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
who gets reactive arthritis
young adults 20-40 years old
26
what is the Reiter's syndrome/Reactive arthritis triad
Urethritis Conjuntivitis/Uveitis/Iritis Arthritis
27
symptoms of reactive arthritis
fever/fatigue/malaise asymmetrical monoarthritis or oligoarthritis enthesitis mucocutaneous lesions ocular lesions; conjuntivits, iritis visceral manifestations; mild renal disease, carditis
28
what mucocutaneous lesions can be seen in reactive arthritis
Keratodema Blenorrhagica (brown, raised plaques on soles and palms) Circinate balanitis (painless penile ulcers secondary to syphillis) Painless oral ulcers Hyperkeratotic nails
29
tests to confirm reactive arthritis
elevated ESR, CRP, PV negative ANA and Rf cultures (blood/urine/stool) joint fluid analysis (rule out infection)
30
Tx of reactive arthritis
``` 90% resolves spontaneously within 6 months NSAIDS Corticosteroids Antibiotics for underlying infection DMARD - if resistant or chronic ```
31
what is Enteropathic arthritis
Associated with inflammatory bowel disease eg. Crohn’s, Ulcerative colitis, inflammatory bowel disease
32
how will Enteropathic arthritis present
Arthritis in several joints, especially the knees, ankles, elbows, and wrists, and sometimes in the spine, hips, or shoulders
33
what are features of IBD associated with Enteropathic arthritis
Crohn's suffers will have sacroiliitis (20% of time) | Worsening of symptoms during flare-ups
34
clinical symptoms of EA
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
Ix for EA
``` 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
Tx for EA
``` treat IBD analgesia steroids DMD Anti-TNF ```
37
what is not recommended in EA
NSAIDS - exacerbate IBD
38
what is vasculitis
inflammation of blood vessels, often with ischema, necrosis and organ inflammation can affect any blood vessel
39
what is primary vasculitis
inflammatory response that targets the vessel walls and has no known cause. Sometimes this is autoimmune.
40
what is secondary vasculitis
triggered by an infection, a drug, or a toxin or may occur as part of another inflammatory disorder or cancer.
41
what are the 2 types of large vessel vasculitis
``` Takayasu arteritis (TA) Giant Cell Arteritis (GCA) ```
42
features of TA
affects those under 40 years and is commoner in females. It is much more prevalent in Asian populations.
43
features of GCA
affects those over 50 years. It typically causes temporal arteritis but the aorta and other large vessels may be affected
44
what are GCA and TA characterised by
granulomatous infiltration of the walls of the large vessels.
45
how is a giant cell made
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
clinical findings of large vessel vasculitis
``` Bruit esp at Carotid artery Blood pressure difference of extremities Claudication in arms/legs Carotodynia or vessel tenderness hypertension ```
47
what is temporal arteritis associated with
PMR 50% with GCA have PMR 15% with PMR develop GCA
48
symptoms of temporal arteritis
unilateral temporal headache scalp tenderness jaw claudication temporal arteries may be prominent with reduced pulsation
49
what are complications of temporal arteritis
risk of blindness due to ischaemia of the optic nerve
50
Ix for large vessel vasculitis
ESR, PV and CRP - all raised Temporal artery biopsy MR angiogram or PET CT may show vessel wall thickening/stenosis
51
why might a temporal artery biopsy be negative even if temporal arteritis is present
skip lesions
52
Mx for GCA
40mg - 60mg Prednisolone
53
what is Kawasaki disease
medium vessel vasculitis seen in children under 5 commonly. can affect various vessels most important is coronary arteries where aneurysms can develop
54
what is polyarteritis nodosa characterised by
necrotizing inflammatory lesions that affect arteries at vessel bifurcations, resulting in microaneurysm formation and aneurysms.
55
what is polyarteritis nodosa associated with
Hep B
56
what are the two sub categories of small vessel vasculitis
ANCA associated vasculitis (AAV) | Non-ANCA associated vasculitis
57
what is granulomatosis with polyangiitis (GPA)
previous name Wegner's | Granulomatous inflammation of respiratory tract, small and medium vessels.
58
what is common in GPA
Necrotising glomerulonephritis common.
59
what is eosinophilic granulomatosis with polyangiitis (EGPA)
previous name Churg-Strauss | Eosinophilic granulomatous inflammation of respiratory tract, small and medium vessels.
60
what is EGPA associated with
Associated with asthma
61
what is microscopic polyangiitis (MPA)
Necrotising vasculitis with few immune deposits.
62
what is associated with MPA
Necrotising glomerulonephritis
63
GPA symptoms
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
x-ray signs of GPA
cavitating nodules in the lungs
65
what does Necrotising glomerulonephritis manifest as
blood and protein in urine
66
many features of EGPA are similar to GPA - what is the main difference
presence of late onset asthma and a high eosinophil count
67
criteria for EGPA - should have 4 or more
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
what is used to detect Anti-neutrophil cytoplasmic antibodies (ANCAs)
immunofluoresence
69
what ANCA is associated with GPA
cANCA
70
what ANCA is associated with MPA and EGPA
pANCA
71
what else can be used to diagnose small vessel vasculitis
PR3 - raised in GPA | MPO - raised in EGPA, but more so in MPA
72
management of AAV
early - methotrexate + steroids late - Cyclophosphamide + steroids (Rituximab + steroids) very late - IV immunoglobulins or Rituximab
73
what is HSP and who gets it
Henoch-Schönlein purpura (HSP) an acute immunoglobulin A (IgA)–mediated disorder children aged 2-11
74
pathogenesis of HSP
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
what precedes most cases of HSP
preceding URTI, pharyngeal infection, or GI infection Most common is group A streptococcus Preceding illness usually predates HSP by 1-3 weeks
76
how does HSP present
``` Purpuric rash typically over buttocks and lower limbs Colicky abdominal pain Bloody diarrhoea Joint pain +/- swelling Renal involvement ```
77
Mx for HSP
self-limiting | tend to resolve within 8 weeks
78
what is it essential to do in a case of HSP
perform urinalysis to screen for renal involvement
79
What test is best for seeing earliest changes in AS
MRI