Vasculitis Flashcards
What is vasculitis?
Inflammation of blood vessels, often with ischaemia, necrosis and organ inflammation (can affect any vessel)
What do the manifestations of vasculitis depend on?
Size and location of involved vessel, and the degree of organ dysfunction and inflammation
What is the prognosis of untreated small vessel vasculitis after two years?
90%
What causes primary vasculitis?
Results from inflammatory response that targets vessel walls and has no known cause, sometimes auto-immune
What causes secondary vasculitis?
May be triggered by infection, drug or toxin, or may occur as part of another inflammatory disorder or cancer
What is the pathogenesis of vasculitis?
Activated dendritic cells express receptors and release inflammatory cytokines that promote T cell activation and vascular inflammation, causing granuloma formation and macrophage activation
What are the common symptoms for all types of vasculitis?
Fever, malaise, weight loss and fatigue
What are the two main causes of large vessel vasculitis?
Takayasu arteritis (TA) and giant cell arteritis (GCA)
What is the epidemiology of Takayasu arteritis?
People < 40 years old, commoner in female, more prevalent in Asian populations
What is the epidemiology of giant cell arteritis?
People > 50 years old, typically cause temporal arteritis but aorta and other large vessels may be affected, associated with polymyalgia rheumatica
What is the presentation of Takayasu arteritis?
Claudication
Bruits = most commonly in the carotid arteries
Blood pressure difference at extremities = pulseless disease
What are the presentations of giant cell arteritis?
Unilateral headache, scalp tenderness and jaw claudication
Temporal arteries prominent with reduced pulsation
Risk of blindness due to ischaemia of optic nerve
What investigations should be done for large vessel vasculitis?
ESR, PV and CRP (raised)
Temporal artery biopsy = skip lesions occur so biopsy may be negative
MR/CT angiogram, USS and PET CT
How should large vessel vasculitis be treated?
40-60mg prednisolone, steroid sparing agent may be used (e.g methotrexate), tocilizumab
What are the two main middle vessel vasculitis?
Polyarteritis nodosa = typically affects muscular arteries, causing aneurysms
Kawasaki’s disease = affects children < 5, febrile exanthematous disease typically affecting the coronary arteries
What are the two types of small vessel vasculitis?
ANCA associated vasculitis (AAV) and ANCA negative
What are some examples of AAVs?
Granulomatosis with polyangiitis (GPA), Eosinophilic granulomatosis with polyangiitis (EGPA), Microscopic polyangiitis (MPA)
What occurs in GPA?
Granulomatous inflammation of the respiratory tract, necrotising glomerulonephritis common
What occurs in EGPA?
Eosinophilic granulomatous inflammation of the respiratory tract, associated with asthma
What occurs in MPA?
Necrotising vasculitis with few immune deposits, necrotising glomerulonephritis very common
What is the epidemiology of GPA?
More common in northern Europeans and men, typically patients aged 35-55, constitutional symptoms and arthralgia common, diagnosed if 2/4 of American College of Rheumatology 1990 criteria are present
What are some ENT features of GPA?
Sinusitis, nasal crusting, epistaxis, mouth ulcers, otitis media and deafness, sensorineural deafness, saddle nose (due to cartilage ischaemia)
What are some respiratory symptoms of GPA?
Pulmonary infiltrates, cough, haemoptysis, diffuse alveolar haemorrhage, cavitating nodules on CXR
What are some cutaneous and renal manifestations of GPA?
Cutaneous = palpable purpura, cutaneous ulcers Renal = necrotising glomerulonephritis
What are some feature of GPA that occur in the nervous system?
Mononeuritis multiplex, sensorimotor polyneuropathy, cranial nerve palsies
What are some ocular features of GPA?
Conjunctivitis, episcleritis, uveitis, optic nerve vasculitis, renal artery occlusion, proptosis
How does EGPA present?
Similar features to GPA = main differences are late onset asthma and a high eosinophil count
How does MPA present?
Shares many features with GPA but respiratory features are rare
What is an example of an ANCA negative small vessel vasculitis?
Henoch-Schonlein purpura (HSP) = acute IgA mediated disorder
What occurs in HSP?
Generalised vasculitis of small vessels of skin, GI tract, kidneys, joints and rarely lungs and CNS
Who tends to get HSP?
Over 75% of cases are in children aged 2-11
What normally precedes HSP?
UTI, pharyngeal infection or GI infection = most commonly group A Strep, preceding illness occurs 1-3 weeks earlier
How does HSP present?
Purpuric rash typically over buttocks and limbs, colicky abdominal pain, bloody diarrhoea, joint pain +/- swelling, renal involvement
What is the prognosis of HSP?
Usually self limiting = resolves within 8 weeks
Relapses may occur for months to years
Must perform urinalysis screen for renal involvement
What investigations can be done for small vessels vasculitis?
Urine dipstick
FBC, liver and renal profile, inflammatory markers
ANCA and specific antibodies, connective tissue screening, compliment levels
CT scan
Nerve conduction tests and tissue biopsy
What is the prognosis of untreated AAVs?
Usually fatal
What is the treatment for localised/early systemic AAVs?
Methotrexate + steroids
What is the treatment for generalised/systemic AAVs?
Cyclophosphamide + steroids (1st line)
Ritiximab + steroids (alternative)
Plasma exchange if creatine > 500
Followed with azathioprine (or alternatives e.g methotrexate)
What is the treatment for refractory AAVs?
IV immunoglobulin, ritiximab
What are ANCAs?
Anti-Neutrophil Cytoplasmic Antibodies = auto-antibodies against antigens in the cytoplasm of neutrophil granulocytes
What is used to detect ANCA and differentiate patterns?
Immunofluorescence
What are the different ANCA patterns present in AAVs?
GPA = cANCA EGPA = pANCA MPA = pANCA
Do ANCA, anti-PR3 and anti-MPO stay constant in disease?
No = vary with disease activity
What are AAVs associated with?
Formation of immune complexes
What may happen to c3/4 levels during active disease?
Levels may fall as complement is consumed
