Vasculitis Flashcards
What is polymyalgia rheumatic
Common chronic inflammatory condition of unknown cause that affects mainly elderly individuals
Polymyalgia rheumatica mainly affects which age group
those over 50
Polymyagia rheumatic is associated with which condition
Giant cell arteritis
-40% with GCA develop PR
-15% PR develop GCA
Risk factors for polymyalgia rheumatica
Over 50
Northern European descent
Which joints are commonly affected by polymyalgia rheumatica
Shoulder
Hips
Symptoms of polymyagia rheumatica
Symmetrical myalgia of hip and shoulder girdle
Stiffness > 45m
Pain worse with movement
Low fever
Reduced appetite
Weight loss
Malaise
What can relief the pain caused by polymyalgia rheumatica
Oral steroids
Clinical signs of polymyalgia rheumatic
Limited joint movement due to pain
Normal muscle strength
Tenderness
Dermatomyositis also causes myalgia. What are the differences between polymyalgia rheumatica and dermatomyositis
PR does not cause muscle weakness whereas dermatomyositis does
PR causes pain whereas dermatomyositis doesn’t really cause pain
investigations for polymyagia rheumatica
Clinical
Bloods - ESR and CRP (raised)
therapeutic trial of steroids
Management of polymyalgia rheumatica
investigations before starting steroids
Oral steroid therapy- prednisolone
Describe the steroid therapy for polymyalgia rheumatica
Does not need to be started right away - do check ups before starting
Start prednisolone at 15
Gradually decrease the dose
Symptoms should resolve
What investigations need to be done before starting steroids
Screen for
- diabetes
- peptic ulcers
- osteoporosis (DEXA scan)
- mental health history
- hypertension
2 types of large vessel vasculitis
Takayasu’s arteritis
Giant cell arteritis
Which vessels are affected by Takayasu’s arteritis
Aorta
Main branches of the aorta
Risk factors for takayasu’s arteritis
Female
<40 years old
Asian
What can vascular inflammation lead to
stenosis
occlusion
aneurysm
Symptoms of takayasu’s arteritis
Systemic upset - mild fever / malaise / weight loss
Arthralgia
Night sweats
Claudication in upper and lower limbs
Skin nodules
Clinical signs of takayasu’s arteritis
Absent upper extremity pulse
Difference in blood pressure between the extremities
Bruit
Aortic regurgitaiton
What is bruit
audible vascular sound associated with turbulent blood flow
What causes bruit in takayasu’s arteritis
vascular stenosis / aneurysms
Investigations for takayasu’s arteritis
Bloods - inflammatory markers raised
MR Angiogram
Management for takayasu’s arteritis
Steroids
Which arteries are most commonly affected by giant cell arteritis (temporal arteritis)
Temporal arteries - arteries around the temples
Risk factors for GCA
> 50 years, most commonly late 60+
Polymyalgia rheumatica
Symptoms of GCA
temporal headache
Jaw claudication - pain on chewing food
Amaurosis fugax
Visual disturbances
Thickened temporal artery
Scalp tenderness
polymyagia rheumatica symptoms
What is amaurosis fugax
transient loss of vision in one or both eyes
“dark curtain descending”
Clinical signs of GCA
Non-pulsatile, thickened temporal arteries
Complications of GCA
Permanent blindness
Stroke
Investigations for GCA
Bloods - ESR
Temporal artery US
Temporal artery biopsy
CT angiogram / MR angiogram if still unsure
When should you suspect GCA in a patient
Patient over 50 years old, new onset of headache with raised inflammatory markers
What is important to be measured in bloods for GCA
ESR
What can be seen on temporal artery biopsy in GCA
Transmural inflammation of the layers of arteries
Patchy infiltration by giant cells, lymphocytes
Thickened vessel wall
Why does temporal artery biopsy have low sensitivity but high specificity for GCA
Low sensitivity due to skip lesions
= biopsy may be taken from a normal segment so a negative biopsy does not rule out the disease
Management of GCA
Immediate Oral steroids - prednisolone
Gradually reduce steroids dosage
Steroid sparing agents if cannot reduce steroid dosage
What are the steroid sparing agents
Methotrexate
Azathioprine
Leflunamide
Why should you reduce the steroid dosage gradually instead of quickly
Because symptoms can relapse
Differences between takayasu’s arteritis and GCA
Takayasu arteritis mainly affects the aorta whereas GCA affects temporal arteries
Takayasu arteritis mainly affects younger patients whereas GCA affects older patients
Common biopsy finding between takayasu and GCA
skip lesions
What is ANCA associated small vessel vasculitis
Group of autoimmune inflammation conditions of small to medium vessels associated with anti-neutrophil cytoplasmic antibodies (ANCA)
