Multi-system Autoimmune Disease

Question 1

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

Answer 1

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

Question 2

Spondyloarthropathies types

• … spondylitis
• Psoriatic Arthropathy
• … Arthropathy
• Intestinal Arthropathy
• … Ankylosing Spondylitis
  
  • Hallmark ocular involvement is UVEITIS

Answer 2

• Ankylosing spondylitis
• Psoriatic Arthropathy
• Reactive Arthropathy
• Intestinal Arthropathy
• Juvenile Ankylosing Spondylitis
  
  • Hallmark ocular involvement is UVEITIS

Question 3

Spondyloarthropathies types

• Ankylosing spondylitis
• … Arthropathy
• Reactive Arthropathy
• … Arthropathy
• Juvenile Ankylosing Spondylitis
  
  • Hallmark ocular involvement is …

Answer 3

• Ankylosing spondylitis
• Psoriatic Arthropathy
• Reactive Arthropathy
• Intestinal Arthropathy
• Juvenile Ankylosing Spondylitis
  
  • Hallmark ocular involvement is UVEITIS

Question 4

Spondyloarthropathies types

• … spondylitis
• … Arthropathy
• … Arthropathy
• Intestinal Arthropathy
• Juvenile Ankylosing Spondylitis
  
  • Hallmark … involvement is UVEITIS

Answer 4

• Ankylosing spondylitis
• Psoriatic Arthropathy
• Reactive Arthropathy
• Intestinal Arthropathy
• Juvenile Ankylosing Spondylitis
  
  • Hallmark ocular involvement is UVEITIS

Question 5

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-…

Answer 5

• 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

Question 6

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

Answer 6

• 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

Question 7

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

Answer 7

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

Question 8

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

Answer 8

• 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

Question 9

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 …

Answer 9

• 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

Question 10

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 …

Answer 10

• 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

Question 11

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

Answer 11

• 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

Question 12

Imaging for Ankylosing Spondylitis diagnosis

Answer 12

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

Question 13

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

Answer 13

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

Question 14

Ankylosing Spondylitis

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

Answer 14

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

Question 15

Ankylosing Spondylitis

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

Answer 15

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

Question 16

Examining tenderness over sacroiliac joint

Answer 16

Question 17

… 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.

Answer 17

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).

Question 18

Positive Schober’s Test

Answer 18

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

Question 19

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

Answer 19

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

Question 20

Ankylosis of spine

Answer 20

on right

Question 21

Ankylosed sacroiliac joints

Answer 21

Question 22

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

Answer 22

• 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

Question 23

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

Answer 23

• 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

Question 24

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 …

Answer 24

• 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

Question 25

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

Answer 25

• 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

Question 26

Other organ involvement - Ankylosing Spondylitis

• Anterior … (iritis) – 40%
• … tendonitis (enthesitis)
• Aortic … (4%)
• Apical lung … (1.5%)
• A-V conduction defects (rare)
• Amyloidosis (rare)

Answer 26

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

Question 27

Other organ involvement - Ankylosing Spondylitis

• … uveitis (iritis) – 40%
• Achillies … (enthesitis)
• … regurgitation (4%)
• Apical lung fibrosis (1.5%)
• A-V … defects (rare)
• …dosis (rare)

Answer 27

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

Question 28

Association of HLA-B27 and Spondyloarthropathies

Answer 28

general healthy can be positive for HLA-B27 - note

Question 29

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

Answer 29

• 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

Question 30

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

Answer 30

• 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

Question 31

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.

Answer 31

• 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.

Question 32

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.

Answer 32

• 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.

Question 33

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.

Answer 33

• 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.

Question 34

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.

Answer 34

• 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.

Question 35

Reactive Arthritis Triad

• Common organisms:
• Chlamydia / Gonorrhoea, Shigella / Salmonella / Yersinia / Campylobacter / C.Difficile, Flu
• Cant see, cant pee, cant climb a tree…

Answer 35

• 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

Question 36

Reactive Arthritis Triad

• Common organisms:
• … / Gonorrhoea, Shigella / Salmonella / Yersinia / Campylobacter / C.Difficile, Flu
• …: discharge, erythema, burning, photophobia
• …: dysuria, urgency, frequency, discharge
• …: Knees, ankles, feet

