Vasculitis Flashcards
What is vasculitis?
- Inflammation of blood vessel walls
- Associated with the ANCA protein
- Classified by vessel size
What are the causes of vasculitis?
- Infection (majority)
- Drugs
- Cancer
- Autoimmune (RA)
- Cold temperature
Describe the categories of Vasculitis?
- Large vessels
- Medium vessels
- Small vessels
Name types of large vessel vasculitis?
- Giant cell/ Temporal arteritis
- Takayasus’s arteritis
Name types of medium vessel vasculitis?
- Kawasaki’s
- Polyarteritis nodosa
Name types of small vessel vasculitis?
Henoch-schonlein purpura
Name some of the clinical manifestations of vasculitis?
- Arthritis
- Kidney failure
- Purpura rashes
What is the treatment for vasculitis?
Corticosteroids
How is vasculitis diagnosed?
- Raised ESR and CRP in blood
- Definitive diagnosis made by biopsy of vessel
Describe Henoch-schonlein purpura?
- Children
- Begins in foot then moves up
- Arthritis
- Abdominal pain
- Purpura rash in LLs
What are the causes of cutaneous vasculitis?
- Idiopathic
- Drugs
- Infection
- Malignancy
- Manifestation of small/medium ANCA associated vasculitis
Name some complications of Henoch-Schonlein Purpura?
- Orchitis
- GI bleeds
- Diarrhoea
- IgA nephropathy
Describe the management of Henoch Schonlein Purpura?
- Usually self-limiting
- Corticosteroids for complications
What can sometimes trigger Henoch Schonlein Purpura?
Streptoccocal sore throat
Describe Granulomatosis with polyangiitis (GPA)?
- Granuloma formation
- Nasal passages, airways and kidney
- Presentation with epistaxis, nasal crusting, sinusitis
- Deafness and proptosis may occur
- Pauci-immune glomerulonephritis
What was the previous name of Granulomatosis with polyangiitis (GPA)?
Wegener’s granulomatosis
What is ANCA?
- Autoantibodies which attack neutrophils and monocytes
- Often present in vasculitis
How can you test for ANCA?
- Indirect immunofluoresence
- ELISA for PR3 / MPO
What are the uses of ANCA?
- Diagnosis
- Prognosis
- Response to treatment
- Monitoring for relapse
What are the pitfalls of ANCA testing?
- Must be done in clinical setting
- Not all autoimmune vasculiis is positive for ANCA
How do you treat someone who is positive for ANCA?
- Remission induction
- Remission maintenace
What drugs are used for remission induction?
- Prednisolone AND
-
For severe disease:
- Cyclophosphamide
- Rituximab
-
For moderate disease:
- Methotrexate
- Mycophenolate
What drugs would be used for remission maintenance?
- Azathioprine
- Methotrexate
What are the main subtypes of ANCA vasculitis?
- Microscopic polyangitis
- Granulomatosis with polyangitis
Describe Microscopic polyangitis?
- Necrotising small-vessel vasculitis
- Rapidly progressive glomerulonephritis
- Often with alveolar haemorrhage
- MPO antibody positive
Gold standard for diagnosis of ANCA vasculitis?
Biopsy of kidneys or lesions in sinuses/upper airways
Describe the management of ANCA-associated vasculitis?
- High dose glucocorticoids
- Cyclophosphamide
- Followed by maintenace therapy:
- Glucocorticoids + methotrexate
- Plasmapheresis for fulminant lung disease
Describe Takayasu arteritis?
- Aorta and its major branches
- Granulomatous inflammation of vessel wall leading to occlusion
- Claudication, fever, arthralgia, weight loss
- Tx:
- High dose glucocorticoids + immunosuppressants
Describe Kawasaki disease?
aka Mucocutaneous lymph node syndrome
- Systemic vasculitis in children <5 years old
- May eventually affect the coronary vessels
- Fever, generliased rash, enlarged lymph nodes, strawberry tongue
- Inflamed oral mucosa and conjunctiva
- Tx:
- High doses of Aspirin + IV immunoglobulin
Describe Polyarteritis nodosa?
- HBV is an important risk factor
- Fever, myalgia, arthralgia, weight loss
- Palpable purpura, ulceration, livedo reticularis
What is pictured here?
- Palpable purpura of systemic vasculitis
- aka Polyarteritis nodosa
What is pictured here?
Livedo reticularis
How is Polyarteritis nodosa diagnosed?
- MRI
- Multiple aneurysms
- Smooth narrowing of mesenteric, hepatic or renal systems
- Muscle / Sural nerve biopsy
Describe the treatment of polyarteritis nodosa?
- High-dose glucocorticoids + cyclophosphamide
- Maintenance therapy
- Glucocorticoid + methotrexate
What is another name for Churg-Strauss syndrome?
Eosinophilic granulomatosis with polyangitis
Describe Eosinophilic granulomatosis with polyangitis?
- Small vessel vasculitis with eosinophilia
- Prodrome can occur for several years before onset
- Allergic rhinitis, nasal polyposis, late-onset asthma
Describe the acute presentation of Eosinophilic granulomatosis with polyangitis?
- Triad of acute presentation:
- Purpura
- Asymmetric mononeuritis multiplex
- Eosinophilia
Describe the diagnosis and management of Eosinophilic granulomatosis with polyangitis?
- Vessel biopsy
- Small vessel vasculitis
- Eosinophilic infiltration of vessel wall
- Management
- High dose glucocorticoids + cyclophosphamide
- Maintenance therapy
Describe Behcet’s disease?
- Vasculitis of unknown cause
- Oral ulcers
- Deep and multiple
- Genital ulcers
- Erythema nodosum, acneiform lesions
- Neurological involvement can occur
Describe the management of Behcet’s disease?
- Topical glucocorticoid
- Prednisolone mouthwash
- Erythema nodosum, arthralgia
- Colchicine
- Resistant oral and genital ulcers
- Thalidomide
