Rheumatology (Vasculitis) Flashcards
(74 cards)
1
Q
Vasculitis
A
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.
2
Q
large vessel vasculitis
A
Temporal (giant cell) arteritis
Takayasu’s arteritis
3
Q
Medium vessel vasculitides
A
Polyarteritis nodosa
Kawasaki disease
4
Q
small vessel vasculitis
A
1) ANCA associated vasculitis
2) Immune complex vasculitis
3) Behcets disease
5
Q
ANCA-associated vasculitides
A
- Granulomatosis with polyangiitis (Wegener’s granulomatosis)
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
- Microscopic polyangiitis
6
Q
Immune complex vasculitides
A
- Henoch-Schönlein purpura
- Anti-glomerular basement membrane disease (Goodpasture’s syndrome)
7
Q
causes of vasculitis
A
- 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)
8
Q
presentation of small-vessel vasculitides:
A
- Palpable purpura
- Tiny papules
- Splinter haemorrhages
- Urticaria
- Vesicles
- Rarely livedo reticularis
- Renal involvement
9
Q
presentation of medium-sized vessel vasculitides:
A
- Ulcers
- Digital infarcts
- Nodules
- Livedo reticularis
- Hypertension if there is damage to the renal vessels
- Papular and necrotic skin lesions
10
Q
presentation of large vessel vasculitides:
A
- End-organ damage e.g. TIA/stroke
- Hypertension
- Aneurysms
- Dissection with or without haemorrhage or rupture
11
Q
initial tests for vasculitis
A
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
12
Q
general management of vasculitis
A
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.
13
Q
temporal arteritis is a type of
A
giant cell arteritis (GCA)
14
Q
giant cell arteritis (GCA)
A
inflammation of medium- and large-sized arteries of unknown aetiology which is strongly linked with polymyalgia rheumatica (PMR).
15
Q
complication of temporal arteritis
A
irreversible loss of vision secondary to optic nerve ischaemia, making it a medical emergency.
16
Q
RF for TA
A
- Female sex
- White
- Polymyalgia rheumatica history
17
Q
presentation of temporal arteritis
A
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
18
Q
investigations for temporal arteritis
A
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
19
Q
management of temporal arteritis
A
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
20
Q
Takayasu arteritis
A
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.
21
Q
Takayasu’s arteritis risk factor
A
Family history
Female sex
Asian ethnicity
Age <40 years
22
Q
A
23
Q
Takayasu’s arteritis classical presentation
A
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.
24
Q
takayasu arteritis 2 stages
A
Systemic
Occlusive
25
Takayasu arteritis systemic stage
* Fever, fatigue, weight loss
* Arthralgia and non-specific pains
* Tenderness over the sites of the affected arteries
26
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
27
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
28
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
29
management of Takayasu arteritis
**First line**v glucocorticoids on a tapering regimen + low-dose aspirin + bone protection
**Second line**If control is not achieved, then immunosuppressive therapy may be considered
30
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.
31
Polyarteritis nodosa (PAN) is associated with
Hepatitis B infection
32
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
33
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
34
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
35
Thromboangiitis obliterans, also known as
Buerger’s disease
36
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.
37
Strongest RF for developing thromboangiitis obliterans (Buergers)
SMOKING TOBACCO
MALE
young
38
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**
3. Rest pain that can be eased by hanging the legs over the edge of the bed
4. Superficial thrombophlebitis
5. Raynaud’s phenomenon
39
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
40
criteria used to diagnose thromboangiitis obliterans
Shinoya criteria
often a diagnosis made after excluding other vascular diseases
41
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
42
granulomatosis with polyangiitis (GPA) used to be called
Wegener’s granulomatosis,
43
granulomatosis with polyangiitis (Wegeners)
is a rare ANCA-associated autoimmune vasculitis typically affecting the: **upper and lower respiratory tracts** and the **kidneys.**
44
Risk Factors for Granulomatosis With Polyangiitis
* Family history
* Staphylococcus aureus nasal carriage
* Previous parvovirus infection
45
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
46
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
47
Management of Granulomatosis with Polyangitis
**Corticosteroids** with or without cyclophosphamide, and methotrexate in some scenarios
48
Eosinophilic Granulomatosis with Polyangiitis formerly known as
Churg-Strauss syndrome
49
Eosinophilic Granulomatosis with Polyangiitis (Churg Strauss)
ANCA-associated small to medium sized vessel vasculitis of unknown aetiology associated with astma
50
Eosinophilic Granulomatosis with Polyangiitis classical triad
1) Asthma
2) Granulomatous inflammatiuon
3) Eosiniphilia
51
Presentation of eosinophilic granulomatosis with polyangiitis
Respiratory
- Asthma
- Nasal polyps
- Sinusitis
SKin
- palpable purpura
General symptoms
- fever
- arhtralgia
- weight loss
- chest pain
- palpitations
52
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
53
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
54
management of eosinophilic granulomatosis with polyangiitis
**Corticosteroids** with or without cyclophosphamide, and methotrexate or azathioprine in some scenarios.
55
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.
56
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.
57
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
58
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
59
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
60
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
61
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
62
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
63
Fibromyalgia
is a disorder characterised by widespread pain with tender points at specific sites of unknown aetiology
64
fibromyaglia rf
* Family history of fibromyalgia
* History of other rheumatological conditions
* Age between 20-60 years
* Female sex
65
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
*
66
ACR criteria for fibromyalgia
- history of chronic widespread pain >3 months
- Patients must exhibit >11 of 18 tender points
67
management of fibromyalgia
**First line:** pharmacotherapy + aerobic exercise + cognitive behavioural therapy
* Amitriptyline, duloxetine, pregabalin or gabapentin
**Second line** Analgesics naproxen or tramadol
68
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
69
risk factor for chronic fatigue syndrome
* Female sex
* EBV infection
* COVID-19
* Life stressors
70
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
71
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.
72
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
73
management of ME
1) Cognitive behavioural therapy
2) Management of symptoms:
* SSRIs for depression
* Low-dose tricyclic antidepressants/trazodone for sleep problems
74
