Multi-system Autoimmune Disease Flashcards
(113 cards)
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.
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
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
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
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
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
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
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
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
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
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
12
Q
Imaging for Ankylosing Spondylitis diagnosis
A
Sacroiliac joint X-ray, if normal or equivocal, then CT or MRI, or HLA-B27 test
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
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*
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*
16
Q
Examining tenderness over sacroiliac joint
A
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).
18
Q
Positive Schober’s Test
A
Less than 5cm increase in length with forward flexion: Decreased lumbar spine range of motion, ankylosing spondylitis
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
20
Q
Ankylosis of spine
A
on right
21
Q
Ankylosed sacroiliac joints
A
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
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
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
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
* 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
26
_Other organ involvement - Ankylosing Spondylitis_
* Anterior ... (iritis) – 40%
* ... tendonitis (enthesitis)
* Aortic ... (4%)
* Apical lung ... (1.5%)
* A-V conduction defects (rare)
* Amyloidosis (rare)
* Anterior **uveitis** (iritis) – 40%
* **Achillies** tendonitis (enthesitis)
* Aortic **regurgitation** (4%)
* Apical lung **fibrosis** (1.5%)
* A-V conduction defects (rare)
* Amyloidosis (rare)
27
_Other organ involvement - Ankylosing Spondylitis_
* ... uveitis (iritis) – 40%
* Achillies ... (enthesitis)
* ... regurgitation (4%)
* Apical lung fibrosis (1.5%)
* A-V ... defects (rare)
* ...dosis (rare)
* **Anterior** uveitis (iritis) – 40%
* Achillies tendonitis (enthesitis)
* **Aortic** regurgitation (4%)
* Apical lung fibrosis (1.5%)
* A-V **conduction** defects (rare)
* **Amyloidosis** (rare)
28
_Association of HLA-B27 and Spondyloarthropathies_
general healthy can be positive for HLA-B27 - note
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
* 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
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
* **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
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.
* **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.
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.
* 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.
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.
* 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.
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.
* 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.
35
_Reactive Arthritis Triad_
* Common organisms:
* Chlamydia / Gonorrhoea, Shigella / Salmonella / Yersinia / Campylobacter / C.Difficile, Flu
* Cant see, cant pee, cant climb a tree...
* 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
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
* 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
37
Most common organism causing reactive arthritis
Chlamydia (but can be Gonorrhoea, Shigella / Salmonella / Yersinia / Campylobacter / C.Difficile, Flu)
38
triad for...
* reactive arthritis:
* Conjunctivitis: discharge, erythema, burning, photophobia
* Urethritis: dysuria, urgency, frequency, discharge
* Arthritis: Knees, ankles, feet
39
Reactive arthritis images
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
* 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
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
* **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**
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
* 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
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
* 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
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
* Arthritis with **DIP** joint involvement predominant
* Arthritis **mutilans**
* Symmetric polyarthritis – indistinguishable from RA
* Asymmetric oligoarticular arthritis
* **Predominant** spondylitis
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
* Arthritis with DIP joint involvement predominant
* Arthritis mutilans
* **Symmetric** polyarthritis – indistinguishable from RA
* **Asymmetric** oligoarticular arthritis
* Predominant spondylitis
46
Symmetric polyarthritis – indistinguishable from ...
Symmetric polyarthritis – indistinguishable from **RA**
47
X-ray shows what?
Classic ‘pencil in cup’ erosive changes - Psoriatic Arthritis
48
Anti-TNF in Psoriatic Arthritis
49
_Seronegative Spondyloarthropathy Summary_
50
Which joints are diseased?
PIPs / DIPs
51
Name the radiological appearance seen on the x-ray
Pencil in cup - psoriatic arthritis
52
Name the pathology
Dactylitis
53
What is vasculitis?
Vasculitis means inflammation of the blood vessels.
54
Vasculitis Presentations
55
_Vasculitis Classification_
56
Vasculitis - Classification tree
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?
* 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
Vasculitis is classified into 3 groups, which are...
Small, medium and large
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
* **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
_Examination Features - GCA_
* Abnormal ... Temporal Artery
* Tender
* Thickened
* Reduced/ Absent ...
* ... Tenderness
* Cranial Nerve ...
* Vascular ...
* ... Pulses
* **Abnormal Superficial Temporal Artery**
* **Tender**
* **Thickened**
* **Reduced/ Absent Pulsation**
* **Scalp Tenderness**
* **Cranial Nerve Palsies**
* **Vascular Bruits**
* **Asymmetric Pulses**
61
_Vasculitis Mimickers:_
* Infections: Hep B/C, N. Meningitidis
* Drug: ..., ...
