • Anterior ischaemic optic neuropathy may cause a Pallid disc oedema (caused by inflammatory occlusion of posterior ciliary arteries and subsequent ischaemia of optic nerve)
75
Q

GCA Investigations…

A
  • FBC, U&Es, LFTs, CRP, ESR.
  • CXR
  • Urinalysis
  • Temporal Artery biopsy
  • USS Temporal Arteries
76
Q

GCA Investigations…

  • …, U&Es, LFTs, CRP, ESR.
  • CXR
  • U..
  • Temporal Artery B…
  • … of Temporal Arteries
A
  • FBC, U&Es, LFTs, CRP, ESR.
  • CXR
  • Urinalysis
  • Temporal Artery biopsy
  • USS Temporal Arteries
77
Q

Biopsy and USS - GCA

  • … … biopsy (TAB) should be considered whenever a diagnosis of GCA is suspected.
  • This should not delay … therapy
  • TAB may be … in some patients (Skip lesions)
  • Imaging techniques show promise for the diagnosis and monitoring of GCA. … sensitivity.
  • These do not replace TAB for cranial GCA.
  • … limited as it requires a high level of experience and training.
  • PET and MRI currently reserved for investigation of suspected …-vessel GCA.
A
  • Temporal artery biopsy (TAB) should be considered whenever a diagnosis of GCA is suspected.
  • This should not delay glucocorticosteroid therapy
  • TAB may be negative in some patients (Skip lesions)
  • Imaging techniques show promise for the diagnosis and monitoring of GCA. High sensitivity.
  • These do not replace TAB for cranial GCA.
  • USS limited as it requires a high level of experience and training.
  • PET and MRI currently reserved for investigation of suspected large-vessel GCA.
78
Q

Biopsy and USS - GCA

  • Temporal artery biopsy (TAB) should be considered whenever a diagnosis of GCA is suspected.
  • This should not delay glucocorticosteroid therapy
  • TAB may be negative in some patients (… lesions)
  • … techniques show promise for the diagnosis and monitoring of GCA. High sensitivity.
  • These do not replace TAB for cranial GCA.
  • USS limited as it requires a … level of experience and training.
  • PET and … currently reserved for investigation of suspected large-vessel GCA.
A
  • Temporal artery biopsy (TAB) should be considered whenever a diagnosis of GCA is suspected.
  • This should not delay glucocorticosteroid therapy
  • TAB may be negative in some patients (Skip lesions)
  • Imaging techniques show promise for the diagnosis and monitoring of GCA. High sensitivity.
  • These do not replace TAB for cranial GCA.
  • USS limited as it requires a high level of experience and training.
  • PET and MRI currently reserved for investigation of suspected large-vessel GCA.
79
Q

This is a Temporal Biopsy sample showing ….

A

Immune cell infiltration

80
Q

Treatment - GCA

  • … – immediately (tapering regimen)
    • Uncomplicated GCA (no jaw claudication or visual disturbance)
    • 40mg … daily.
  • Evolving visual loss or amaurosis fugax (complicated GCA):
    • 500 mg to 1 g of i.v. … for 3 days before oral ….
  • Established visual loss
    • 60 mg … daily to protect the contralateral eye.
  • Also additional management
A
  • Steroids – immediately (tapering regimen)
    • Uncomplicated GCA (no jaw claudication or visual disturbance)
    • 40mg prednisolone daily.
  • Evolving visual loss or amaurosis fugax (complicated GCA):
    • 500 mg to 1 g of i.v. methylprednisolone for 3 days before oral glucocorticosteroids.
  • Established visual loss
    • 60 mg prednisolone daily to protect the contralateral eye.
81
Q

Treatment - GCA

  • Steroids – immediately (tapering regimen)
    • Uncomplicated GCA (no jaw … or … disturbance)
    • …mg prednisolone daily.
  • Evolving visual loss or amaurosis fugax (complicated GCA):
    • 500 mg to 1 g of i.v. methylprednisolone for 3 days before oral glucocorticosteroids.
  • Established visual loss
    • …. mg prednisolone daily to protect the … eye.
  • Also additional management
A
  • Steroids – immediately (tapering regimen)
    • Uncomplicated GCA (no jaw claudication or visual disturbance)
    • 40mg prednisolone daily.
  • Evolving visual loss or amaurosis fugax (complicated GCA):
    • 500 mg to 1 g of i.v. methylprednisolone for 3 days before oral glucocorticosteroids.
  • Established visual loss
    • 60 mg prednisolone daily to protect the contralateral eye.
82
Q

