Vasculitis Flashcards by Emilia McColm

Define vasculitis

Inflammatory disease of the blood vessels

How well did you know this?

Not at all

Perfectly

How is vasculitis classified?

Large vessel
Medium vessel
Small vessel
(4. Variable)

How well did you know this?

Not at all

Perfectly

Examples of large vessel vasculitis (2)

Giant cell arteritis

Takayasu arteritis

How well did you know this?

Not at all

Perfectly

Examples of medium vessel vasculitis (2)

Polyarteritis nodosa

Kawasaki disease

How well did you know this?

Not at all

Perfectly

Examples of small vessel vasculitis

ANCA positive - GPA, EGPA, MPA

Immune complex - anti-GBM, cryoglobulinaemic, IgAV, HUV

How well did you know this?

Not at all

Perfectly

Examples of variable vessel vasculitis (2)

Behcet’s

Cogan’s

How well did you know this?

Not at all

Perfectly

Giant cell arteritis - pathology

Systemic granulomatous arteritis (large vessel vasculitis). It affects the aorta and its major branches, particularly extracranial branches of the carotid artery.

Granulomatous lesions may be seen on biopsy (although up to 50% are normal)

How well did you know this?

Not at all

Perfectly

Most common type of GCA

Temporal arteritis

How well did you know this?

Not at all

Perfectly

Who is most typically affected by GCA?

Older (>50), white, female > males

How well did you know this?

Not at all

Perfectly

Temporal arteritis features

usually rapid onset (e.g. < 1 month)
headache
jaw claudication
visual disturbances (anterior ischemic optic neuropathy)
tender, palpable temporal artery

How well did you know this?

Not at all

Perfectly

Temporal arteritis investigations

ESR > 50 mm/hr
CRP may also be elevated
Temporal artery biopsy- granulomatous inflammation, but normal in 50% due to skip lesions
Creatine kinase and EMG = normal

How well did you know this?

Not at all

Perfectly

Temporal arteritis treatment

High-dose prednisolone + urgent opthal review (same day)

How well did you know this?

Not at all

Perfectly

What other condition is GCA/TA associated with?

What are the symptoms?

PMR (polymyalgia rheumatica)
Aching, morning stiffness in proximal limb muscles (not weakness)
also lethargy, depression, low-grade fever, anorexia, night sweats

How well did you know this?

Not at all

Perfectly

Takayasu arteritis - what is it?

Typically affects…?

Granulomatous inflammation, affecting aorta and its major branches

Young (< 40), mostly Asians, more common in females

How well did you know this?

Not at all

Perfectly

Takayasu arteritis symptoms

Intermittent claudication (usually upper limb)
Systemic features e.g. malaise, headache

How well did you know this?

Not at all

Perfectly

Takayasu arteritis signs

Decreased arterial pulse
BP difference between arms > 10 mmHg
Carotid bruit (listen for any large vessel bruit)
Aortic regurg

How well did you know this?

Not at all

Perfectly

Takayasu arteritis investigations

ESR raised
Arteriogram - narrowing of the aorta or its proximal branches (usually focal/segmental)
CT PET with increased FDG uptake in affected vessel walls

How well did you know this?

Not at all

Perfectly

Polyarteritis nodosa - what is it/pathology?

Vasculitis affecting medium-sized vessels with necrotizing inflammation leading to aneurysm formation.
–> major organ infarcts, ruptured aneurysms

How well did you know this?

Not at all

Perfectly

PAN is associated with?

More common in?

