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Rheumatology SB > Spondyloarhropathies and Vasculitis > Flashcards

Spondyloarhropathies and Vasculitis Flashcards

1
Q

definition of Spondyloarthropathy

A

Family of inflammatory arthritides characterized by involvement of both the spine and joints, principally in genetically predisposed (HLA B27 positive) individuals

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2
Q

what is associated with Spondyloarthropathy

A

HLA B27

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3
Q

disease subgroups of Spondyloarthropathy

A

Ankylosing Spondylitis
Psoriatic Arthritis
Reactive Arthritis ( Reiter’s Syndrome)
Enteropathic Arthritis

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4
Q

what is the difference between mechanical and inflammatory pain

A

Mechanical- worsened by activity, typically worst at end of day, better with rest
Inflammatory- worse with rest, better with activity, early morning stiffness

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5
Q

shared rheumatological features of Spondyloarthropathy

A

Sacroiliac and spinal involvement
Enthesitis: inflammation at insertion of tendons into bones eg Achilles tendinitis, plantar fasciitis…
Inflammatory arthritis
Dactylitis (“sausage” digits)- inflammation of entire digit

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6
Q

shared extra-articular features

A

Ocular inflammation (Anterior uveitis, conjuntivitis)
Mucocutaneous lesions
Rare Aortic incompetence or heart block
No rheumatoid nodules

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7
Q

what is AS

A

Chronic systemic inflammatory disorder that primarily affects the spine.

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8
Q

what is the hallmark of AS

A

Sacroiliac joint involvement (sacroiliitis)

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9
Q

Symptoms of AS

A 
Gradual onset low back pain
Worse at night
Morning stiffness relieved by exercise 
Pain radiates from sacroiliac joint to hips/buttocks 
Improves at end of the day
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10
Q

who gets AS

A

M > F
Late adolescence or early adulthood
+ve HLA B27
FH of AS

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11
Q

criteria for AS

A
  1. Limited lumbar motion
  2. Lower back pain for 3 months
    - Improved with exercise
    - Not relieved by rest
  3. Reduced chest expansion
  4. Bilateral, Grade 2 to 4, sacroiliitis on X ray
  5. Unilateral, Grade 3 to 4, sacroiliitis on X ray
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12
Q

extra articular features of AS

A 
Enthesitis, esp Achilles tendonitis and plantar fasciitis 
Acute Iritis - can lead to blindness 
Anterior uveitis 
Osteoporosis
Aortic value incompetence
Pulmonary apical fibrosis
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13
Q

what is AS also known as

A 
'A' Disease
Axial Arthritis
Anterior Uveitis
Aortic Regurgitation
Apical fibrosis
Amyloidosis/ Ig A Nephropathy
Achilles tendinitis
PlAntar Fasciitis
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14
Q

what can be seen on examination of AS

A

Modified Schober test - lumbar flexion
Poor chest examination
Question mark posture

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15
Q

x-ray of AS

A 
'Bamboo spine'
sacroiliitis 
Syndesmophytes
sacroiliac scelerosis
vertebral fusion
erosions

changes seen on x-ray are irreversible

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16
Q

Tx of AS

A

1 - NSAIDs + physio, occupational therapy
2 - corticosteroid injections
3- DMD e.g. sulfasalazine
4 - anti TNF e.g. Infliximab

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17
Q

what is Psoriatic Arthritis

A

Inflammatory arthritis associated with psoriasis,
but patients can have PsA without psoriasis
No Rheumatoid nodules
Rheumatoid factor negative

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18
Q

features of PsA

A

Inflammatory Arthritis

Sacroiliitis: often asymmetric
may be associated with spondylitis

Nail involvement (Pitting, onycholysis, hyperkeratosis)

Dactylitis

Enthesitis:

  • Achilles tendinitis
  • Plantar fasciitis

Extra articular features (eye disease)

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19
Q

what are the clinical subgroups of PsA

A
  • Confined to distal interphalangeal joints (DIP) hands/feet
  • Symmetric polyarthritis
  • Spondylitis (spine involvement) with or without peripheral joint involvement
  • Asymmetric oligoarthritis with dactylitis
  • Arthritis mutilans
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20
Q

diagnosis of PsA

A

raised inflammatory markers
Hx
negative Rf

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21
Q

x-ray appearance of PsA

A

Marginal erosions and “whiskering”
“Pencil in cup” deformity
Osteolysis
Enthesitis