Additional Management - GCA

  • … Protection
  • … … Inhibitor (PPI)
  • Regular … Review
  • Relapse Management: steroids or steroid-sparing agent
A
  • Bone Protection
  • Proton Pump Inhibitor (PPI)
  • Regular Specialist Review
  • Relapse Management: steroids or steroid-sparing agent
83
Q

Additional Management - GCA

  • Bone …
  • Proton Pump …
  • Regular … Review
  • Relapse Management: … or …-sparing agent
A
  • Bone Protection
  • Proton Pump Inhibitor (PPI)
  • Regular Specialist Review
  • Relapse Management: steroids or steroid-sparing agent
84
Q

PMR diagnostic criteria (Polymyalgia rheumatica)

Also - PMR is associated with …: 1/50 with … get PMR but 1/10 PMR get ….

A
  • PMR is associated with GCA: 1/50 with GCA get PMR but 1/10 PMR get GCA.
85
Q

PMR diagnostic criteria (Polymyalgia rheumatica)

A
86
Q

Systemic lupus erythematosus (SLE)

  • Chronic, …, … disease
  • Broad spectrum of clinical features involving almost all organs and tissues
  • Prevalence in the UK: … per 100,000
  • …:… = 10-20:1 (GENDER)
  • Peak onset between 15- 40 years
  • More common and severe in those of Afro-Caribbean, India, Hispanic and Chinese origin living in USA and Europe> …
A
  • Chronic, relapsing, remitting disease
  • Broad spectrum of clinical features involving almost all organs and tissues
  • Prevalence in the UK: 97 per 100,000
  • F:M= 10-20:1
  • Peak onset between 15- 40 years
  • More common and severe in those of Afro-Caribbean, India, Hispanic and Chinese origin living in USA and Europe> Caucasians
87
Q

Systemic lupus erythematosus (SLE)

  • …, relapsing, remitting disease
  • Broad spectrum of clinical features involving almost all organs and tissues
  • Prevalence in the UK: 97 per 100,000
  • F:M= 10-20:1
  • Peak onset between …-… years
  • … common and … in those of Afro-Caribbean, India, Hispanic and Chinese origin living in USA and Europe> Caucasians
A
  • Chronic, relapsing, remitting disease
  • Broad spectrum of clinical features involving almost all organs and tissues
  • Prevalence in the UK: 97 per 100,000
  • F:M= 10-20:1
  • Peak onset between 15- 40 years
  • More common and severe in those of Afro-Caribbean, India, Hispanic and Chinese origin living in USA and Europe> Caucasians
88
Q

SLE Signs/Symptoms

  • … rash
  • Discoid rash
  • Serositis – Pleuritis / Pericarditis
  • … ulcers
  • Arthritis – non-erosive
  • Photosensitivity
  • … disorders
  • … disorders
  • ANA + anti Smith / dsDNA / antiphospholipid abs
  • … symptoms
A
  • Malar rash
  • Discoid rash
  • Serositis – Pleuritis / Pericarditis
  • Oral ulcers
  • Arthritis – non-erosive
  • Photosensitivity
  • Blood disorders
  • Renal disorders
  • ANA + anti Smith / dsDNA / antiphospholipid abs
  • Neurologic symptoms
89
Q

SLE Signs/Symptoms

  • Malar rash
  • … rash
  • Serositis – P… / P…
  • Oral ulcers
  • A… – non-erosive
  • …sensitivity
  • Blood disorders
  • Renal disorders
  • ANA + anti Smith / dsDNA / antiphospholipid abs
  • Neurologic symptoms
A
  • Malar rash
  • Discoid rash
  • Serositis – Pleuritis / Pericarditis
  • Oral ulcers
  • Arthritis – non-erosive
  • Photosensitivity
  • Blood disorders
  • Renal disorders
  • ANA + anti Smith / dsDNA / antiphospholipid abs
  • Neurologic symptoms
90
Q

