Vasculitis Flashcards
What is vasculitis?
Vasculitis is inflammation of the blood vessel, often leading to ischaemia and necrosis of the tissue
What are the 3 main sub-types of vasculitis?
Large vessel
Medium vessel
Small vessel
What are the 3 sub-groups of small-vessel vasculitis?
Immune complex small-vessel vasculitis
Anti-GBM disease
ANCA-associated small-vessel vasculitis
What are some examples of large-vessel vasculitides?
Takayasu’s arteritis
Giant cell arteritis
What are some examples of medium-vessel vasculitides?
Polyarteritis nodosa
Kawasaki disease
What are some examples of immune complex small-vessel vasculitides?
Henoch-Schönlein
Hypocomplementemic urticarial vasculitis (Anti-C1q vasculitis)
What are some examples of ANCA-associated small-vessel vasculitides?
Microscopic polyangiitis
Granulomatosis with polyangiitis (Wegener)
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
What are some factors that can cause secondary vasculitis?
Infection
Drugs
Toxins
Other inflammatory disorders
Cancer
What are some common features of all vasculitides?
Fever
Malaise
Weight loss
Fatigue
What is Takayasu’s arteritis?
This is a large cell vasculitis, most commonly affecting the aortic arch
Who is most at risk of Takayasu’s arteritis?
This is most commonly found in those under 40 years and in females
It is also more prevalent in Asian populations
What are some non-conventional features of Takayasu’s arteritis?
Claudication in arms
Blood pressure differences in extremities
Bruit (Most commonly in carotids)
What investigations are required in Takayasu’s arteritis?
Blood tests - Raised inflammatory markers
CT or MR angiography - Stenosis and vessel wall thickening
How is Takayasu’s arteritis usually managed?
Initial management of large vessel vasculitis is 40-60mg prednisolone, with steroid sparing agents given in Takayasu’s arteritis
These include leflunamide and methotrexate
Tocilizumab is a biologic that can be given
What is another name given to giant cell arteritis?
temporal arteritis
What is giant cell arteritis?
A type of systemic vasculitis affecting the large and medium arteries
Who is most at risk of Giant cell arteritis?
It is more commonly found in those over 50, and has a strong link with polymyalgia rheumatica and white ethnicity
What are some un-conventional symptoms of giant cell arteritis?
- Unilateral acute temporal headache (Temporal artery)
- Scalp tenderness
- Temporary visual disturbances (Ophthalmic artery)
- Blindness (Ophthalmic artery)
- Jaw claudication
What are some features of giant cell arteritis not affecting the head?
Carpel tunnel syndrome
Peripheral oedema
What is the main clinical sign of giant cell arteritis?
Inflamed temporal artery
What investigations are required in giant cell arteritis?
Bloods - Raised ESR, CRP
USS
Temporal artery biopsy
PET, CT or CT angiogram
Why might a temporal artery biopsy be negative in giant cell arteritis?
Giant cell arteritis causes skip lesions
How is giant cell arteritis managed?
Initial management of large vessel vasculitis is 40-60mg prednisolone, with steroid sparing agents considered in GCA
These include leflunamide and methotrexate
This dose is then reduced over 18 -24 months
Tocilizumab is a biologic that can be given
What medication is given in giant cell arteritis, in cases of jaw claudication or visual symptoms?
In cases of visual symptoms or jaw claudication, 500-1000mg of methylprednisolone is given daily
What drugs can be given to counteract steroid side effects in giant cell arteritis?
PPIs, bisphosphonates, calcium and vitamin D can be given to counteract side effects of steroids (Gastric ulceration and bone destruction)
What is polyarteritis nodosa?
This is a systemic medium vessel condition occurring mostly in middle age to older patients
What are some causes of secondary polyarteritis nodosa?
Hepatitis B
Hepatitis C
What are some renal symptoms of polyarteritis nodosa?
Renal hypertension
Renal function impairment
What are some cutaneous presentations of polyarteritis nodosa?
