Rheumatology (Vasculitis) Flashcards
Vasculitis
is an umbrella term for a series of conditions represented by inflammation of the blood vessels. Its effects can be transient or cause damage to the vasculature.
It can happen in isolation (primary), or secondary due to infection or in association with another condition such as rheumatoid arthritis.
large vessel vasculitis
Temporal (giant cell) arteritis
Takayasu’s arteritis
Medium vessel vasculitides
Polyarteritis nodosa
Kawasaki disease
small vessel vasculitis
1) ANCA associated vasculitis
2) Immune complex vasculitis
3) Behcets disease
ANCA-associated vasculitides
- Granulomatosis with polyangiitis (Wegener’s granulomatosis)
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
- Microscopic polyangiitis
Immune complex vasculitides
- Henoch-Schönlein purpura
- Anti-glomerular basement membrane disease (Goodpasture’s syndrome)
causes of vasculitis
- Idiopathic – around 50%
- Infection (e.g. Henoch-Schönlein purpura or septic vasculitis)
- Inflammatory disease (e.g. systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), Crohn’s disease, and ulcerative colitis
- Drug-induced (e.g. sulfonamides, penicillins, quinolones, NSAIDs etc.)
- Neoplastic – (e.g. lymphoproliferative disorders or paraproteinaemia)
presentation of small-vessel vasculitides:
- Palpable purpura
- Tiny papules
- Splinter haemorrhages
- Urticaria
- Vesicles
- Rarely livedo reticularis
- Renal involvement
presentation of medium-sized vessel vasculitides:
- Ulcers
- Digital infarcts
- Nodules
- Livedo reticularis
- Hypertension if there is damage to the renal vessels
- Papular and necrotic skin lesions
presentation of large vessel vasculitides:
- End-organ damage e.g. TIA/stroke
- Hypertension
- Aneurysms
- Dissection with or without haemorrhage or rupture
initial tests for vasculitis
C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR):
- Elevated
Anti-neutrophil cytoplasmic autoantibodies (ANCA):
- Associated with certain types of vasculitis, such as granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis.
Urea and electrolytes (U&Es):
- Vasculitis may involve the kidneys leading to renal dysfunction
Urinalysis:
- Vasculitis may involve the kidneys leading to renal dysfunction
Biopsy of the affected tissue:
- Can help with confirming the likely diagnosis
general management of vasculitis
The mainstay of management in vasculitides involves the use of immunosuppression with glucocorticoids (such as prednisolone) with the addition of other immunosuppressants such as cyclophosphamide.
temporal arteritis is a type of
giant cell arteritis (GCA)
giant cell arteritis (GCA)
inflammation of medium- and large-sized arteries of unknown aetiology which is strongly linked with polymyalgia rheumatica (PMR).
complication of temporal arteritis
irreversible loss of vision secondary to optic nerve ischaemia, making it a medical emergency.
RF for TA
- Female sex
- White
- Polymyalgia rheumatica history
presentation of temporal arteritis
Unilateral headache is the primary presenting feature, typically severe and around the temple and forehead. It may be associated with:
- Scalp tenderness (e.g., noticed when brushing the hair)
- Jaw claudication
- Blurred or double vision
- Loss of vision if untreated
Other features
- Symptoms of polymyalgia rheumatica (e.g., shoulder and pelvic girdle pain and stiffness)
- Systemic symptoms (e.g., weight loss, fatigue and low-grade fever)
- Muscle tenderness
- Carpel tunnel syndrome
- Peripheral oedema
investigations for temporal arteritis
Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP):
- Ideally before starting high-dose corticosteroids
- Usually elevated and fall with corticosteroid use
Vascular ultrasonography of the temporal artery:
- Wall thickening which may be non-compressible –halo sign
- Stenosis or occlusion
Temporal artery biopsy:
- Do not perform a biopsy if this delays treatment
- Done if ultrasonography cannot be done or the clinical suspicion is high but ultrasonography is normal
- Shows skip lesions
management of temporal arteritis
Steroids are the mainstay of treatment. They are started immediately, before confirming the diagnosis, to reduce the risk of vision loss. There is usually a rapid and significant response to steroid treatment. Initial treatment is:
- 40-60mg prednisolone daily with no visual symptoms or jaw claudication
- 500mg-1000mg methylprednisolone daily with visual symptoms or jaw claudication
Once the diagnosis is confirmed and the condition is controlled, the steroid dose is slowly weaned over 1-2 years.
