Vasculitis Flashcards
What is vasculitis?
- Inflammation of the blood vessels
* umbrella term for a group of autoimmune conditions called vasculitides
Name the different categories of vasculitis
- Large vessel vasculitis
- Medium vessel vasculitis
- ANCA-associated small vessel vasculitis
- Immune complex small vessel vasculitis
What are the large vessel vasculitis types?
- Takayasu arteritis
* Giant cell arteritis
What are the medium vessel vasculitis types?
- Polyarteritis nodosa
* Kawasaki disease
What are the ANCA-associated small vessel vasculitis types?
- Microscopic polyangiitis
- Wegener’s: Granulomatosis with polyangiitis
- Churg-Strauss: Eosinophilic granulomatosis with polyangiitis
What are the immune complex small vessel vasculitis types?
- Cryoglobulinemic vasculitis
- IgA vasculitis (Henoch schonlein purpura)
- Hypocomplementemic urticarial vasculitis
What is giant cell arteritis?
- Large vessel vasculitis
* Systemic vasculitis that affects the aorta and its major branches
What is the clinical presentation of giant cell arteritis?
- Headache: subacute onset, constant, temporal with tenderness, little relief with analgesics
- Visual symptoms
- Jaw claudication (sore to eat)
- Polymyalgia rheumatica symptoms (stiffness in the shoulder and pelvic girdle)
- Constitutional upset: weight loss, malaise, night sweats
What are the complications of giant cell arteritis?
- Visual loss: acute ischaemic neuropathy: sudden painless loss of vision, occasionally preceded by amaurosis fungax
- Large vessel vasculitis: vascular stenosis and aneurysm
- CVA: obstruction or occlusion of internal carotid artery or vertebral arteries
How do you diagnose giant cell arteritis?
- Clinical presentation: symptoms, age, no alternate diagnosis, associated features
- Clinical examination findings: temporal artery asymmetry, thickening and loss of pulsatility (tenderness)
- acute phase response: ESR/CRP
- Further investigation: biopsy
Describe the correlation between age and temporal arteritis
- Rare below 50
* Peak incidence 70-79
Explain what would confirm giant cell arteritis on a biopsy of the temporal artery
- Interruption of the internal elastic laminae
- Mononuclear inflammatory cell infiltrate within vessel wall
- Multinucleated giant cells in 40-60% of cases
If biopsy of the temporal artery are not available, what other investigations can be used to investigate giant cell arteritis?
- Temporal artery USS
- MRI
- PET CT
What is the treatment of giant cell arteritis?
•Prednisolone 60mg for 1 month, taper to 15mg by 12 weeks
•Aim to discontinue corticosteroids by 12-18 months •Corticosteroid sparing therapy in patients with disease relapse:
- mycophenolate mofetil
- methotrexate
- tocilizumab
What are the causes of a cutaneous small vessel vasculitis?
- Can be idiopathic
- drugs
- infection: HCV, HBV, gonococcus, meningococcus, HIV
- Secondary RA/CTD/PBC/UC
- Malignancy: haematological > solid organ
- Manifestation of small/medium vessel ANCA associated vasculitis
Describe the common patient characteristics of those with henoch-schonlein purpura
- children 2-11 years or adults mean age 43
- Male>females
- Often after an infection
What are the clinical signs and symptoms of henoch schonlein purpura?
- Classic purpuric rash: buttocks and thighs>lower legs
- Urticarial rash, confluent petechiae, eccymoses, ulcers
- Arthralgia/arthritis in the lower limb in 75%
What are the complications of henoch schonlein purpura?
- GI: pain, bleeding, diarrhoea, rarely intussusception
- Renal: IgA nephropathy
- Urological: orchitis
Describe an overview of the diagnosis of henoch schonlein purpura
- Exclude secondary causes: history, examination, immunology, virology
- Assess extent if involvement: urinalysis/urine PCR
Describe the management and prognosis of Henoch schonlein purpura
- Often self limiting and no treatment required (up to 16 weeks)
- Corticosteroids for certain complications: testicualr torsion, GI disease, arthritis
- Relapses in 5-10%, this tends to occur within 12 months
What is it important to exclude in a suspected henoch schonlein prupura?
- Virus
- Malignancy
- other autoimmune conditions
- drugs
Describe the pathology of granulomatosis with polyangiitis
- Granulomatous necrotising inflammatory lesions of the upper and lower respiratory tract
- Often pauci-immune glomerulonephritis
explain the triad of disease in glomerulomatosis polyangiitis
- upper airway/ENT: rhinitis, chronic sinusitis, chronic otitis media, saddle nose deformity, nasal septum perforation
- Renal: pouch-immune glomerulonephritis
- lower respiratory: parenchymal nodules and cavitation, alveolar haemorrhage
What are the constitutional symptoms in granulomatosis with polyangiitis?
- fatigue
- Weight loss
- fever/sweats
- Myalgia/arthralgia
- Failure to thrive in elderly
What are the organ specific features of granulomatosis with polyangiitis?
- Oral cavity: ulcerations
- Lungs: cavities, bleeds, lung infiltrates
- skin: nodules on elbow, pupura
- Eyes: pseudotumours, conjunctivitis
- Nose: stuffiness, nosebleeds, saddle nose
- Heart: pericarditis
- Kidneys: glomerulonephritis
What is ANCA?
- Autoantibodies directed against the cytoplasmic constituents of neutrophils and monocytes
- Two types: cANCA (cytyoplasmic) and pANCA (periphery of neutrophil)
What are the means of testing for ANCA?
- Indirect immunofluorescence
* ELISA for PR3/MPO
What is PR3 ANCA an indicator of?
Granulomatosis with polyangiitis
What is MPO ANCA indicative of?
Microscopic polyangiitis
What does a high ANCA mean?
That relapse is more likely
What can ANCA testing be used for?
- Diagnosis
- Prognostic info
- Assessing a response to treatment
- Monitoring for early signs of relapse
Describe the treatment of ANCA associated small vessel vasculitis
1) remission induction for 3-6 months: switch of vasculitis activity: prednisolone and cyclophosphamide or rituximab or methotrexate (milder) or mycophenolate (milder)
2) Remission maintenance 2+ years: prevent relapse: azathioprine or methotrexate or rituximab
