Vasculitis Flashcards
What is vasculitis?
An inflammatory blood vessel disorder
Clinical features result from the damage of blood vessel walls with subsequent thrombosis, ischemia, bleeding, and/or aneurysm formation.
How is vasculitis classified?
By the predominant size, type, and location of involved blood vessels
It is a large, heterogeneous group of diseases.
What factors should be considered in the history for diagnosing vasculitis?
- age
- gender
- ethnicity
- comprehensive drug history
- family history of vasculitis
- constitutional symptoms
Constitutional symptoms include fever, weight loss, malaise, fatigue, diminished appetite, and sweats.
What is the ‘Glove and sweater’ approach in vasculitis diagnosis?
A series of questions from hands to head and down to trunk
It helps to identify symptoms related to vasculitis.
What vital signs should be checked during a physical exam for vasculitis?
Blood pressure and pulse
Important to assess for hypertension and regularity/rate of pulse.
What skin findings might indicate vasculitis?
- palpable purpura
- livedo reticularis
- nodules
- digital ulcers
- gangrene
- nail bed capillary changes
These findings are crucial for the diagnosis.
What are the primary types of small-vessel vasculitis?
- Microscopic polyangiitis (MPA)
- Granulomatosis with polyangiitis (GPA)
- Eosinophilic granulomatosis with polyangiitis (EGPA)
- IgA vasculitis
GPA was formerly known as Wegener’s granulomatosis and EGPA as Churg-Strauss syndrome.
Name a medium-vessel vasculitis.
Polyarteritis nodosa (PAN)
Kawasaki disease
This is a type of vasculitis affecting medium-sized vessels.
What are examples of large-vessel vasculitis?
- Takayasu arteritis (TAK)
- Giant cell arteritis (GCA)
These conditions primarily affect large blood vessels.
True or False: Secondary vasculitis is less common than primary vasculitis.
False
Secondary vasculitis is much more common than primary.
What initial tests are needed to exclude alternate diagnoses in vasculitis?
- FBC
- U&Es
- LFTs
- CRP
- PV
- ESR
- Specific serology : ANA, ANCA, RF
- hep screen, HIV
- cryoglobulins
- serum and urine protein electrophoresis
What is the first-line treatment for vasculitis?
Corticosteroids
They are essential for managing inflammation.
What are some examples of second-line treatments for vasculitis?
- Cytotoxic medications
- Immunomodulatory agents
- Biologic agents
Examples include cyclophosphamide, methotrexate, azathioprine, and rituximab.
Fill in the blank: Hypersensitivity vasculitis is a common cause of skin vasculitis caused by _______.
[medication]
This condition arises as an adverse reaction to certain drugs.
What can Atrial Myxomas present with that is similar to vasculitis?
A vasculitis-like syndrome
An echocardiogram (ECHO) can exclude this condition.
What investigations are needed to delineate extent of organ involvement in vasculitis?
- CK
- blood cultures
- ECG
- CT
- MRI
- PET
what are common features of vasculitis
- Joint and muscle pain
- Peripheral neuropathy
- Renal impairment
- Purpura (purple-coloured non-blanching spots caused by blood leaking from the vessels under the skin)
- Necrotic skin ulcers
- Gastrointestinal symptoms (e.g., diarrhoea, abdominal pain and bleeding)
what investigations are indicated in vasculiutis and what might they show (2)
- inflamm markers e.g. CRP, ESR usually raised
-
anti-neutrophil cytoplasmic antibodies (ANCA) are associated with specific types of vasculitis
- p-ANCA (or MPO antibodies) – microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis
- c-ANCA (or PR3 antibodies) – granulomatosis with polyangiitis
what is Henoch-Schonlein Purpura (HSP)
IgA vasculitis that commonly presents in children <10 with a purpuric rash affecting lower limbs and buttocks
- inflammation occurs due to immunoglobulin A deposits and blood vessels
where does HSP affect
- skin
- kidneys
- GI tract
what triggers HSP
upper airway infection or gastroenteritis
what are the 4 classic features of HSP
- Purpura (100%),
- Joint pain (75%),
- Abdominal pain (50%)
- Renal involvement (IgA nephritis) (50%)
what is the management of HSP
primarily supportive with analgesia, rest and good hydration
- urine dip: renal involvement
- BP: HTN
what is Kawasaki’s disease
medium-vessel vasculitis affecting young children, typically under 5 years of age
- no clear cause
what are clinical features of Kawasaki’s disease (5)
- Persistent high fever for more than 5 days
- Widespread erythematous maculopapular rash
- Desquamation (skin peeling) of palms and soles
- Bilateral conjunctivitis
- Strawberry tongue (red tongue with prominent papillae)
what is a key complication of Kawasaki’s disease
coronary artery aneurysms
how is Kawasaki’s disease treated
aspirin and IV immunoglobulins
What is Behçet’s disease and what are its main features
complex inflammatory condition affecting the blood vessels and tissues
- main features are recurrent oral and genital ulcers
What gene is Behçet’s disease associated with
HLA B51
Describe in detail the features of Behçet’s disease
Oral ulcers are expected at least 3 times per- in patients with Behçet’s disease
- painful, sharply circumscribed erosions with a red halo occurring on the oral mucosa and heal over 2-4 weeks
- Genital ulcers are similar in appearance to oral ulcers
- Ulcers may leave a scar
What test can be used to diagnose Behçet’s disease
Pathergy test
- involves using a sterile needle to make multiple pricks on the forearm
- area is reviewed 24-48 hours later to look for erythema (redness) and induration (thickening), indicating non-specific skin hypersensitivity
What medication is involved in the management of Behçet’s disease
- Topical steroids for mouth ulcers (e.g., soluble betamethasone tablets)
- Systemic steroids (e.g., oral prednisolone)
- Colchicine can be helpful with symptoms
- Topical anaesthetics for genital ulcers (e.g., lidocaine ointment)
- Immunosuppressants (e.g., azathioprine)
- Biologic therapy (e.g., infliximab)
What are major complications of Behçet’s disease
- vision loss
- ruptured pulmonary aneurysms
- bowel perforation
- neuro-Behçet’s disease
How does granulomatosis with polyangiitis present
- URT: epistaxis, sinusitis, nasal crusting
- LRT: dyspndea, haemoptysis
- GN
- saddle shaped nose
