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
Polyarteritis nodosa (PAN)
is a rare vasculitis affecting medium-sized arteries with necrotising inflammation leading to aneurysm formation.
It can affect any organ but it spares the pulmonary and glomerular arteries. The reason for this is unknown.
Polyarteritis nodosa (PAN) is associated with
Hepatitis B infection
presentation of PAN
Non specific
- Fever
- Weight loss
- Headache
- Myalgia
- Hypertension
Other organ specific symptoms
Dermatological
- Livedo reticularis
- Purpura
Renal
- Hypertension
- AKI
- Haematuria
GI
- Postprandial abdominal pain due to bowel ischaemia
investigations for PAN
C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR):
- Elevated
Hepatitis B surface antigen (HBsAg):
- Positive in 30% of patients with PAN
Perinuclear-antineutrophil cytoplasmic antibodies (p-ANCA):
- Negative, if these are positive, these suggest another form of vasculitis
Digital subtraction angiography (DSA), which may show:
- Microaneurysms
- Focal occlusive lesions in medium-sized vessels
Echocardiogram:
- To exclude alternate diagnoses such as endocarditis
Small artery biopsy may be considered:
- This may show necrotising inflammation
PAN management
Management involves using oral corticosteroids on a tapering regime with or without disease-modifying anti-rheumatic drugs (DMARDs)
Avoidance of hepatitis B infection and immunisation may reduce PAN associated with hepatitis B but does not eliminate PAN altogether
Thromboangiitis obliterans, also known as
Buerger’s disease
Buerger’s disease
an inflammatory vasculitis leading to thrombosis of medium- and small-sized vessels. The thrombosis may lead to arterial ischaemia in the distal extremities and superficial thrombophlebitis, possibly leading to gangrene and ulceration.
Strongest RF for developing thromboangiitis obliterans (Buergers)
SMOKING TOBACCO
MALE
young
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
investigations for Buergers
Full blood count (FBC):
- To screen for myeloproliferative diseases
Fasting glucose:
- To screen for diabetes
C-reactive protein(CRP) and erythrocyte sedimentation rate (ESR):
- Normal but may be elevated if gangrene is present
Coagulation assay and thrombophilia screen:
- To screen for hypercoagulable states
Urea and electrolytes (U&Es):
- Normal – kidney dysfunction suggests an autoimmune disease
Rheumatological autoantibodies – to screen for rheumatological diseases:
- Anti-nuclear (ANA)
- Rheumatoid factor
- Anti-nucleophilic cytoplasmic antibody (ANCA)
- Anti-centromere antibodies
- Topoisomerase I antibodies (Scl-70)
Echocardiogram:
- To identify potential embolic sources
Arterial duplex:
- Corkscrew-shaped collateral vessels are present
CT angiography/MR angiography:
- If the arterial duplex is insufficient
Corkscrew-shaped collateral vessels are present
Biopsy may be considered:
- This should be avoided in ischaemic tissue
- This can show inflammatory thrombosis with sparing of the internal elastic lamina
Diagnosis
criteria used to diagnose thromboangiitis obliterans
Shinoya criteria
often a diagnosis made after excluding other vascular diseases
management of Buergers
- Immediate smoking cessation
- If patients have critical ischaemia/severe claudication – surgical revascularisation
- Dry gangrenous limbs should be reviewed monthly to monitor for infection
granulomatosis with polyangiitis (GPA) used to be called
Wegener’s granulomatosis,
granulomatosis with polyangiitis (Wegeners)
is a rare ANCA-associated autoimmune vasculitis typically affecting the: upper and lower respiratory tracts and the kidneys.
Risk Factors for Granulomatosis With Polyangiitis
- Family history
- Staphylococcus aureus nasal carriage
- Previous parvovirus infection
Granulomatosis With Polyangiitis presentation
Upper respiratory tract
- Epistaxis
- Sinusitis
- Saddle shaped nose (secondary to destruction of nasal cartilage)
Lower resp tract
- Cough
- Haemoptysis
- SoB
Renal
- glomerulonephritis
General
- fatigue
- malaise
- fever
- night sweats
- anorexia
- weight loss
investigations for Granulomatosis With Polyangiitis (Wegeners)
Urinalysis and microscopy:
- Haematuria
- Proteinuria
CT chest:
- Lung nodules which may cavitate may be seen
- Infiltrates may be seen
**Anti-neutrophil cytoplasmic antibodies (ANCA) **– pANCA and cANCA:
- c-ANCA is associated with granulomatosis with polyangiitis
Urea and electrolytes (U&Es):
- May show renal dysfunction
C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR)
- May be elevated
Renal biopsy:
- Confirms glomerulonephritis
Management of Granulomatosis with Polyangitis
Corticosteroids with or without cyclophosphamide, and methotrexate in some scenarios
Eosinophilic Granulomatosis with Polyangiitis formerly known as
Churg-Strauss syndrome
Eosinophilic Granulomatosis with Polyangiitis (Churg Strauss)
ANCA-associated small to medium sized vessel vasculitis of unknown aetiology associated with astma
Eosinophilic Granulomatosis with Polyangiitis classical triad
1) Asthma
2) Granulomatous inflammatiuon
3) Eosiniphilia
Presentation of eosinophilic granulomatosis with polyangiitis
Respiratory
- Asthma
- Nasal polyps
- Sinusitis
SKin
- palpable purpura
General symptoms
- fever
- arhtralgia
- weight loss
- chest pain
- palpitations
Eosinophilic granulomatosis with polyangiits vs Granulomatosis polyangiitis (GPA)
Granulomatosis with polyangiitis (GPA)
- GPA does not have asthma
- GPA does not have eosinophilia
- GPA is associated with cANCA, EGPA is associated with pANCA
- Peripheral neuropathy more common in EGPA
investigations for eosinophilic granulomatosis
Full blood count:
- Eosinophils are raised
Anti-neutrophil cytoplasmic antibodies (ANCA):
- pANCA is associated with EGPA
Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP):
- Usually raised during active vasculitis
Urea and electrolytes (U&Es):
- To screen for glomerulonephritis
Urinalysis:
- To screen for glomerulonephritis
Pulmonary function tests:
- To assess for asthma, which is common in EGPA
Chest x-ray or CT scan:
- This may show interstitial infiltrates or nodules
management of eosinophilic granulomatosis with polyangiitis
Corticosteroids with or without cyclophosphamide, and methotrexate or azathioprine in some scenarios.
