Spondyloarhropathies and Vasculitis Flashcards
definition of Spondyloarthropathy
Family of inflammatory arthritides characterized by involvement of both the spine and joints, principally in genetically predisposed (HLA B27 positive) individuals
what is associated with Spondyloarthropathy
HLA B27
disease subgroups of Spondyloarthropathy
Ankylosing Spondylitis
Psoriatic Arthritis
Reactive Arthritis ( Reiter’s Syndrome)
Enteropathic Arthritis
what is the difference between mechanical and inflammatory pain
Mechanical- worsened by activity, typically worst at end of day, better with rest
Inflammatory- worse with rest, better with activity, early morning stiffness
shared rheumatological features of Spondyloarthropathy
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
shared extra-articular features
Ocular inflammation (Anterior uveitis, conjuntivitis)
Mucocutaneous lesions
Rare Aortic incompetence or heart block
No rheumatoid nodules
what is AS
Chronic systemic inflammatory disorder that primarily affects the spine.
what is the hallmark of AS
Sacroiliac joint involvement (sacroiliitis)
Symptoms of AS
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
who gets AS
M > F
Late adolescence or early adulthood
+ve HLA B27
FH of AS
criteria for AS
- Limited lumbar motion
- Lower back pain for 3 months
- Improved with exercise
- Not relieved by rest - Reduced chest expansion
- Bilateral, Grade 2 to 4, sacroiliitis on X ray
- Unilateral, Grade 3 to 4, sacroiliitis on X ray
extra articular features of AS
Enthesitis, esp Achilles tendonitis and plantar fasciitis Acute Iritis - can lead to blindness Anterior uveitis Osteoporosis Aortic value incompetence Pulmonary apical fibrosis
what is AS also known as
'A' Disease Axial Arthritis Anterior Uveitis Aortic Regurgitation Apical fibrosis Amyloidosis/ Ig A Nephropathy Achilles tendinitis PlAntar Fasciitis
what can be seen on examination of AS
Modified Schober test - lumbar flexion
Poor chest examination
Question mark posture
x-ray of AS
'Bamboo spine' sacroiliitis Syndesmophytes sacroiliac scelerosis vertebral fusion erosions
changes seen on x-ray are irreversible
Tx of AS
1 - NSAIDs + physio, occupational therapy
2 - corticosteroid injections
3- DMD e.g. sulfasalazine
4 - anti TNF e.g. Infliximab
what is Psoriatic Arthritis
Inflammatory arthritis associated with psoriasis,
but patients can have PsA without psoriasis
No Rheumatoid nodules
Rheumatoid factor negative
features of PsA
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)
what are the clinical subgroups of PsA
- 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
diagnosis of PsA
raised inflammatory markers
Hx
negative Rf
x-ray appearance of PsA
Marginal erosions and “whiskering”
“Pencil in cup” deformity
Osteolysis
Enthesitis
Tx of PsA
NSAIDs
Corticosteroids
DMARDS
Anti-TNF
what is reactive arthritis
Infection induced systemic illness characterized primarily by an inflammatory synovitis from which viable microorganisms cannot be cultured
when do symptoms of Reactive arthritis occur
1-4 weeks after infection
who gets reactive arthritis
young adults 20-40 years old
what is the Reiter’s syndrome/Reactive arthritis triad
Urethritis
Conjuntivitis/Uveitis/Iritis
Arthritis
symptoms of reactive arthritis
fever/fatigue/malaise
asymmetrical monoarthritis or oligoarthritis
enthesitis
mucocutaneous lesions
ocular lesions; conjuntivits, iritis
visceral manifestations; mild renal disease, carditis
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
tests to confirm reactive arthritis
elevated ESR, CRP, PV
negative ANA and Rf
cultures (blood/urine/stool)
joint fluid analysis (rule out infection)
Tx of reactive arthritis
90% resolves spontaneously within 6 months NSAIDS Corticosteroids Antibiotics for underlying infection DMARD - if resistant or chronic
what is Enteropathic arthritis
Associated with inflammatory bowel disease eg. Crohn’s, Ulcerative colitis, inflammatory bowel disease
how will Enteropathic arthritis present
Arthritis in several joints, especially the knees, ankles, elbows, and wrists, and sometimes in the spine, hips, or shoulders
what are features of IBD associated with Enteropathic arthritis
Crohn’s suffers will have sacroiliitis (20% of time)
Worsening of symptoms during flare-ups
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
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
Tx for EA
treat IBD analgesia steroids DMD Anti-TNF
what is not recommended in EA
NSAIDS - exacerbate IBD
what is vasculitis
inflammation of blood vessels, often with ischema, necrosis and organ inflammation
can affect any blood vessel
what is primary vasculitis
inflammatory response that targets the vessel walls and has no known cause. Sometimes this is autoimmune.