SLE PATHOPHYSIOLOGY

A
91
Q

SLE PATHOPHYSIOLOGY

A
92
Q

SLE – 2019 classification criteria

  • …. positive ≥1:80
  • … clinical and ≥10 points (count highest in each criterion)
A
  • ANA positive ≥1:80
  • One clinical and ≥10 points (count highest in each criterion)
93
Q

SLE – 2019 classification criteria

  • ANA positive ≥…
  • One clinical and ≥… points (count highest in each criterion)
A
  • ANA positive ≥1:80
  • One clinical and ≥10 points (count highest in each criterion)
94
Q

SLE

A
95
Q

SLE Investigations

  • … – … protein: … ratio
  • Full blood count
  • … and electrolytes
  • ESR
  • … function test
  • Antibodies: ANA; ENA; Anti –dsDNA; Lupus anticoagulant; ANTI C1q;
  • C3, C…
A
  • Urinalysis – urinary protein: creatinine ratio
  • Full blood count
  • Urea and electrolytes
  • ESR
  • CRP
  • Liver function test
  • Antibodies: ANA; ENA; Anti –dsDNA; Lupus anticoagulant; ANTI C1q;
  • C3, C4
96
Q

SLE Investigations

  • Urinalysis – urinary protein: creatinine ratio
  • … blood count
  • Urea and …
  • CRP
  • Liver function test
  • Antibodies: ANA; ENA; Anti –dsDNA; Lupus anticoagulant; ANTI C1q;
  • C…, C…
A
  • Urinalysis – urinary protein: creatinine ratio
  • Full blood count
  • Urea and electrolytes
  • ESR
  • CRP
  • Liver function test
  • Antibodies: ANA; ENA; Anti –dsDNA; Lupus anticoagulant; ANTI C1q;
  • C3, C4 - if complement is low, disease is more active
97
Q

In SLE, if complement is low, disease is … active

A

In SLE, if complement is low, disease is more active (C3 and C4 - measure)

98
Q

SLE Principals of management

  • Nonpharmacological management (… protection; … cessation; … risk modification; … prevention)
A
  • Nonpharmacological management (sun protection; smoking cessation; CVD risk modification; osteoporosis prevention)
99
Q

Sjogren’s syndrome

  • Lymphocytic infiltration of … glands
  • S…: Dry eyes (lacrimal gland involvement) and xerostomia (salivary gland involvement)
  • Extra-glandular disease: joint pain, skin disease and pulmonary disease
  • F…, myalgia
  • Complications:
    • Non-… Lymphoma
    • Chronic dry eyes -> … and … damage
    • Chronic dry mouth -> … caries
    • … heart block
A
  • Lymphocytic infiltration of exocrine glands
  • Sicca: Dry eyes (lacrimal gland involvement) and xerostomia (salivary gland involvement)
  • Extra-glandular disease: joint pain, skin disease and pulmonary disease
  • Fatigue, myalgia
  • Complications:
    • Non-Hodgkins Lymphoma
    • Chronic dry eyes -> corneal and conjunctival damage
    • Chronic dry mouth -> dental caries
    • Congenital heart block
100
Q

Sjogren’s syndrome

  • … infiltration of exocrine glands
  • Sicca: Dry … (lacrimal gland involvement) and xerostomia (… … involvement)
  • Extra-glandular disease: joint pain, skin disease and pulmonary disease
  • Fatigue, myalgia
  • Complications:
    • Non-Hodgkins …
    • Chronic … … -> corneal and conjunctival damage
    • Chronic … … -> dental caries
    • Congenital heart block
A
  • Lymphocytic infiltration of exocrine glands
  • Sicca: Dry eyes (lacrimal gland involvement) and xerostomia (salivary gland involvement)
  • Extra-glandular disease: joint pain, skin disease and pulmonary disease
  • Fatigue, myalgia
  • Complications:
    • Non-Hodgkins Lymphoma
    • Chronic dry eyes -> corneal and conjunctival damage
    • Chronic dry mouth -> dental caries
    • Congenital heart block
101
Q

Sjogren’s Syndrome Diagnosis

  • Need …/6 criteria including one of the red criterion
A
  • Need 4/6 criteria including one of the red criterion
102
Q