Henoch-Schönlein Purpura (HSP)
IgA vasculitis is an IgA-mediated autoimmune hypersensitivity vasculitis of childhood whose main features are skin purpura, arthritis, abdominal pain, and nephritis. Its aetiology is unknown.
HSP pathophysiology
IgA immune complexes are involved in IgA vasculitis, depositing in the small blood vessels of the skin, joints, kidneys, and gastrointestinal tract. IgA vasculitis has some overlap with IgA nephropathy.
IgA vasculitis is often seen in children following an infection.
HSP risk factors
- Prior infection (such as an upper respiratory tract infection or less commonly, a gastrointestinal infection)
- Age 2-10 years
- Male sex
- History of allergy
HSP RF
- Prior infection (such as an upper respiratory tract infection or less commonly, a gastrointestinal infection)
- Age 2-10 years
- Male sex
- History of allergy
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
HSP vs Idiopathic thrombocytopenic purpura (ITP)
- No preceding infection
- No arthralgia
- No abdominal pain
- Platelet levels are low in ITP but normal in IgA vasculitis
investigations for HSP
Blood pressure:
- Assesses for renal involvement – blood pressure is raised if this is true
- Monitored for at least 6 months even if initial results are normal
Urinalysis:
- Assesses for renal involvement
- May show haematuria or proteinuria or casts
Urea and electrolytes (U&Es):
- Assesses for renal involvement
Skin biopsy
- Shows leukocytoclastic vasculitis with IgA deposition
Renal biopsy – required to diagnose IgA nephropathy:
Shows mesangial IgA deposition
management of HSP
Most cases of IgA vasculitis are self-limiting. Patients with mild symptoms without renal involvement can have supportive treatment e.g. pain relief and rehydration
- If there is renal involvement, corticosteroids with immunosuppressants such as azathioprine, and mycophenolate mofetil are used.
- If there is severe nephritis, IV cyclophosphamide and oral or IV corticosteroids are used
Fibromyalgia
is a disorder characterised by widespread pain with tender points at specific sites of unknown aetiology
fibromyaglia rf
- Family history of fibromyalgia
- History of other rheumatological conditions
- Age between 20-60 years
- Female sex
presentation of fibromyalgia
- Chronic, widespread body pain
- Diffuse tenderness to palpation on physical examination without evidence of systemic disease
- Fatigue unrelieved by rest
- Sleep disturbances
- Mood disturbances
- Cognitive dysfunction
- Headaches
- Numbness/tingling sensations
- Stiffness
- Sensitivity to sensory stimuli such as bright lights, strong odours, or noises
*
ACR criteria for fibromyalgia
- history of chronic widespread pain >3 months
- Patients must exhibit >11 of 18 tender points
management of fibromyalgia
First line: pharmacotherapy + aerobic exercise + cognitive behavioural therapy
- Amitriptyline, duloxetine, pregabalin or gabapentin
Second line Analgesics naproxen or tramadol
Chronic fatigue syndrome (CFS)
also known as myalgic encephalomyelitis (ME) is a complex chronic medical condition of unknown aetiology affecting multiple body systems. It presents with severely debilitating physical and mental fatigue.
Around 50-80% of patients with CFS/ME start suddenly with a flu-like illness
risk factor for chronic fatigue syndrome
- Female sex
- EBV infection
- COVID-19
- Life stressors
presentation of ME
1) Flu-like symptoms (malaise, myalgia)
2) Persistent disabling fatigue
- This is not relieved by sleep, rest, or reduced activity
- This is usually over 3 months
- Fatigue may show remissions
- Fatigue must be moderate-severe and persistent >50% of the time
3) Post-exertional malaise or fatigue
4) Short-term memory impairment
5) Concentration impairment
6) Sore throat
7) Generalised arthralgia without inflammation
8) Headache or migraine onset after fatigue
9) Sleep problems
- Unrefreshing sleep
- Insomnia/hypersomnia
- Disturbed sleep-wake cycle
10) Dizziness/light-headedness/malaise after standing up from sitting
11) Diffuse muscular pain
12) Tender but non-enlarged lymph nodes may be felt, but this is uncommon
investigations for ME
NICE recommend carrying out a large panel of blood tests to exclude other pathologies. These involve FBC, ESR, CRP, glucose, TFTs, ANA, RF, HIV, U&Es, calcium, CK, coeliac screening, ferritin etc.
diagnosis of ME
Suspect diagnosis if the patient has all four of the following features for a minimum of 6 weeks in adults and 4 weeks in children and young people:
- Post-exertional malaise
- Debilitating fatigue
- Cognitive difficulties
- Unrefreshing sleep/sleep disturbance
management of ME
1) Cognitive behavioural therapy
2) Management of symptoms:
- SSRIs for depression
- Low-dose tricyclic antidepressants/trazodone for sleep problems