Other medications include:
- Aspirin 75mg daily decreases vision loss and strokes
- Proton pump inhibitor (e.g., omeprazole) for gastroprotection while on steroids
- Bisphosphonates and calcium and vitamin D for bone protection while on steroids
Takayasu arteritis
Takayasu’s arteritis is a rare autoimmune inflammatory disorder of unknown aetiology affecting the aorta and its main branches. It predominantly affects young women.
The vascular inflammation can cause stenosis, aneurysm, and occlusion of these arteries.
Takayasu’s arteritis risk factor
Family history
Female sex
Asian ethnicity
Age <40 years
Takayasu’s arteritis classical presentation
The classical presentation of Takayasu’s arteritis is a
1. young,
2. female patient with systemic upset (fever and malaise)
3. with unequal blood pressures in the upper limbs.
takayasu arteritis 2 stages
Systemic
Occlusive
Takayasu arteritis systemic stage
- Fever, fatigue, weight loss
- Arthralgia and non-specific pains
- Tenderness over the sites of the affected arteries
Takayasu arteritis occlusive stage
features present depending on the site of ischaemia secondary to occlusion:
Cardiac:
- Angina
- Congestive cardiac failure
Pulmonary
- Haemoptysis
- Pleuritis
Vascular
- Jaw claudication
- Claudication of the extremities
- Back pain due to the involvement of the aorta
- Hypertension
Gastrointestinal
- Abdominal pain due to bowel ischaemia/infarction
Renal
- Haematuria
Dermatological
- Erythema multiforme
Neurological
- Dizziness
- Headaches
- Transient ischaemic attacks (TIAS)
- Visual disturbances
- Seizures
- Strokes
Takayasu arteritis signs on Examination
- Systolic blood pressure difference of >10mmHg between the arms
- Peripheral pulses may be weak or absent
A good way of remembering this sign is the pun “can’t Takaya pulse” - High blood pressures due to renal artery involvement
- Arterial bruits
- Anaemia
- Muscle wasting
takayasu arteritis investigations
Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP):
- Usually elevated in active disease
CT angiography (CTA) – required for diagnosis:
- Shows narrowing/occlusion of affected vessels
- Aortic aneurysms may be seen
- Thickening of blood vessel walls may be seen
Magnetic resonance angiography (MRA):
- Shows soft tissue as well as arterial walls
- Can identify active and inactive disease
management of Takayasu arteritis
First linev glucocorticoids on a tapering regimen + low-dose aspirin + bone protection
Second lineIf control is not achieved, then immunosuppressive therapy may be considered
presentation of Buergers
1. Ischaemia of the extremities:
- Cold extremities
- Changes in skin colour of extremities
- Pallor of extremities
- Paraesthesia in extremities
- Absent or weak distal pulses
- Positive Allen’s test
2. Claudication
- Rest pain that can be eased by hanging the legs over the edge of the bed
- Superficial thrombophlebitis
- Raynaud’s phenomenon
presentation of HSP
1) Palpable non-blanching purpura over the back of the legs, buttocks, and extensor surfaces of the arms
2) Abdominal pain
3) Joint pain
4) Signs of renal disease and IgA nephropathy:
- Microscopic haematuria
- Proteinuria
- Nephrotic syndrome
- Renal failure
ACR criteria for fibromyalgia
- history of chronic widespread pain >3 months
- Patients must exhibit >11 of 18 tender points