Purpura
Livido reticularis
Ulcers
What is a common musculoskeletal symptoms of polyarteritis nodosa?
Muscle pain
What are some neurological presentations of polyarteritis nodosa?
Mononeuritis
Polyneuropathy
What are some gastrointestinal presentations of polyarteritis nodosa?
Abdominal pain
Diarrhoea (±blood)
Bowel perforation
What is livido reticularis?
A transient or persistent, blotchy, reddish-blue to purple, net-like cyanotic pattern that occurs in polyarteritis nodosa
What investigations are required in polyarteritis nodosa?
Biopsy of affected tissue
Angiogram
There are no specific blood tests
How is non-organ threatening polyarteritis nodosa treated?
In cases of non-organ threatening disease steroids are given with an immunosuppressant, such as azathioprine, methotrexate or mycophenolate
How is organ threatening polyarterits nodosa treated?
Steroids are given with cyclophosphamide
What is Kawasaki disease?
This is a very rare, medium cell vasculitis, also known as mucocutaneous lymph node syndrome, occurring mostly in children under 5
Who is most at risk of Kawasaki disease?
Children under 5
Children from Northeastern Asia, especially Japan and Korea
How does Kawasaki disease present?
Initially it presents as a normal infection, however, it then goes on to cause symptoms such as:
- High fever
- Widespread rash
- Bilateral conjunctivitis
- Strawberry tongue
- Coronary artery aneurysm
What are the 2 main treatments of Kawasaki disease?
Aspirin
IV immunoglobulin
What is used in Kawasaki disease that does not respond to IV immunoglobulin therapy?
Corticosteroids
What are some general MSK and cutaneous symptoms of small vessel vasculitides?
Palpable purpura
Cutaneous ulcers
Arthrlagia
Myalgia/Myositis
What are some common ENT presentations in small vessel vasculitides?
Sinusitis
Nasal crusting
Epistaxes
Mouth ulceration
Otitis media and deafness
Saddle nose (Cartilage damage)
Sub-glottic inflammation
What are some respiratory symptoms of small vessel vasculitides?
Cough
haemoptysis
Pulmonary infiltrates
Diffuse alveolar haemorrhage
Cavitating nodules
Asthma
What are some ocular symptoms of small vessel vasculitides?
Conjunctivitis
Episcleritis
Uveitis
Optic nerve artery vasculitis
Retinal artery occlusion
Proptosis-pseudotumour
What are some neurological symptoms of small vessel vasculitides?
mononeuritis multiplex
Sensorimotor polyneuropathy
Cranial nerve palsy
What is ANCA?
Anti-Neutrophil Cytoplasmic Antibodies
A form of auto-antibodies against the cytoplasms of neutrophils within the blood vessels
What are the 2 forms of ANCA antibodies?
P-ANCA - MPO antibody - Against nucleus cytoplasm (P = Perinuclear)
C-ANCA - PR2 antibody - Against all cytoplasm (C= Cytoplasmic)
What is Granulomatosis with polyangiitis (Wegener’s)
This is a vasculitis characterised by granulomatous inflammation, affecting the small and medium sized vessels of the lungs, eyes or kidneys
Who is most at risk of developing GPA?
Northern Europeans
Males (Slightly)
35-55 years old
What types of symptoms will occur in GPA?
Respiratory symptoms
Necrotising glomerulonephritis
Granulomatous inflammation
Nasal symptoms
Ocular features
What respiratory symptoms are most common in GPA?
Upper airways disease
Pulmonary parenchymal disease
What investigations are required in small vessel vasculitides?
Bloods
urine dipstick testing
Immunofluorescence
Biopsy
What will blood testing show in small vessel vasculitides?
Raised ESR, PV and CRP
Anaemia in chronic disease
Complement activation
Abnormal U+E in renal involvement
What will be seen on urine dipstick testing in small vessel vasculitides?
Haematuria
What is the predominant antibody in GPA?
P-ANCA
What further tests can be performed in small vessel vasculitis to show organ involvement?
