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RHEUMATOLOGY > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

What is vasculitis?

A

An inflammatory blood vessel disorder

Clinical features result from the damage of blood vessel walls with subsequent thrombosis, ischemia, bleeding, and/or aneurysm formation.

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2
Q

How is vasculitis classified?

A

By the predominant size, type, and location of involved blood vessels

It is a large, heterogeneous group of diseases.

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3
Q

What factors should be considered in the history for diagnosing vasculitis?

A
  • age
  • gender
  • ethnicity
  • comprehensive drug history
  • family history of vasculitis
  • constitutional symptoms

Constitutional symptoms include fever, weight loss, malaise, fatigue, diminished appetite, and sweats.

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4
Q

What is the ‘Glove and sweater’ approach in vasculitis diagnosis?

A

A series of questions from hands to head and down to trunk

It helps to identify symptoms related to vasculitis.

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5
Q

What vital signs should be checked during a physical exam for vasculitis?

A

Blood pressure and pulse

Important to assess for hypertension and regularity/rate of pulse.

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6
Q

What skin findings might indicate vasculitis?

A
  • palpable purpura
  • livedo reticularis
  • nodules
  • digital ulcers
  • gangrene
  • nail bed capillary changes

These findings are crucial for the diagnosis.

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7
Q

What are the primary types of small-vessel vasculitis?

A
  • 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.

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8
Q

Name a medium-vessel vasculitis.

A

Polyarteritis nodosa (PAN)
Kawasaki disease

This is a type of vasculitis affecting medium-sized vessels.

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9
Q

What are examples of large-vessel vasculitis?

A
  • Takayasu arteritis (TAK)
  • Giant cell arteritis (GCA)

These conditions primarily affect large blood vessels.

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10
Q

True or False: Secondary vasculitis is less common than primary vasculitis.

A

False

Secondary vasculitis is much more common than primary.

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11
Q

What initial tests are needed to exclude alternate diagnoses in vasculitis?

A
  • FBC
  • U&Es
  • LFTs
  • CRP
  • PV
  • ESR
  • Specific serology : ANA, ANCA, RF
  • hep screen, HIV
  • cryoglobulins
  • serum and urine protein electrophoresis
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12
Q

What is the first-line treatment for vasculitis?

A

Corticosteroids

They are essential for managing inflammation.

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13
Q

What are some examples of second-line treatments for vasculitis?

A
  • Cytotoxic medications
  • Immunomodulatory agents
  • Biologic agents

Examples include cyclophosphamide, methotrexate, azathioprine, and rituximab.

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14
Q

Fill in the blank: Hypersensitivity vasculitis is a common cause of skin vasculitis caused by _______.

A

[medication]

This condition arises as an adverse reaction to certain drugs.

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15
Q

What can Atrial Myxomas present with that is similar to vasculitis?

A

A vasculitis-like syndrome

An echocardiogram (ECHO) can exclude this condition.

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16
Q

What investigations are needed to delineate extent of organ involvement in vasculitis?

A
  • CK
  • blood cultures
  • ECG
  • CT
  • MRI
  • PET
17
Q

what are common features of vasculitis

A
  • 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)
18
Q

what investigations are indicated in vasculiutis and what might they show (2)

A
  • 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
19
Q

what is Henoch-Schonlein Purpura (HSP)

A

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

20
Q

where does HSP affect

A
  • skin
  • kidneys
  • GI tract
21
Q

what triggers HSP

A

upper airway infection or gastroenteritis

22
Q

what are the 4 classic features of HSP

A
  • Purpura (100%),
  • Joint pain (75%),
  • Abdominal pain (50%)
  • Renal involvement (IgA nephritis) (50%)
23
Q

what is the management of HSP

A

primarily supportive with analgesia, rest and good hydration
- urine dip: renal involvement
- BP: HTN

24
Q

what is Kawasaki’s disease

A

medium-vessel vasculitis affecting young children, typically under 5 years of age
- no clear cause

25
Q

what are clinical features of Kawasaki’s disease (5)

A
  • 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)
26
Q

what is a key complication of Kawasaki’s disease

A

coronary artery aneurysms

27
Q

how is Kawasaki’s disease treated

A

aspirin and IV immunoglobulins

28
Q

What is Behçet’s disease and what are its main features

A

complex inflammatory condition affecting the blood vessels and tissues
- main features are recurrent oral and genital ulcers

29
Q

What gene is Behçet’s disease associated with

A

HLA B51

30
Q

Describe in detail the features of Behçet’s disease

A

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

31
Q

What test can be used to diagnose Behçet’s disease

A

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

32
Q

What medication is involved in the management of Behçet’s disease

A
  • 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)
33
Q

What are major complications of Behçet’s disease

A
  • vision loss
  • ruptured pulmonary aneurysms
  • bowel perforation
  • neuro-Behçet’s disease
34
Q

How does granulomatosis with polyangiitis present

A
  • URT: epistaxis, sinusitis, nasal crusting
  • LRT: dyspndea, haemoptysis
  • GN
  • saddle shaped nose

RHEUMATOLOGY (14 decks)

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