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22
Q

Tx of PsA

A

NSAIDs
Corticosteroids
DMARDS
Anti-TNF

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23
Q

what is reactive arthritis

A

Infection induced systemic illness characterized primarily by an inflammatory synovitis from which viable microorganisms cannot be cultured

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24
Q

when do symptoms of Reactive arthritis occur

A

1-4 weeks after infection

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25
Q

who gets reactive arthritis

A

young adults 20-40 years old

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26
Q

what is the Reiter’s syndrome/Reactive arthritis triad

A

Urethritis
Conjuntivitis/Uveitis/Iritis
Arthritis

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27
Q

symptoms of reactive arthritis

A

fever/fatigue/malaise
asymmetrical monoarthritis or oligoarthritis
enthesitis
mucocutaneous lesions
ocular lesions; conjuntivits, iritis
visceral manifestations; mild renal disease, carditis

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28
Q

what mucocutaneous lesions can be seen in reactive arthritis

A

Keratodema Blenorrhagica (brown, raised plaques on soles and palms)
Circinate balanitis (painless penile ulcers secondary to syphillis)
Painless oral ulcers
Hyperkeratotic nails

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29
Q

tests to confirm reactive arthritis

A

elevated ESR, CRP, PV
negative ANA and Rf
cultures (blood/urine/stool)
joint fluid analysis (rule out infection)

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30
Q

Tx of reactive arthritis

A 
90% resolves spontaneously within 6 months
NSAIDS
Corticosteroids
Antibiotics for underlying infection
DMARD - if resistant or chronic
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31
Q

what is Enteropathic arthritis

A

Associated with inflammatory bowel disease eg. Crohn’s, Ulcerative colitis, inflammatory bowel disease

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32
Q

how will Enteropathic arthritis present

A

Arthritis in several joints, especially the knees, ankles, elbows, and wrists, and sometimes in the spine, hips, or shoulders

33
Q

what are features of IBD associated with Enteropathic arthritis

A

Crohn’s suffers will have sacroiliitis (20% of time)

Worsening of symptoms during flare-ups

34
Q

clinical symptoms of EA

A

GI- loose, watery stool with mucous and blood)
Weight loss, low grade fever
Eye involvement ( uveitis)
Skin involvement ( pyoderma gangrenosum)
Enthesitis ( Archilles tendonitis, plantar fasciitis, lateral epicondylitis)
Oral- apthous ulcers

35
Q

Ix for EA

A 
Upper and lower GI endoscopy with biopsy showing ulceration/ colitis
Joint aspirate- no organisms or crystals
Raised inflammatory markers- CRP, PV
X ray/ MRI showing sacroiliitis
USS showing synovitis/ tenosynovitis
36
Q

Tx for EA

A 
treat IBD 
analgesia
steroids
DMD
Anti-TNF
37
Q

what is not recommended in EA

A

NSAIDS - exacerbate IBD

38
Q

what is vasculitis

A

inflammation of blood vessels, often with ischema, necrosis and organ inflammation
can affect any blood vessel

39
Q

what is primary vasculitis

A

inflammatory response that targets the vessel walls and has no known cause. Sometimes this is autoimmune.

40
Q

what is secondary vasculitis

A

triggered by an infection, a drug, or a toxin or may occur as part of another inflammatory disorder or cancer.

41
Q

what are the 2 types of large vessel vasculitis

A 
Takayasu arteritis (TA)
Giant Cell Arteritis (GCA)
42
Q

features of TA

A

affects those under 40 years and is commoner in females. It is much more prevalent in Asian populations.

43
Q

features of GCA

A

affects those over 50 years. It typically causes temporal arteritis but the aorta and other large vessels may be affected

44
Q

what are GCA and TA characterised by

A

granulomatous infiltration of the walls of the large vessels.

45
Q

how is a giant cell made

A

granuloma - area formed by body to wall off something the body sees as a threat

  • formed in response to infection
  • enclose them to prevent them cause damage
  • collection of macrophages that are walled off around the edge
  • macrophages merge together to make a giant cell
46
Q

clinical findings of large vessel vasculitis

A 
Bruit esp at Carotid artery
Blood pressure difference of extremities
Claudication in arms/legs
Carotodynia or vessel tenderness
hypertension
47
Q

what is temporal arteritis associated with

A

PMR
50% with GCA have PMR
15% with PMR develop GCA

48
Q

symptoms of temporal arteritis

A

unilateral temporal headache
scalp tenderness
jaw claudication
temporal arteries may be prominent with reduced pulsation