Sjogren’s Syndrome Diagnosis

  • Need 4/6 criteria including one of the BOLD criterion
    • Dry eyes >… months / using … … TDS:
    • Dry mouth >… months / need … to swallow dry foods / recurrent …. salivary glands
    • Shirmer’s test <5mm/5 mins / positive vital dye staining
    • Abnormal parotid sialography or salivary scintigraphy or unstimulated whole salivary flow (<1.5ml/15mins)
    • Focal lymphocytic sialoadenditis on lip biopsy
    • Anti-… / Anti … positive
A
  • Need 4/6 criteria including one of the BOLD criterion
    • Dry eyes >3 months / using artificial tears TDS:
    • Dry mouth >3 months / need liquids to swallow dry foods / recurrent swollen salivary glands
    • Shirmer’s test <5mm/5 mins / positive vital dye staining
    • Abnormal parotid sialography or salivary scintigraphy or unstimulated whole salivary flow (<1.5ml/15mins)
    • Focal lymphocytic sialoadenditis on lip biopsy
    • Anti-Ro / Anti La positive
103
Q

Sjogren’s Syndrome Diagnosis

  • Need …/6 criteria including one of the BOLD criterion
    • Dry … >3 months / using artificial tears TDS:
    • Dry … >3 months / need liquids to swallow dry foods / recurrent swollen salivary glands
    • … test <5mm/5 mins / positive vital dye staining
    • Abnormal parotid sialography or salivary scintigraphy or unstimulated whole salivary … (<1.5ml/15mins)
    • Focal lymphocytic sialoadenditis on lip biopsy
    • Anti-Ro / Anti La positive
A
  • Need 4/6 criteria including one of the BOLD/italic criterion
    • Dry eyes >3 months / using artificial tears TDS:
    • Dry mouth >3 months / need liquids to swallow dry foods / recurrent swollen salivary glands
    • Shirmer’s test <5mm/5 mins / positive vital dye staining
    • Abnormal parotid sialography or salivary scintigraphy or unstimulated whole salivary flow (<1.5ml/15mins)
    • Focal lymphocytic sialoadenditis on lip biopsy
    • Anti-Ro / Anti La positive
104
Q

Sjogren’s Syndrome Treatment

  • Conservative:
    • Stop … / … hygiene /
    • Artificial … and …
  • Medications:
    • Cholinergic agents: pilocarpine / cevimeline
    • … / Steroids
    • … eg. hydroxychloroquine / methotrexate / rituximab
A
  • Conservative:
    • Stop smoking / oral hygiene /
    • Artificial tears and saliva
  • Medications:
    • Cholinergic agents: pilocarpine / cevimeline
    • NSAIDs / Steroids
    • Immunosuppressants eg. hydroxychloroquine / methotrexate / rituximab
105
Q

Sjogren’s Syndrome Treatment

  • Conservative:
    • Stop smoking / oral hygiene /
    • … tears and saliva
  • Medications:
    • … agents: pilocarpine / cevimeline
    • NSAIDs / …
    • Immunosuppressants eg. hydroxychloroquine / … / …
A
  • Conservative:
    • Stop smoking / oral hygiene /
    • Artificial tears and saliva
  • Medications:
    • Cholinergic agents: pilocarpine / cevimeline
    • NSAIDs / Steroids
    • Immunosuppressants eg. hydroxychloroquine / methotrexate / rituximab
106
Q

Spot Diagnosis

  • … syndrome:
    • Reynaud’s (WHITE BLUE RED)
    • Telangeictasia of lips
    • Tight shiny skin on finger - scelrodactyly
    • Calcinosis
    • Oesophageal dismotility - reflux
A
  • Crest syndrome:
    • Reynaud’s (WHITE BLUE RED)
    • Telangeictasia of lips
    • Tight shiny skin on finger - scelrodactyly
    • Calcinosis
    • Oesophageal dismotility - reflux
107
Q

Systemic Sclerosis (Scleroderma)

  • … activation, vascular damage and excessive deposition of collagen
  • More frequent in ages ..-..
  • 5:1 …(gender)
  • Antibodies:
    • anti topoisomerase (anti-Scl-70)
    • Anti … (ACA)
    • anti-RNA polymerase III
    • ANA
A
  • Immune activation, vascular damage and excessive deposition of collagen
  • More frequent in ages 30-50
  • 5:1 female
  • Antibodies:
    • anti topoisomerase (anti-Scl-70)
    • Anti centromere (ACA)
    • anti-RNA polymerase III
    • ANA
108
Q