Study These Flashcards

Hepatitis B

Middle aged men

PAN symptoms

Study These Flashcards

Malaise, arthralgia, weight loss
Mononeuritis multiplex
Sensorimotor polyneuropathy
Testicular pain
Haematuria

PAN signs

Study These Flashcards

Pyrexia
Hypertension
Livedo reticularis
Renal failure (due to renal artery involvement)
Digital infarcts

PAN investigations

Study These Flashcards

Check Hep B serology
Perinuclear antineutrophil cytoplasmic antibodies (ANCA) positive in 20%
Mesenteric/renal angiogram -saccular aneurisms, vessel narrowing, dilatation

Kawasaki disease - what is it, who does it usually affect

Study These Flashcards

Uncommon medium vessel vasculitis

Children

Kawasaki disease features

Study These Flashcards

Conjunctival injection
Rash
Adenopathy (cervical lymphadenopathy)
Strawberry tongue (and red cracked lips)
Hands and feet (red + later peel)
and
BURN = high grade fever which lasts for >5 days (resistant to antipyretics)

Kawasaki disease management

Screen for coronary artery aneurysms (echo) High dose aspirin IVIG

Granulomatosis with polyangiitis (previously known as Wegener's) - what is it?

Autoimmune small vessel necrotising granulomatous vasculitis, affecting the upper and lower resp tract and the kidneys

Granulomatosis with polyangiitis features

Upper respiratory tract - epistaxis, sinusitis, nasal crusting, saddle shape nose deformity Lower respiratory tract - dyspnoea, haemoptysis Kidneys - rapidly progressive glomerulonephritis ('pauci-immune', 80% of patients) Can also get vasculitic rash, eye involvement and CN lesions

Granulomatosis with polyangiitis investigations

cytoplasmic (c)ANCA positive in > 90% CXR - may have cavitating lesions Renal biopsy - epithelial crescents in Bowman's capsule

Granulomatosis with polyangiitis management

steroids cyclophosphamide plasma exchange

Eosinophilic granulomatosis with polyangiitis (previously known as Churg-Strauss) - what is it?

ANCA-associated small vessel vasculitis

Eosinophilic granulomatosis with polyangiitis features

Asthma, blood eosinophilia (>10%), paranasal sinusitis, allergic rhinitis, mononeuritis multiplex

Eosinophilic granulomatosis with polyangiitis investigations

perinuclear (p)ANCA positive in 60%

Simple difference between GPA and EGPA

GP has kidney involvement GP = cANCA EGPA = pANCA

Microscopic polyangiitis features

Renal impairment: raised creatinine, haematuria, proteinuria Systemic symptoms: fever, lethargy, myalgia, weight loss Rash: palpable purpura Cough, dyspnoea, haemoptysis Mononeuritis multiplex

Microscopic polyangiitis investigations

pANCA (against myeloperoxidase) - positive in 50-75% cANCA (against proteinase 3) - positive in 40% NO GRANULOMATOSIS

What is anti-GBM/pathology (Goodpasture's)

Rare small vessel vasculitis associated with pulmonary haemorrhage followed by rapidly progressive glomerulonephritis It is caused by anti-glomerular basement membrane antibodies against type IV collagen

Associations with anti-GBM

HLA DR2 Men Ages 20-30, and 60-70 (two peaks)

Anti-GBM investigations

Renal biopsy: linear IgG deposits along the basement membrane Raised transfer factor secondary to pulmonary haemorrhages

Anti-GBM management

plasma exchange steroids cyclophosphamide

What is Behcet’s?

Complex multisystem disorder associated with presumed autoimmune-mediated inflammation of the arteries and veins.

Behcet's classic triad

Oral ulcers Genital ulcers Anterior uveitis

Other features of Behcet's

VTE, arthritis, neuro involvement e.g. aseptic meningitis, abdo pain, diarrhoea, colitis, erythema nodosum

Behcet's investigations

Clinical diagnosis | Positive pathergy test is suggestive (puncture site following needle prick becomes inflamed with small pustule forming)

Behcet's epidemiology

More common in the eastern Mediterranean (e.g. Turkey), in men, and in young adults Associated with HLA B51

General diagnostic approach to vasculitis

Lab tests - RF, ANA, ANCA, cryoglobulins, C3, C4, ESR, CRP Tissue biopsy of affected organ Imaging e.g. CT PET for large vessels, angiography for PAN, echo for Kawasaki's Rule out differentials - blood culture, viral hep and HIV screen, echo, protein electrophoresis

Vasculitis mimics

Drugs Infections e.g. HIV, sepsis, hepatitis Malignancy e.g. myeloma

Vasculitis Flashcards

(46 cards)