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Y6 T&O + MSK > Vasculitides > Flashcards

Vasculitides Flashcards

1
Q

How is vasculitis classified

A

Large:
- Takayasu’s arteritis
- Giant cell arteritis
- Polymyalgia rheumatica

Medium:
- Polyarteritis nodosa
- Kawasaki disease

Small:
- Behcets disease
- Granulomatosis with polyangiitis (Wegener’s)
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
- Microscopic polyangiitis
- IgA vasculitis/Henoch-schonlein purpura
- Goodpasture’s/anti-GBM
- Cyroglobulinaemic vasculitis
- Anti-C1q vasculitis

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2
Q

Aetiology and associations for Takayasu’s arteritis

A

Involves the aorta and its branches
Middle-aged Asian/Japanese women

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3
Q

Symptoms and signs of Takayasu’s arteritis

A

Inflammatory phase: FLAWS, head or neck pain, tenderness over arteries
Carotid: CNS disease
Syncope on raising arms (subclavian steal)
Pulseless phase: weak/absent UL/LL pulses and upper limb claudication, tenderness over affected arteries

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4
Q

Investigations for takayasu’s arteritis

A

ESR/CRP: raised
MRA, CTGA - image aorta and arterial tree
FDG-PET: active inflammation

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5
Q

Management for Takayasu’s arteritis

A

High dose steroid e.g. prednisolone 20mgs PO
Steroid-sparing: cyclophosphamide, methotrexate
Biologics
Endovascular interventions

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6
Q

Aetiology and associations of polyarteritis nodosa

A

Idiopathic and associated with hepatitis B

GI, renal, coronary arteries affected

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7
Q

Symptoms and signs of polyarteritis nodosa

A

Constitutional (fever, malaise, lethargy)
Skin rash
Abdominal pain
Rectal bleeding
Peripheral neuropathy
Hypertension

*NO glomerulonephritis

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8
Q

Investigations for polyarteritis nodosa

A

Hep B serology
Angiography - Rosary sign

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9
Q

Management for polyarteritis nodosa

A

Mild: PO steroids
Systemic: IV steroids ± IV cyclophosphamide
Anti-virals for current hep B infection

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10
Q

Symptoms and signs of Kawasaki disease

A

Often <5yo

5 days fever
Conjunctivitis (non purulent, exudative)
Rash, polymorphic
Adenopathy, cervical
Strawberry tongue (prominent papillae and erythema)
Hands and feet swollen, red, tender

Other: Pericarditis with effusion, congestive HF, joint pain or oedema, neurological manifestations, GI manifestations, urological manifestations, dermatological manifestations

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11
Q

Investigations for Kawasaki disease

A

ECG

FBC: anaemia, leucocytosis/thrombocytosis
CRP/ESR: raised

Echo: Rule out coronary artery aneurysm

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12
Q

Management for kawasaki disease

A

<10 days from onset:
1. IVIg + high dose aspirin
2. Steroids e.g. IV methylpred or PO pred
3. Immunomodulation e.g. ciclosporin

> 10 days: low dose aspirin PO for 6-8 weeks

+ follow up echo

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13
Q

Association, symptoms and signs of Behcets disease

A

HLA-B51

TRIAD:
1. Recurrent oral ulcers
2. Genital ulcers
3. Uveitis, iritis, retinal vasculitis , optic neuropathy

Rash, arthritis, pericarditis, colitis, thrombophlebitis, DVT,

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14
Q

Investigations for Behcets disease

A

Clinical Diagnosis
CRP/ESR raised
Pathergy test - needle prick becomes inflamed and sterile pustule develops within 48 hours

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15
Q

Management for Behcets disease

A

Prednisolone
Colchicine
Azathioprine, Cyclosporin, Cyclophosphamide sometimes used
TNF alpha blockers
Type I IFN (IFN alpha) rarely

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16
Q

Aetiology, symptoms and signs of Granulomatosis with polyangiitis

A

c-ANCA (against proteinase 3)