Systemic Sclerosis (Scleroderma)

  • Immune activation, vascular damage and excessive deposition of …
  • More frequent in ages 30-50
  • 5:1 …
  • Antibodies:
    • anti … (anti-Scl-70)
    • Anti centromere (ACA)
    • anti-… polymerase III
    • ANA
A
  • Immune activation, vascular damage and excessive deposition of collagen
  • More frequent in ages 30-50
  • 5:1 female
  • Antibodies:
    • anti topoisomerase (anti-Scl-70)
    • Anti centromere (ACA)
    • anti-RNA polymerase III
    • ANA
109
Q

Systemic Sclerosis Clinical Features

  • Sclerodactyly
  • … phenomenon
  • Heartburn
  • Abnormal nailfold …
  • …iectasia
  • Pulmonary arterial hypertension
  • … lung disease
  • Lower bowel: distension / diarrhea / constipation
  • Renal failure / proteinuria
  • … dysfunction
A
  • Sclerodactyly
  • Raynaud’s phenomenon
  • Heartburn
  • Abnormal nailfold capillaries
  • Telangiectasia
  • Pulmonary arterial hypertension
  • Interstitial lung disease
  • Lower bowel: distension / diarrhea / constipation
  • Renal failure / proteinuria
  • Erectile dysfunction
110
Q

Systemic Sclerosis Clinical Features

  • Sclerodactyly
  • Raynaud’s phenomenon
  • Abnormal … capillaries
  • Telangiectasia
  • Pulmonary arterial …
  • Interstitial lung disease
  • … bowel: distension / diarrhea / constipation
  • … failure / proteinuria
  • Erectile dysfunction
A
  • Sclerodactyly
  • Raynaud’s phenomenon
  • Heartburn
  • Abnormal nailfold capillaries
  • Telangiectasia
  • Pulmonary arterial hypertension
  • Interstitial lung disease
  • Lower bowel: distension / diarrhea / constipation
  • Renal failure / proteinuria
  • Erectile dysfunction
111
Q

Systemic Sclerosis Types

  • Limited = …. (calcinosis, Raynaud’s, Esophageal dysmotility, sclerodactyly, telangiectasias)
  • …= scleroderma proximal to elbows
  • Overlap syndromes with SLE / …
  • Sine …
A
  • Limited = CREST (calcinosis, Raynaud’s, Esophageal dysmotility, sclerodactyly, telangiectasias)
  • Diffuse= scleroderma proximal to elbows
  • Overlap syndromes with SLE / Sjogren’s
  • Sine scleroderma
112
Q

Systemic Sclerosis Treatment

  • Conservative
    • Stop …
    • Controlled …
    • Wear …
    • Warm … baths
  • Medications
    • Raynaud’s – amlodipine / losartan / fluoxetine / sildenafil
    • Reflux - …
    • …- mycophenolate, methotrexate, cyclophosphamide, steroids, rituximab, IV immunoglobulins
A
  • Conservative
    • Stop smoking
    • Controlled exercise
    • Wear gloves
    • Warm wax baths
  • Medications
    • Raynaud’s – amlodipine / losartan / fluoxetine / sildenafil
    • Reflux - omeprazole
    • Immunosuppressants- mycophenolate, methotrexate, cyclophosphamide, steroids, rituximab, IV immunoglobulins
113
Q

Systemic Sclerosis Treatment

  • Conservative
    • Stop smoking
    • Controlled exercise
    • Wear gloves
    • … … baths
  • Medications
    • …. – amlodipine / losartan / fluoxetine / sildenafil
    • … - omeprazole
    • Immunosuppressants- mycophenolate, methotrexate, cyclophosphamide, steroids, rituximab, IV immunoglobulins
A
  • Conservative
    • Stop smoking
    • Controlled exercise
    • Wear gloves
    • Warm wax baths
  • Medications
    • Raynaud’s – amlodipine / losartan / fluoxetine / sildenafil
    • Reflux - omeprazole
    • Immunosuppressants- mycophenolate, methotrexate, cyclophosphamide, steroids, rituximab, IV immunoglobulins