Vasculitis Flashcards
(40 cards)
1
Q
What is vasculitis?
A
- Inflammation of blood vessel walls
- Associated with the ANCA protein
- Classified by vessel size
2
Q
What are the causes of vasculitis?
A
- Infection (majority)
- Drugs
- Cancer
- Autoimmune (RA)
- Cold temperature
3
Q
Describe the categories of Vasculitis?
A
- Large vessels
- Medium vessels
- Small vessels
4
Q
Name types of large vessel vasculitis?
A
- Giant cell/ Temporal arteritis
- Takayasus’s arteritis
5
Q
Name types of medium vessel vasculitis?
A
- Kawasaki’s
- Polyarteritis nodosa
6
Q
Name types of small vessel vasculitis?
A
Henoch-schonlein purpura
7
Q
Name some of the clinical manifestations of vasculitis?
A
- Arthritis
- Kidney failure
- Purpura rashes
8
Q
What is the treatment for vasculitis?
A
Corticosteroids
9
Q
How is vasculitis diagnosed?
A
- Raised ESR and CRP in blood
- Definitive diagnosis made by biopsy of vessel
10
Q
Describe Henoch-schonlein purpura?
A
- Children
- Begins in foot then moves up
- Arthritis
- Abdominal pain
- Purpura rash in LLs
11
Q
What are the causes of cutaneous vasculitis?
A
- Idiopathic
- Drugs
- Infection
- Malignancy
- Manifestation of small/medium ANCA associated vasculitis
12
Q
Name some complications of Henoch-Schonlein Purpura?
A
- Orchitis
- GI bleeds
- Diarrhoea
- IgA nephropathy
13
Q
Describe the management of Henoch Schonlein Purpura?
A
- Usually self-limiting
- Corticosteroids for complications
14
Q
What can sometimes trigger Henoch Schonlein Purpura?
A
Streptoccocal sore throat
15
Q
Describe Granulomatosis with polyangiitis (GPA)?
A
- Granuloma formation
- Nasal passages, airways and kidney
- Presentation with epistaxis, nasal crusting, sinusitis
- Deafness and proptosis may occur
- Pauci-immune glomerulonephritis
16
Q
What was the previous name of Granulomatosis with polyangiitis (GPA)?
A
Wegener’s granulomatosis
17
Q
What is ANCA?
A
- Autoantibodies which attack neutrophils and monocytes
- Often present in vasculitis
18
Q
How can you test for ANCA?
A
- Indirect immunofluoresence
- ELISA for PR3 / MPO
19
Q
What are the uses of ANCA?
A
- Diagnosis
- Prognosis
- Response to treatment
- Monitoring for relapse
20
Q
What are the pitfalls of ANCA testing?
A
- Must be done in clinical setting
- Not all autoimmune vasculiis is positive for ANCA
21
Q
How do you treat someone who is positive for ANCA?
A
- Remission induction
- Remission maintenace
22
Q
What drugs are used for remission induction?
A
- Prednisolone AND
-
For severe disease:
- Cyclophosphamide
- Rituximab
-
For moderate disease:
- Methotrexate
- Mycophenolate
23
Q
What drugs would be used for remission maintenance?
A
- Azathioprine
- Methotrexate
24
Q
What are the main subtypes of ANCA vasculitis?
A
- Microscopic polyangitis
- Granulomatosis with polyangitis
25
Describe Microscopic polyangitis?
* Necrotising small-vessel vasculitis
* Rapidly progressive glomerulonephritis
* Often with alveolar haemorrhage
* MPO antibody positive
26
Gold standard for diagnosis of ANCA vasculitis?
Biopsy of kidneys or lesions in sinuses/upper airways
27
Describe the management of ANCA-associated vasculitis?
* High dose glucocorticoids
* + Cyclophosphamide
* Followed by maintenace therapy:
* Glucocorticoids + methotrexate
* Plasmapheresis for fulminant lung disease
28
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
29
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
30
Describe Polyarteritis nodosa?
* HBV is an important risk factor
* Fever, myalgia, arthralgia, weight loss
* Palpable purpura, ulceration, livedo reticularis
31
What is pictured here?
* Palpable purpura of systemic vasculitis
* aka Polyarteritis nodosa
32
What is pictured here?
Livedo reticularis
33
How is Polyarteritis nodosa diagnosed?
* MRI
* Multiple aneurysms
* Smooth narrowing of mesenteric, hepatic or renal systems
* Muscle / Sural nerve biopsy
34
Describe the treatment of polyarteritis nodosa?
* High-dose glucocorticoids + cyclophosphamide
* Maintenance therapy
* Glucocorticoid + methotrexate
35
What is another name for Churg-Strauss syndrome?
Eosinophilic granulomatosis with polyangitis
36
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
37
Describe the acute presentation of Eosinophilic granulomatosis with polyangitis?
* Triad of acute presentation:
* Purpura
* Asymmetric mononeuritis multiplex
* Eosinophilia
38
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
39
Describe Behcet's disease?
* Vasculitis of unknown cause
* Oral ulcers
* Deep and multiple
* Genital ulcers
* Erythema nodosum, acneiform lesions
* Neurological involvement can occur
40
Describe the management of Behcet's disease?
* Topical glucocorticoid
* Prednisolone mouthwash
* Erythema nodosum, arthralgia
* Colchicine
* Resistant oral and genital ulcers
* Thalidomide