Answer 36

• 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

Question 37

Most common organism causing reactive arthritis

Answer 37

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

Question 38

triad for…

Answer 38

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

Question 39

Reactive arthritis images

Answer 39

Question 40

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

Answer 40

• 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

Question 41

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

Answer 41

• 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

Question 42

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

Answer 42

• 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

Question 43

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

Answer 43

• 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

Question 44

THE MOLL AND WRIGHT CLASSIFICATION OF PSORIATIC ARTHRITIS

• Arthritis with … joint involvement predominant
• Arthritis M…
• Symmetric polyarthritis – indistinguishable from RA
• Asymmetric oligoarticular arthritis
• … spondylitis

Answer 44

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

Question 45

THE MOLL AND WRIGHT CLASSIFICATION OF PSORIATIC ARTHRITIS

• Arthritis with DIP joint involvement predominant
• Arthritis mutilans
• … polyarthritis – indistinguishable from RA
• … oligoarticular arthritis
• Predominant spondylitis

Answer 45

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

Question 46

Symmetric polyarthritis – indistinguishable from …

Answer 46

Symmetric polyarthritis – indistinguishable from RA

Question 47

X-ray shows what?

Answer 47

Classic ‘pencil in cup’ erosive changes - Psoriatic Arthritis

Question 48

Anti-TNF in Psoriatic Arthritis

Answer 48

Question 49

Seronegative Spondyloarthropathy Summary

Answer 49

Question 50

Which joints are diseased?

Answer 50

PIPs / DIPs

Question 51

Name the radiological appearance seen on the x-ray

Answer 51

Pencil in cup - psoriatic arthritis

Question 52

Name the pathology

Answer 52

Dactylitis

Question 53

What is vasculitis?

Answer 53

Vasculitis means inflammation of the blood vessels.

Question 54

Vasculitis Presentations

Answer 54

Question 55

Vasculitis Classification

Answer 55

Question 56

Vasculitis - Classification tree

Answer 56

Question 57

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?

Answer 57

• 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

Question 58

Vasculitis is classified into 3 groups, which are…

Answer 58

Small, medium and large

Question 59

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

Answer 59

• 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

Question 60

Examination Features - GCA

• Abnormal … Temporal Artery
  
  • Tender
  • Thickened
  • Reduced/ Absent …
• … Tenderness
• Cranial Nerve …
• Vascular …
• … Pulses

Answer 60

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

Question 61

Vasculitis Mimickers:

• Infections: Hep B/C, N. Meningitidis
• Drug: …, …
• Medications: NSAIDs, Abx, Thiazide Diuretics, Warfarin
• Malignancy: Lymphomas, …
• Inflammatory: … / SLE

Answer 61

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

Question 62

Vasculitis Mimickers:

• Infections: Hep B/C, N. …
• Drug: Cocaine, Amphetamines
• Medications: …, Abx, … Diuretics, …
• Malignancy: …, Myeloma
• Inflammatory: RA / …

Answer 62

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

Question 63

ANCA positive Vasculitis Treatment

Answer 63

Question 64

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 / …)

Answer 64

• 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)

Question 65

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)

Answer 65

• 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)

Question 66

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

• …. (3 days IV Methylpred 1g)
• Cyclophosphamide / R…
• Azathioprine / M…

Answer 66

• Corticosteroids (3 days IV Methylpred 1g)
• Cyclophosphamide / Rituximab
• Azathioprine / Methotrexate

Question 67

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

• Corticosteroids (… days IV Methylpred 1g)
• C… / Rituximab
• A… / Methotrexate

Answer 67

• Corticosteroids (3 days IV Methylpred 1g)
• Cyclophosphamide / Rituximab
• Azathioprine / Methotrexate

Question 68

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 …

Answer 68

• 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

Question 69

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

Answer 69

• 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

Question 70

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 …

Answer 70

• 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

Question 71

Examination Features - GCA

• Abnormal Superficial Temporal Artery
  
  • …
  • …
  • …/ … Pulsation
• Scalp …
• … Nerve Palsies
• … Bruits
• Asymmetric …

Answer 71

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

Question 72

Visual Loss - GCA

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

Answer 72

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

Question 73

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)

Answer 73

• 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)

Question 74

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)

Answer 74

• 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)

Question 75

GCA Investigations…

Answer 75

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

Question 76

GCA Investigations…

• …, U&Es, LFTs, CRP, ESR.
• CXR
• U..
• Temporal Artery B…
• … of Temporal Arteries

Answer 76

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

Question 77

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.

Answer 77

• 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.

Question 78

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.

Answer 78

• 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.

Question 79

This is a Temporal Biopsy sample showing ….

Answer 79

Immune cell infiltration

Question 80

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

Answer 80

• 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.

Question 81

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

Answer 81

• 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.