  1. URT: epistaxis, rhinitis, saddle nose
  2. LRT: pulmonary haemorrhage → haemoptysis
  3. Renal: glomerulonephritis → haematuria

± sore eyes, ears, joints, gangrene

17
Q

Investigations for Granulomatosis with polyangiitis

A

Urinalysis: haematuria, proteinuria, red cell clasts

cANCA
CRP/ESR raised

CXR shows cavitating lesions

18
Q

Aetiology, symptoms and signs of Eosinophilic granulomatosis with polyangiitis

A

p-ANCA

TRI-PHASIC:
1. Allergic: Asthma/rhintiis
2. Eosinophilic: Tissue damage in lung and GIT
- Lung – eosinophilic pneumonitis – transient pulmonary infiltrates
- Skin – nodules
- Nervous system- mononeuritis multiplex
- Sinusitis
- Heart – myocarditis
3. Vasculitic: widespread organ damage and death

19
Q

Aetiology, symptoms and signs for microscopic polyangiitis

A

P-ANCA presence
Men > women

Rapidly progressing glomerulonephritis
Pulmonary haemorrhage → haemoptysis
+ vasculitic skin rashes, myositis, mononeuritis multiplex

20
Q

Aetiology of IgA vasculitis

A

Post-strep infection → nephritic syndrome 2 days after

21
Q

Symptoms and signs of IgA vasculitis

A

Purpura on leg and buttocks
TRIAD
1. Purpuric rash on buttocks and extensors of lower limbs
2. Abdominal pain
3. Arthralgia
(oedema)

22
Q

Investigations for IgA vasculitis

A

Raised ESR and CRP
Reduced complement C4 and C3 (sometimes)
IgA may be raised on blood test
Biopsy (skin, kidney) to demonstrate small vessel vasculitis with IgA immune complexes

23
Q

Aetiology, symptoms and signs for Goodpasture’s disease

A

Anti-GBM
TRIAD
- Glomerulonephritis
- Haemoptysis (pulmonary haemorrhage)
- Anti-GBM antibodies

24
Q

Investigations for Goodpasture’s disease

A

ESR/CRP: raised
CXR and pulmonary function test: pulmonary haemorrhage
Renal biopsy: glomerulonephritis + antibody deposition across the BM
Anti-GBM antibodies

25
Q

Management for Goodpasture’s disease

A

High dose prednisolone
Cyclophosphamide
Plasmapharesis
Rituximab

26
Q

Aetiology of cryoglobulinaemia

A

Disease due to immune complexes that precipitate at low temperatures
Type 1 – monoclonal antibody that is not a rheumatoid factor
Type 2 – monoclonal rheumatoid factor (mixed)
Type 3 – polyclonal rheumatoid factor (mixed)

Essential = no underlying disease
Secondary to lymphoma, CTD, hep C

27
Q

Symptoms and signs of cryoglobulinaemia

A

Skin rash
Arthralgias
Glomerulonephritis
Interstitial lung disease
Neuropathy

28
Q

Investigations for cryoglobulinaemia

A

Cryoglobulin within serum
Reduced complement C3, C4
Raised ESR
Investigate for underlying condition (eg hepatitis C serology, ANA, consider lymphoma)
Biopsy to demonstrate small vessel vasculitis with immune complex deposition

29
Q

Management for cryoglobulinaemia

A

Treat underlying condition
Prednisolone
Azathioprine as steroid sparing agent
Plasmapharesis in severe disease (rarely)
Rituximab may be effective

30
Q

Symptoms and signs of anti-C1q disease

A

Recurrent urticaria with lesions that persist >24 hours (urticarial vasculitis)
Purpura
Arthralgias
Glomerulonephritis
Ocular inflammation
Obstructive airways symptoms
Angiooedema
May occur in patients with SLE

31
Q

Investigations for anti-C1q disease

A

Antibodies specific for C1q
Low complement C3 and C4 in more severe cases
Biopsy to show vasculitis

32
Q

Management for anti-C1q disease

A

NSAIDs
Prednisolone
Azathioprine
Mycophenolate for severe disease

Y6 T&O + MSK (23 decks)

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