* Medications: NSAIDs, Abx, Thiazide Diuretics, Warfarin
* Malignancy: Lymphomas, ...
* Inflammatory: ... / SLE
* Infections: Hep B/C, N. Meningitidis
* Drug: **Cocaine, Amphetamines**
* Medications: NSAIDs, Abx, Thiazide Diuretics, Warfarin
* Malignancy: Lymphomas, **Myeloma**
* Inflammatory: **RA** / SLE
62
_Vasculitis Mimickers:_
* Infections: Hep B/C, N. ...
* Drug: Cocaine, Amphetamines
* Medications: ..., Abx, ... Diuretics, ...
* Malignancy: ..., Myeloma
* Inflammatory: RA / ...
* **Infections: Hep B/C, N. Meningitidis**
* **Drug: Cocaine, Amphetamines**
* **Medications: NSAIDs, Abx, Thiazide Diuretics, Warfarin**
* **Malignancy: Lymphomas, Myeloma**
* **Inflammatory: RA / SLE**
63
_ANCA positive Vasculitis Treatment_
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 / ...)
* 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
_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)
* 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
_In general, flares of systemic vasculitides require escalation of immunosuppressive treatment:_
* .... (3 days IV Methylpred 1g)
* Cyclophosphamide / R...
* Azathioprine / M...
* **Corticosteroids** (3 days IV Methylpred 1g)
* Cyclophosphamide / **Rituximab**
* Azathioprine / **Methotrexate**
67
_In general, flares of systemic vasculitides require escalation of immunosuppressive treatment:_
* Corticosteroids (... days IV Methylpred 1g)
* C... / Rituximab
* A... / Methotrexate
* Corticosteroids (**3** days IV Methylpred 1g)
* **Cyclophosphamide** / Rituximab
* **Azathioprine** / Methotrexate
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 ...
* 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
_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
* 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
_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 ...
* **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
_Examination Features - GCA_
* Abnormal Superficial Temporal Artery
* ...
* ...
* .../ ... Pulsation
* Scalp ...
* ... Nerve Palsies
* ... Bruits
* Asymmetric ...
* **Abnormal Superficial Temporal Artery**
* **Tender**
* **Thickened**
* **Reduced/ Absent Pulsation**
* **Scalp Tenderness**
* **Cranial Nerve Palsies**
* **Vascular Bruits**
* **Asymmetric Pulses**
72
_Visual Loss - GCA_
* ... Defect
* Afferent ... Defect
* Anterior Ischaemic Optic Neuritis
* Central Retinal Artery ...
* ...
* Early ... Review
* **Field Defect**
* **Afferent Pupillary Defect**
* **Anterior Ischaemic Optic Neuritis**
* **Central Retinal Artery Occlusion**
* **Diplopia**
* **Early Opthalmology Review**
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)
* 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
_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)
* **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
_GCA Investigations..._
* FBC, U&Es, LFTs, CRP, ESR.
* CXR
* Urinalysis
* Temporal Artery biopsy
* USS Temporal Arteries
76
_GCA Investigations..._
* ..., U&Es, LFTs, CRP, ESR.
* CXR
* U..
* Temporal Artery B...
* ... of Temporal Arteries
* **FBC, U&Es, LFTs, CRP, ESR.**
* **CXR**
* **Urinalysis**
* **Temporal Artery biopsy**
* **USS Temporal Arteries**
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.
* **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
_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.
* T**emporal 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
This is a Temporal Biopsy sample showing ....
Immune cell infiltration
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
* **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
_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
* **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
_Additional Management - GCA_
* ... Protection
* ... ... Inhibitor (PPI)
* Regular ... Review
* Relapse Management: steroids or steroid-sparing agent
* **Bone Protection**
* **Proton Pump Inhibitor (PPI)**
* **Regular Specialist Review**
* **Relapse Management: steroids or steroid-sparing agent**
83
_Additional Management - GCA_
* Bone ...
* Proton Pump ...
* Regular ... Review
* Relapse Management: ... or ...-sparing agent
* **Bone Protection**
* **Proton Pump Inhibitor (PPI)**
* **Regular Specialist Review**
* **Relapse Management: steroids or steroid-sparing agent**
84
_PMR diagnostic criteria (Polymyalgia rheumatica)_
_Also -_ PMR is associated with ...: 1/50 with ... get PMR but 1/10 PMR get ....
* PMR is associated with **GCA**: 1/50 with **GCA** get PMR but 1/10 PMR get **GCA**.
85
_PMR diagnostic criteria (Polymyalgia rheumatica)_
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\> ...