Question 82

Additional Management - GCA

• … Protection
• … … Inhibitor (PPI)
• Regular … Review
• Relapse Management: steroids or steroid-sparing agent

Answer 82

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

Question 83

Additional Management - GCA

• Bone …
• Proton Pump …
• Regular … Review
• Relapse Management: … or …-sparing agent

Answer 83

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

Question 84

PMR diagnostic criteria (Polymyalgia rheumatica)

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

Answer 84

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

Question 85

PMR diagnostic criteria (Polymyalgia rheumatica)

Answer 85

Question 86

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> …

Answer 86

• 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

Question 87

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

Answer 87

• 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

Question 88

SLE Signs/Symptoms

• … rash
• Discoid rash
• Serositis – Pleuritis / Pericarditis
• … ulcers
• Arthritis – non-erosive
• Photosensitivity
• … disorders
• … disorders
• ANA + anti Smith / dsDNA / antiphospholipid abs
• … symptoms

Answer 88

• 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

Question 89

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

Answer 89

• 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

Question 90

SLE PATHOPHYSIOLOGY

Answer 90

Question 91

SLE PATHOPHYSIOLOGY

Answer 91

Question 92

SLE – 2019 classification criteria

• …. positive ≥1:80
• … clinical and ≥10 points (count highest in each criterion)

Answer 92

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

Question 93

SLE – 2019 classification criteria

• ANA positive ≥…
• One clinical and ≥… points (count highest in each criterion)

Answer 93

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

Question 94

SLE

Answer 94

Question 95

SLE Investigations

• … – … protein: … ratio
• Full blood count
• … and electrolytes
• ESR
• …
• … function test
• Antibodies: ANA; ENA; Anti –dsDNA; Lupus anticoagulant; ANTI C1q;
• C3, C…

Answer 95

• 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

Question 96

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…

Answer 96

• 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

Question 97

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

Answer 97

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

Question 98

SLE Principals of management

• Nonpharmacological management (… protection; … cessation; … risk modification; … prevention)

Answer 98

• Nonpharmacological management (sun protection; smoking cessation; CVD risk modification; osteoporosis prevention)

Question 99

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

Answer 99

• 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

Question 100

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

Answer 100

• 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

Question 101

Sjogren’s Syndrome Diagnosis

• Need …/6 criteria including one of the red criterion

Answer 101

• Need 4/6 criteria including one of the red criterion

Question 102

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

Answer 102

• 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

Question 103

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

Answer 103

• 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

Question 104

Sjogren’s Syndrome Treatment

• Conservative:
  
  • Stop … / … hygiene /
  • Artificial … and …
• Medications:
  
  • Cholinergic agents: pilocarpine / cevimeline
  • … / Steroids
  • … eg. hydroxychloroquine / methotrexate / rituximab

Answer 104

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

Question 105

Sjogren’s Syndrome Treatment

• Conservative:
  
  • Stop smoking / oral hygiene /
  • … tears and saliva
• Medications:
  
  • … agents: pilocarpine / cevimeline
  • NSAIDs / …
  • Immunosuppressants eg. hydroxychloroquine / … / …

Answer 105

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

Question 106

Spot Diagnosis

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

Answer 106

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

Question 107

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

Answer 107

• 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

Question 108

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

Answer 108

• 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

Question 109

Systemic Sclerosis Clinical Features

• Sclerodactyly
• … phenomenon
• Heartburn
• Abnormal nailfold …
• …iectasia
• Pulmonary arterial hypertension
• … lung disease
• Lower bowel: distension / diarrhea / constipation
• Renal failure / proteinuria
• … dysfunction

Answer 109

• 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

Question 110

Systemic Sclerosis Clinical Features

• Sclerodactyly
• Raynaud’s phenomenon
• …
• Abnormal … capillaries
• Telangiectasia
• Pulmonary arterial …
• Interstitial lung disease
• … bowel: distension / diarrhea / constipation
• … failure / proteinuria
• Erectile dysfunction

Answer 110

• 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

Question 111

Systemic Sclerosis Types

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

Answer 111

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

Question 112

Systemic Sclerosis Treatment

• Conservative
  
  • Stop …
  • Controlled …
  • Wear …
  • Warm … baths
• Medications
  
  • Raynaud’s – amlodipine / losartan / fluoxetine / sildenafil
  • Reflux - …
  • …- mycophenolate, methotrexate, cyclophosphamide, steroids, rituximab, IV immunoglobulins

Answer 112

• 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

Question 113

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

Answer 113

• 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