what is secondary vasculitis
triggered by an infection, a drug, or a toxin or may occur as part of another inflammatory disorder or cancer.
what are the 2 types of large vessel vasculitis
Takayasu arteritis (TA) Giant Cell Arteritis (GCA)
features of TA
affects those under 40 years and is commoner in females. It is much more prevalent in Asian populations.
features of GCA
affects those over 50 years. It typically causes temporal arteritis but the aorta and other large vessels may be affected
what are GCA and TA characterised by
granulomatous infiltration of the walls of the large vessels.
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
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
what is temporal arteritis associated with
PMR
50% with GCA have PMR
15% with PMR develop GCA
symptoms of temporal arteritis
unilateral temporal headache
scalp tenderness
jaw claudication
temporal arteries may be prominent with reduced pulsation
what are complications of temporal arteritis
risk of blindness due to ischaemia of the optic nerve
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
why might a temporal artery biopsy be negative even if temporal arteritis is present
skip lesions
Mx for GCA
40mg - 60mg Prednisolone
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
what is polyarteritis nodosa characterised by
necrotizing inflammatory lesions that affect arteries at vessel bifurcations, resulting in microaneurysm formation and aneurysms.
what is polyarteritis nodosa associated with
Hep B
what are the two sub categories of small vessel vasculitis
ANCA associated vasculitis (AAV)
Non-ANCA associated vasculitis
what is granulomatosis with polyangiitis (GPA)
previous name Wegner’s
Granulomatous inflammation of respiratory tract, small and medium vessels.
what is common in GPA
Necrotising glomerulonephritis common.
what is eosinophilic granulomatosis with polyangiitis (EGPA)
previous name Churg-Strauss
Eosinophilic granulomatous inflammation of respiratory tract, small and medium vessels.
what is EGPA associated with
Associated with asthma
what is microscopic polyangiitis (MPA)
Necrotising vasculitis with few immune deposits.
what is associated with MPA
Necrotising glomerulonephritis
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
x-ray signs of GPA
cavitating nodules in the lungs
what does Necrotising glomerulonephritis manifest as
blood and protein in urine
many features of EGPA are similar to GPA - what is the main difference
presence of late onset asthma and a high eosinophil count
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
what is used to detect Anti-neutrophil cytoplasmic antibodies (ANCAs)
immunofluoresence
what ANCA is associated with GPA
cANCA
what ANCA is associated with MPA and EGPA
pANCA
what else can be used to diagnose small vessel vasculitis
PR3 - raised in GPA
MPO - raised in EGPA, but more so in MPA
management of AAV
early - methotrexate + steroids
late - Cyclophosphamide + steroids
(Rituximab + steroids)
very late - IV immunoglobulins or Rituximab
what is HSP and who gets it
Henoch-Schönlein purpura (HSP)
an acute immunoglobulin A (IgA)–mediated disorder
children aged 2-11
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)
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
how does HSP present
Purpuric rash typically over buttocks and lower limbs Colicky abdominal pain Bloody diarrhoea Joint pain +/- swelling Renal involvement
Mx for HSP
self-limiting
tend to resolve within 8 weeks
what is it essential to do in a case of HSP
perform urinalysis to screen for renal involvement
What test is best for seeing earliest changes in AS
MRI