* 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
_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
* 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
_SLE Signs/Symptoms_
* ... rash
* Discoid rash
* Serositis – Pleuritis / Pericarditis
* ... ulcers
* Arthritis – non-erosive
* Photosensitivity
* ... disorders
* ... disorders
* ANA + anti Smith / dsDNA / antiphospholipid abs
* ... symptoms
* **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
_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
* **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
_SLE PATHOPHYSIOLOGY_
91
_SLE PATHOPHYSIOLOGY_
92
_SLE – 2019 classification criteria_
* .... positive ≥1:80
* ... clinical and ≥10 points (count highest in each criterion)
* **ANA** positive ≥1:80
* **One** clinical and ≥10 points (count highest in each criterion)
93
_SLE – 2019 classification criteria_
* ANA positive ≥...
* One clinical and ≥... points (count highest in each criterion)
* **ANA** positive ≥1:80
* **One** clinical and ≥**10** points (count highest in each criterion)
94
_SLE_
95
_SLE Investigations_
* ... – ... protein: ... ratio
* Full blood count
* ... and electrolytes
* ESR
* ...
* ... function test
* Antibodies: ANA; ENA; Anti –dsDNA; Lupus anticoagulant; ANTI C1q;
* C3, C...
* **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
_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...
* **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
In SLE, if complement is low, disease is ... active
In SLE, if complement is low, disease is **more** active (C3 and C4 - measure)
98
_SLE Principals of management_
* Nonpharmacological management (... protection; ... cessation; ... risk modification; ... prevention)
* Nonpharmacological management **(sun protection; smoking cessation; CVD risk modification; osteoporosis prevention)**
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
* **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
_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
* **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
_Sjogren’s Syndrome Diagnosis_
* Need .../6 criteria including one of the red criterion
* **Need 4/6 criteria including one of the red criterion**
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**
* 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
_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**
* **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
_Sjogren’s Syndrome Treatment_
* Conservative:
* Stop ... / ... hygiene /
* Artificial ... and ...
* Medications:
* Cholinergic agents: pilocarpine / cevimeline
* ... / Steroids
* ... eg. hydroxychloroquine / methotrexate / rituximab
* **Conservative:**
* **Stop smoking / oral hygiene /**
* **Artificial tears and saliva**
* Medications:
* Cholinergic agents: pilocarpine / cevimeline
* **NSAIDs** / Steroids
* **Immunosuppressants** eg. hydroxychloroquine / methotrexate / rituximab
105
_Sjogren’s Syndrome Treatment_
* Conservative:
* Stop smoking / oral hygiene /
* ... tears and saliva
* Medications:
* ... agents: pilocarpine / cevimeline
* NSAIDs / ...
* Immunosuppressants eg. hydroxychloroquine / ... / ...
* Conservative:
* Stop smoking / oral hygiene /
* **Artificial** tears and saliva
* Medications:
* **Cholinergic** agents: pilocarpine / cevimeline
* NSAIDs / **Steroids**
* Immunosuppressants eg. hydroxychloroquine / **methotrexate / rituximab**
106
_Spot Diagnosis_
* ... syndrome:
* Reynaud's (WHITE BLUE RED)
* Telangeictasia of lips
* Tight shiny skin on finger - scelrodactyly
* Calcinosis
* Oesophageal dismotility - reflux
* **Crest** syndrome:
* Reynaud's (WHITE BLUE RED)
* Telangeictasia of lips
* Tight shiny skin on finger - scelrodactyly
* Calcinosis
* Oesophageal dismotility - reflux
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
* **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
_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
* 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
_Systemic Sclerosis Clinical Features_
* Sclerodactyly
* ... phenomenon
* Heartburn
* Abnormal nailfold ...
* ...iectasia
* Pulmonary arterial hypertension
* ... lung disease
* Lower bowel: distension / diarrhea / constipation
* Renal failure / proteinuria
* ... dysfunction
* **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
_Systemic Sclerosis Clinical Features_
* Sclerodactyly
* Raynaud’s phenomenon
* ...
* Abnormal ... capillaries
* Telangiectasia
* Pulmonary arterial ...
* Interstitial lung disease
* ... bowel: distension / diarrhea / constipation
* ... failure / proteinuria
* Erectile dysfunction
* **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
_Systemic Sclerosis Types_
* Limited = .... (calcinosis, Raynaud’s, Esophageal dysmotility, sclerodactyly, telangiectasias)
* ...= scleroderma proximal to elbows
* Overlap syndromes with SLE / ...
* Sine ...
* **Limited = CREST (calcinosis, Raynaud’s, Esophageal dysmotility, sclerodactyly, telangiectasias)**
* **Diffuse= scleroderma proximal to elbows**
* **Overlap syndromes with SLE / Sjogren’s**
* **Sine scleroderma**
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
* 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
_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
* **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**