49
Q

what are complications of temporal arteritis

A

risk of blindness due to ischaemia of the optic nerve

50
Q

Ix for large vessel vasculitis

A

ESR, PV and CRP - all raised
Temporal artery biopsy
MR angiogram or PET CT may show vessel wall thickening/stenosis

51
Q

why might a temporal artery biopsy be negative even if temporal arteritis is present

A

skip lesions

52
Q

Mx for GCA

A

40mg - 60mg Prednisolone

53
Q

what is Kawasaki disease

A

medium vessel vasculitis seen in children under 5 commonly.
can affect various vessels
most important is coronary arteries where aneurysms can develop

54
Q

what is polyarteritis nodosa characterised by

A

necrotizing inflammatory lesions that affect arteries at vessel bifurcations, resulting in microaneurysm formation and aneurysms.

55
Q

what is polyarteritis nodosa associated with

56
Q

what are the two sub categories of small vessel vasculitis

A

ANCA associated vasculitis (AAV)

Non-ANCA associated vasculitis

57
Q

what is granulomatosis with polyangiitis (GPA)

A

previous name Wegner’s

Granulomatous inflammation of respiratory tract, small and medium vessels.

58
Q

what is common in GPA

A

Necrotising glomerulonephritis common.

59
Q

what is eosinophilic granulomatosis with polyangiitis (EGPA)

A

previous name Churg-Strauss

Eosinophilic granulomatous inflammation of respiratory tract, small and medium vessels.

60
Q

what is EGPA associated with

A

Associated with asthma

61
Q

what is microscopic polyangiitis (MPA)

A

Necrotising vasculitis with few immune deposits.

62
Q

what is associated with MPA

A

Necrotising glomerulonephritis

63
Q

GPA symptoms

A

ENT

  • ‘saddle nose’ (due to cartilage ischaemia)
  • sinusitis
  • mouth ulcers
  • otitis media
  • deafness

Resp

  • pulmonary infiltrates
  • cough
  • hemoptysis

Derm

  • palpable purpura
  • cutaneous ulcers

CNS
- cranial nerve palsies

Ocular

  • conjunctivitis
  • uveitis
  • retinal artery occlusion
64
Q

x-ray signs of GPA

A

cavitating nodules in the lungs

65
Q

what does Necrotising glomerulonephritis manifest as

A

blood and protein in urine

66
Q

many features of EGPA are similar to GPA - what is the main difference

A

presence of late onset asthma and a high eosinophil count

67
Q

criteria for EGPA - should have 4 or more

A

Asthma (wheezing, expiratory rhonchi)
Eosinophilia of more than 10% in peripheral blood
Paranasal sinusitis
Pulmonary infiltrates (may be transient)
Histological proof of vasculitis with extravascular eosinophils
Mononeuritis multiplex or polyneuropathy

68
Q

what is used to detect Anti-neutrophil cytoplasmic antibodies (ANCAs)

A

immunofluoresence

69
Q

what ANCA is associated with GPA

70
Q

what ANCA is associated with MPA and EGPA

71
Q

what else can be used to diagnose small vessel vasculitis

A

PR3 - raised in GPA

MPO - raised in EGPA, but more so in MPA

72
Q

management of AAV

A

early - methotrexate + steroids
late - Cyclophosphamide + steroids
(Rituximab + steroids)
very late - IV immunoglobulins or Rituximab

73
Q

what is HSP and who gets it

A

Henoch-Schönlein purpura (HSP)
an acute immunoglobulin A (IgA)–mediated disorder
children aged 2-11

74
Q

pathogenesis of HSP

A

Generalized vasculitis involving the small vessels of the skin, the gastrointestinal (GI) tract, the kidneys, the joints, and, rarely, the lungs and the central nervous system (CNS)

75
Q

what precedes most cases of HSP

A

preceding URTI, pharyngeal infection, or GI infection
Most common is group A streptococcus
Preceding illness usually predates HSP by 1-3 weeks

76
Q

how does HSP present

A 
Purpuric rash typically over buttocks and lower limbs
Colicky abdominal pain
Bloody diarrhoea
Joint pain +/- swelling
Renal involvement
77
Q

Mx for HSP

A

self-limiting

tend to resolve within 8 weeks

78
Q

what is it essential to do in a case of HSP

A

perform urinalysis to screen for renal involvement

79
Q

What test is best for seeing earliest changes in AS

Rheumatology SB (7 decks)

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