What are the conditions part of ANCA vasculitis
Granulomatosis with polyangiitis
Eosinophilic granulomatosis with polyangiitis
Microscopic polyangiitis
What is granulomatosis with polyangiitis
Granulomatous inflammation affecting small and medium sized vessels in the upper and lower resp tract, eyes, kidneys
Classic triad of granulomatosis with polyangiitis
Upper resp tract involvement
Lower resp tract involvement
Pauci-immune glomerulonephritis
What are the signs of URT involvement in granulomatosis with polyangiitis
Chronic sinusitis
Epistaxis
Saddle nose deformity
Otitis media
Mouth ulcers
Sensorineural deafness
What are the signs of LRT involvement in granulomatosis with polyangiitis
Haemoptysis
Cough
Pleuritis
Alveolar haemorrhage
Bilateral cavitating nodules on CXR
What is pauci immune glomerulonephritis
Rapidly progressive condition leading to renal failure within days, strongly associated with ANCA
Presentation of pauci-immune glomerulonephritis
Proteinuria
Haematuria
Frothy urine due to proteinuria
What are the ocular involvement of granulomatosis with polyangiitis
Conjunctivitis
Optic nerve vasculitis
Retinal artery occlusion
Uveitis
Proptosis
What are the nervous system manifestations of GPA
mononeuritis multiplex
polyneuropathy
What is mononeuritis multiplex
peripheral neuropathy of a single nerve at least 2 different areas of peripheral nervous system
Difference between mono neuritis multiplex and polyneuropathy
Polyneuropathy is damage to multiple nerves
What is eosinophilic granulomatosis with polyangiitis
granulomatous inflammation mostly affecting small and medium vessels in resp tract and skin
Diagnostic criteria of EGPA
Late onset adult asthma
Eosinophilia
Paranasal sinusitis
Pulmonary infiltrates
Histological confirmation of vasculitis
Neuropathy - mononeuritis multiplex / polyneuropathy
Difference between granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis
EGPA has high eosinophil count
EGPA causes late onset adult asthma whereas GPA doesn’t
What is microscopic polyangiitis
Small to medium vessel vasculitis characterised by renal, pulmonary disease and pANCA
Which symptom is very common in microscopic polyangiitis
Necrotising glomerulonephritis
Symptoms of microscopic polyangiitis
Necrotising glomerulonephritis
Palpable purpura
Neuropathy
Alveolar haemorrhage
Haemoptysis
Investigations for ANCA- associated polyangiitis
Bloods
Immunofluorescence
Biopsy of affected areas
What may blood tests show in ANCA-associated polyangiitis
Raised inflammatory markers
Anaemia
Low complement level during active disease
What does immunofluorescence look at in ANCA associated polyangiitis
ANCA
PR3
MPO
What are ANCA
autoantibodies against antigens in the cytoplasm of neutrophils
ANCA present in GPA
cANCA
ANCA present in EGPA
pANCA
ANCA present in MPA
pANCA
Can ANCA be diagnostic for ANCA associated polyangiitis
No, because a proportion of patients may be ANCA negative
and the level of those autoantibodies vary with disease level
Management of ANCA associated polyangiitis depends on
involvement of organs
- localised
- early systemic
- generalised
- systemic
- unresponsive
Localised ANCA associated polyangiitis =
only upper +/- lower resp tract involved
generalised ANCA associated polyangiitis =
Early renal involvement
Systemic ANCA associated polyangiitis =
Late renal involvement
Vital organ failure
Management of localised and early systemic ANCA associated polyangiitis
Methotrexate + steroids
Management of generalised ANCA associated polyangiitis
Cyclophosphamide + steroids
Azathioprine for maintenance
Management of systemic ANCA associated polyangiitis
Cyclophosphamide + steroids
Plasma exchange if creatine > 500
Azathioprine for maintenance
Management of refractory ANCA associated polyangiitis
IV immunoglobulins
Rituximab
What is Henoch-Schonlein Purpura
IgA mediated generalised vasculitis affecting small vessels of the skin, GI, kidneys, joints
Which age group is most commonly affected by Henoch-Schonlein Purpura
2-11 years old
Patients with Henoch-Schonlein Purpura usually had which preceding infections
Group A strep infection
viral URTI
pharyngeal infection
GI infection
Symptoms of Henoch-Schonlein Purpura
Purpuric rash over buttocks and lower limbs
Abdominal pain
Bloody diarrhea
Arthralgia
Nephritis
Investigations for Henoch Schonlein Purpura
Clinical
Urine dip stick
Bloods
Management for Henoch Schonlein Purpura
Self limiting
Regular urine dips for 12 months to check for renal impairment