CT chest scan
Nerve conduction testing
Muscle MRI
Tissue biopsy
How is remission induced in organ threatening GPA?
- Glucocorticoids + Rituximab/Cyclophosphamide
- Avocopan - C5 inhibitor may be added
- Plasma exchange when creatinine >300umol
How is remission maintained in organ threatening GPA?
- Tituximab or azathioprine/methotrexate
- Mycophenolate or leflunamide
How is resistant disease managed in organ threatening GPA?
- Rituximab + Cyclophosphamide
- IV immunoglobulin therapy
How is remission induced in non-organ threatening GPA?
Glucocorticoids + Rituximab/Methotrexate/Mycophenolate
How is remission maintained in non-organ threatening GPA?
- Rituximab/Azathioprine/Methotrexate
- Mycophenolate or Leflunamide
What is microscopic polyangiitis?
Microscopic polyangiitis is a necrotising vasculitis of the small vessels, with few immune deposits, typically with pulmonary, renal and skin involvement
What are the most common presentations of microscopic polyangiitis?
Glomerulonephritis
Nervous system dysfunction
Diffuse alveolar haemorrhage
What is the predominant antibody found in microscopic polyangiitis?
C-ANCA
How is remission induced in organ threatening microscopic polyangiitis?
- Glucocorticoids + Rituximab/Cyclophosphamide
- Avocopan - C5 inhibitor may be added
- Plasma exchange when creatinine >300umol
How is remission maintained in organ threatening microscopic polyangiitis?
- Tituximab or azathioprine/methotrexate
- Mycophenolate or leflunamide
How is resistant organ threatening microscopic polyangiitis managed?
- Rituximab + Cyclophosphamide
- IV immunoglobulin therapy
How is remission induced in non-organ threatening microscopic polyangiitis?
Glucocorticoids + Rituximab/Methotrexate/Mycophenolate
How is remission maintained in non-organ threatening microscopic polyangiitis?
- Rituximab/Azathioprine/Methotrexate
- Mycophenolate or Leflunamide
What is eosinophilic granulomatosis with polyangiitis (Churg-Strauss)?
Eosinophilic granulomatosis with polyangiitis is an aesinophilic inflammation affecting small and medium sized vessels, most commonly in the respiratory tract and skin
How will EGPA most commonly present?
- Nervous system dysfunction
- Skin irritation
- Late onset asthma
- Granulomatous inflammation
How can EGPA be distinguished from GPA?
EGPA will have a high eosinophil count and is a C-ANCA predominant condition
What is the predominant antibody in EGPA?
C-ANCA
How is remission induced in organ threatening EGPA?
Steroids + Cyclophosphamide
How is remission maintained in organ threatening EGPA?
Azathioprine/Methotrexate/Mepolizumab/ Rituximab
How is remission induced in non-organ threatening EGPA?
Steroids
How is resistant, non-organ threatening EGPA managed?
Mepolizumab - IL5 inhibitor
What is Henoch-Schönlein purpura (IgA Vasculitis)?
This is an acute IgA-mediated generalised vasculitis involving the small vessels of the skin, the GI tract, the kidneys, the joints, and rarely, the lungs and rarely, the CNS
Who is most likely to get Henoch-Schönlein purpura?
HSP occurs mostly in children aged 2-11
What is thought to cause HSP?
It is thought to be caused by a preceding URT infection, pharyngeal infection or GI infection, with group A streptococcus being the most common causative infection, occurring 1-3 weeks before HSP develops
How does HSP usually present?
- Purpuric rash, typically on the buttocks and lower limbs
- Colicky abdominal pain
- Bloody diarrhoea
- Joint pain ± Swelling
- Renal involvement (50%)
How is HSP usually managed?
HSP is usually self limiting and symptoms tend to resolve within 8 weeks, however, relapses may occur for months to years
How is resistant HSP treated?
Steroids and immunosuppressive drugs
urinalysis for renal involvement
